AHA/ACC guidelines

Accepted Manuscript
2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart
Disease
Karen K. Stout, MD, FACC, Chair, Writing Committee, Curt J. Daniels, MD, Vice
Chair, Writing Committee, Jamil A. Aboulhosn, MD, FACC, FSCAI, Writing Committee
Member, Biykem Bozkurt, MD, PhD, FACC, FAHA, Writing Committee Member, Craig
S. Broberg, MD, FACC, Writing Committee Member, Jack M. Colman, MD, FACC,
Writing Committee Member, Stephen R. Crumb, DNP, AACC, Writing Committee
Member, Joseph A. Dearani, MD, FACC, Writing Committee Member, Stephanie
Fuller, MD, MS, FACC, Writing Committee Member, Michelle Gurvitz, MD, FACC,
Writing Committee Member, Paul Khairy, MD, PhD, Writing Committee Member,
Michael J. Landzberg, MD, FACC, Writing Committee Member, Arwa Saidi, MB, BCH,
FACC, Writing Committee Member, Anne Marie Valente, MD, FACC, FAHA, FASE,
Writing Committee Member, George F. Van Hare, MD, Writing Committee Member
PII: S0735-1097(18)36846-3
DOI: 10.1016/j.jacc.2018.08.1029
Reference: JAC 25255
To appear in: Journal of the American College of Cardiology
Please cite this article as: Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM,
Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare
GF, 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease, Journal of
the American College of Cardiology (2018), doi: 10.1016/j.jacc.2018.08.1029.
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2018 AHA/ACC Guideline for the Management of Adults With
Congenital Heart Disease
A Report of the American College of Cardiology/American Heart Association Task Force on
Clinical Practice Guidelines
Developed in Collaboration With the American Association for Thoracic Surgery, American Society of
Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease,
Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
WRITING COMMITTEE MEMBERS*
Karen K. Stout, MD, FACC, Chair†
Curt J. Daniels, MD, Vice Chair*†‡
Jamil A. Aboulhosn, MD, FACC, FSCAI*§ Stephanie Fuller, MD, MS, FACC#
Biykem Bozkurt, MD, PhD, FACC, FAHA║ Michelle Gurvitz, MD, FACC**
Craig S. Broberg, MD, FACC*† Paul Khairy, MD, PhD*†
Jack M. Colman, MD, FACC† Michael J. Landzberg, MD, FACC*†
Stephen R. Crumb, DNP, AACC† Arwa Saidi, MB, BCH, FACC*†
Joseph A. Dearani, MD, FACC¶ Anne Marie Valente, MD, FACC, FAHA, FASE††
George F. Van Hare, MD‡‡
ACC/AHA TASK FORCE MEMBERS
Glenn N. Levine, MD, FACC, FAHA, Chair
Patrick T. O’Gara, MD, MACC, FAHA, Chair-Elect
Jonathan L. Halperin, MD, FACC, FAHA, Immediate Past Chair
Nancy M. Albert, PhD, RN, FAHA§§ Zachary D. Goldberger, MD, MS, FACC, FAHA
Sana M. Al-Khatib, MD, MHS, FACC, FAHA Mark A. Hlatky, MD, FACC
Joshua A. Beckman, MD, MS, FAHA John Ikonomidis, MD, PhD, FAHA
Kim K. Birtcher, PharmD, MS, AACC José Joglar, MD, FACC, FAHA
Biykem Bozkurt, MD, PhD, FACC, FAHA§§ Richard J. Kovacs, MD, FACC, FAHA§§
Ralph G. Brindis, MD, MPH, MACC§§ Laura Mauri, MD, MSc, FAHA
Joaquin E. Cigarroa, MD, FACC E. Magnus Ohman, MD, FACC§§
Lesley H. Curtis, PhD, FAHA§§ Mariann R. Piano, RN, PhD, FAHA, FAAN
Anita Deswal, MD, MPH, FACC, FAHA Susan J. Pressler, PhD, RN, FAHA§§
Lee A. Fleisher, MD, FACC, FAHA Barbara Riegel, PhD, RN, FAHA
Federico Gentile, MD, FACC Frank W. Sellke, MD, FACC, FAHA§§
Samuel S. Gidding, MD, FAHA§§ Win-Kuang Shen, MD, FACC, FAHA§§
Duminda N. Wijeysundera, MD, PhD
*Writing committee members are required to recuse themselves from voting on sections to which their specific
relationships with industry may apply; see Appendix 1 for recusal information. †ACC/AHA RepresentaƟve.
‡InternaƟonal Society for Adult Congenital Heart Disease RepresentaƟve. §Society for Cardiovascular Angiography
and Interventions Representative. ║ACC/AHA Task Force on Clinical Practice Guidelines Liaison. ¶Society of
Thoracic Surgeons Representative. #American Association for Thoracic Surgery Representative. **ACC/AHA Task

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Force on Performance Measures Liaison. ††American Society of Echocardiography RepresentaƟve. ‡‡Heart
Rhythm Society Representative. §§Former Task Force member; current member during the writing effort.
This document was approved by the American College of Cardiology Clinical Policy Approval Committee in May
2018, the American Heart Association Science Advisory and Coordinating Committee in June 2018, and the
American Heart Association Executive Committee in July 2018.
The American College of Cardiology requests that this document be cited as follows: Stout KK, Daniels CJ,
Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg
MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC guideline for the management of adults with congenital
heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical
Practice Guidelines. J Am Coll Cardiol 2018.
This article has been copublished in Circulation.
Copies: This document is available on the World Wide Web sites of the American College of Cardiology
(www.acc.org) and the American Heart Association (professional.heart.org). For copies of this document, please
contact the Elsevier Inc. Reprint Department via fax (212-633-3820) or e-mail (reprints@elsevier.com).
Permissions: Multiple copies, modification, alteration, enhancement, and/or distribution of this document are not
permitted without the express permission of the American College of Cardiology. Requests may be completed
online via the Elsevier site (http://www.elsevier.com/about/policies/author-agreement/obtaining-permission).
© 2018 by the American Heart Association, Inc., and the American College of Cardiology Foundation.

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Table of Contents
Preamble...........................................................................................................................................5
1. Introduction...................................................................................................................................9
1.1. Methodology and Evidence Review...............................................................................................9
1.2. Organization of the Writing Committee ........................................................................................9
1.3. Document Review and Approval .................................................................................................10
1.4. Scope of the Guideline................................................................................................................10
1.5. Abbreviations .............................................................................................................................13
2. Background and Pathophysiology.................................................................................................14
2.1. Anatomic and Physiological Terms..............................................................................................14
2.2. Severity of ACHD.........................................................................................................................14
2.3. The ACHD Anatomic and Physiological Classification...................................................................18
3. General Principles ........................................................................................................................19
3.1. ACHD Program............................................................................................................................19
3.2. Access to Care.............................................................................................................................21
3.3. Delivery of Care ..........................................................................................................................21
3.4. Evaluation of Suspected and Known CHD....................................................................................26
3.4.1. Electrocardiogram................................................................................................................26
3.4.2. Ionizing Radiation Principles.................................................................................................27
3.4.3. Echocardiography ................................................................................................................27
3.4.4. CMR Imaging........................................................................................................................28
3.4.5. Cardiac Computed Tomography ...........................................................................................29
3.4.6. Cardiac Catheterization........................................................................................................30
3.4.7. Exercise Testing....................................................................................................................31
3.5. Transition Education...................................................................................................................31
3.6. Exercise and Sports.....................................................................................................................32
3.7. Mental Health and Neurodevelopmental Issues..........................................................................34
3.8. Endocarditis Prevention..............................................................................................................34
3.9. Concomitant Syndromes.............................................................................................................35
3.10. Acquired Cardiovascular Disease...............................................................................................36
3.11. Noncardiac Medical Issues........................................................................................................37
3.12. Noncardiac Surgery...................................................................................................................37
3.13. Pregnancy, Reproduction, and Sexual Health ............................................................................39
3.13.1. Pregnancy ..........................................................................................................................39
3.13.2. Contraception ....................................................................................................................41
3.13.3. Infertility Treatment...........................................................................................................42
3.13.4. Sexual Function..................................................................................................................42
3.14. Heart Failure and Transplant.....................................................................................................44
3.14.1. Heart Failure ......................................................................................................................44
3.14.2. Heart Transplant ................................................................................................................44
3.14.3. Multiorgan Transplant........................................................................................................45
3.15. Palliative Care...........................................................................................................................45
3.16. Cyanosis....................................................................................................................................47
3.17. Pharmacological Therapy for ACHD...........................................................................................49
4. Specific Lesions ............................................................................................................................49
4.1. Shunt Lesions..............................................................................................................................49
4.1.1. Atrial Septal Defect ..............................................................................................................50
4.1.2. Anomalous Pulmonary Venous Connections.........................................................................54
4.1.3. Ventricular Septal Defect......................................................................................................56

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4.1.4. Atrioventricular Septal Defect ..............................................................................................59
4.1.5. Patent Ductus Arteriosus......................................................................................................62
4.2. Left-Sided Obstructive Lesions ....................................................................................................64
4.2.1. Cor Triatriatum.....................................................................................................................64
4.2.2. Congenital Mitral Stenosis....................................................................................................65
4.2.3. Subaortic Stenosis................................................................................................................67
4.2.4. Congenital Valvular Aortic Stenosis ......................................................................................68
4.2.4.1. Turner Syndrome ..........................................................................................................70
4.2.4.2. Aortopathies .................................................................................................................71
4.2.5. Supravalvular Aortic Stenosis ...............................................................................................71
4.2.6. Coarctation of the Aorta.......................................................................................................73
4.3. Right-Sided Lesions.....................................................................................................................75
4.3.1. Valvular Pulmonary Stenosis ................................................................................................76
4.3.1.1. Isolated PR After Repair of PS........................................................................................77
4.3.2. Branch and Peripheral Pulmonary Stenosis ..........................................................................80
4.3.3. Double-Chambered Right Ventricle ......................................................................................81
4.3.4. Ebstein Anomaly ..................................................................................................................83
4.3.5. Tetralogy of Fallot ................................................................................................................86
4.3.6. Right Ventricle to Pulmonary Artery Conduit........................................................................91
4.4. Complex Lesions .........................................................................................................................93
4.4.1. Transposition of the Great Arteries ......................................................................................93
4.4.1.1. Transposition of the Great Arteries With Atrial Switch...................................................93
4.4.1.2. Transposition of the Great Arteries With Arterial Switch................................................96
4.4.1.3. Transposition of the Great Arteries With Rastelli Type Repair........................................98
4.4.1.4. Congenitally Corrected Transposition of the Great Arteries ...........................................99
4.4.2. Fontan Palliation of Single Ventricle Physiology (Including Tricuspid Atresia and Double Inlet
Left Ventricle)..............................................................................................................................101
4.4.3. Hypoplastic Left Heart Syndrome/Norwood Repair ............................................................106
4.4.4. Truncus Arteriosus .............................................................................................................106
4.4.5. Double Outlet Right Ventricle.............................................................................................106
4.4.6. Severe PAH and Eisenmenger Syndrome............................................................................106
4.4.6.1. Severe PAH..................................................................................................................107
4.4.6.2. Eisenmenger Syndrome...............................................................................................109
4.4.7. Coronary Anomalies...........................................................................................................111
4.4.7.1 Anomalous Coronary Artery Evaluation........................................................................113
4.4.7.2. Anomalous Aortic Origin of Coronary Artery................................................................114
4.4.7.3. Anomalous Coronary Artery Arising From the PA.........................................................116
4.4.8. Coronary Artery Fistula ......................................................................................................116
5. Evidence Gaps and Future Directions..........................................................................................117
Appendix 1. Author Relationships With Industry and Other Entities (Relevant)................................120
Appendix 2. Reviewer Relationships With Industry and Other Entities (Comprehensive)..................124
References.....................................................................................................................................132

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Preamble
Since 1980, the American College of Cardiology (ACC) and American Heart Association (AHA) have
translated scientific evidence into clinical practice guidelines (guidelines) with recommendations to
improve cardiovascular health. These guidelines, which are based on systematic methods to evaluate
and classify evidence, provide a cornerstone for quality cardiovascular care. The ACC and AHA sponsor
the development and publication of guidelines without commercial support, and members of each
organization volunteer their time to the writing and review efforts. Guidelines are official policy of the
ACC and AHA.
Intended Use
Practice guidelines provide recommendations applicable to patients with or at risk of developing
cardiovascular disease. The focus is on medical practice in the United States, but guidelines developed in
collaboration with other organizations can have a global impact. Although guidelines may be used to
inform regulatory or payer decisions, they are intended to improve patients’ quality of care and align
with patients’ interests. Guidelines are intended to define practices meeting the needs of patients in
most, but not all, circumstances and should not replace clinical judgment.
Clinical Implementation
Management in accordance with guideline recommendations is effective only when followed by both
practitioners and patients. Adherence to recommendations can be enhanced by shared decision-making
between clinicians and patients, with patient engagement in selecting interventions on the basis of
individual values, preferences, and associated conditions and comorbidities.
Methodology and Modernization
The ACC/AHA Task Force on Clinical Practice Guidelines (Task Force) continuously reviews, updates, and
modifies guideline methodology on the basis of published standards from organizations, including the
Institute of Medicine (P-1, P-2), and on the basis of internal reevaluation. Similarly, the presentation and
delivery of guidelines are reevaluated and modified on the basis of evolving technologies and other
factors to facilitate optimal dissemination of information to healthcare professionals at the point of
care.
Toward this goal, this guideline continues the introduction of an evolved format of presenting
guideline recommendations and associated text called the “modular knowledge chunk format.” Each
modular “chunk” includes a table of related recommendations, a brief synopsis, recommendation-
specific supportive text, and when appropriate, flow diagrams or additional tables. References are
provided at the end of the document in their respective sections. Additionally, this format will facilitate
seamless updating of guidelines with focused updates as new evidence is published, as well as content
tagging for rapid electronic retrieval of related recommendations on a topic of interest. This evolved
approach format was instituted when this guideline was near completion; therefore, the present
document represents a transitional format that best suits the text as written. Future guidelines will fully
implement this format, including provisions for limiting the amount of text in a guideline.
Recognizing the importance of cost–value considerations in certain guidelines, when
appropriate and feasible, an analysis of the value of a drug, device, or intervention may be performed in
accordance with the ACC/AHA methodology (P-3).
To ensure that guideline recommendations remain current, new data are reviewed on an
ongoing basis, with full guideline revisions commissioned in approximately 6-year cycles. Publication of
new, potentially practice-changing study results that are relevant to an existing or new drug, device, or
management strategy will prompt evaluation by the Task Force, in consultation with the relevant
guideline writing committee, to determine whether a focused update should be commissioned. For

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additional information and policies regarding guideline development, we encourage readers to consult
the ACC/AHA guideline methodology manual (P-4) and other methodology articles (P-5–P-8).
Selection of Writing Committee Members
The Task Force strives to avoid bias by selecting experts from a broad array of backgrounds. Writing
committee members represent different geographic regions, sexes, ethnicities, races, intellectual
perspectives/biases, and scopes of clinical practice. The Task Force may also invite organizations and
professional societies with related interests and expertise to participate as partners, collaborators, or
endorsers.
Relationships With Industry and Other Entities
The ACC and AHA have rigorous policies and methods to ensure that guidelines are developed without
bias or improper influence. The complete relationships with industry and other entities (RWI) policy can
be found at http://www.acc.org/guidelines/about-guidelines-and-clinical-documents/relationships-with-
industry-policy. Appendix 1 of the present document lists writing committee members’ relevant RWI.
For the purposes of full transparency, writing committee members’ comprehensive disclosure
information is available online
(http://jaccjacc.acc.org/Clinical_Document/ACHD_Exec_Summ_and_Full_Text_ Comp_RWI_Table_08-
02-18.pdf ). Comprehensive disclosure information for the Task Force is available at
http://www.acc.org/guidelines/about-guidelines-and-clinical-documents/guidelines-and-documents-
task-forces.
Evidence Review and Evidence Review Committees
In developing recommendations, the writing committee uses evidence-based methodologies that are
based on all available data (P-4–P-7). Literature searches focus on randomized controlled trials (RCTs)
but also include registries, nonrandomized comparative and descriptive studies, case series, cohort
studies, systematic reviews, and expert opinion. Only key references are cited.
An independent evidence review committee (ERC) is commissioned when there are 1 or more
questions deemed of utmost clinical importance that merit formal systematic review. The systematic
review will determine which patients are most likely to benefit from a drug, device, or treatment
strategy and to what degree. Criteria for commissioning an ERC and formal systematic review include: a)
the absence of a current authoritative systematic review, b) the feasibility of defining the benefit and
risk in a time frame consistent with the writing of a guideline, c) the relevance to a substantial number
of patients, and d) the likelihood that the findings can be translated into actionable recommendations.
ERC members may include methodologists, epidemiologists, healthcare providers, and biostatisticians.
The recommendations developed by the writing committee on the basis of the systematic review are
marked with “SR”.
Guideline-Directed Management and Therapy
The term guideline-directed management and therapy (GDMT) encompasses clinical evaluation,
diagnostic testing, and pharmacological and procedural treatments. For these and all recommended
drug treatment regimens, the reader should confirm the dosage by reviewing product insert material
and evaluate the treatment regimen for contraindications and interactions. The recommendations are
limited to drugs, devices, and treatments approved for clinical use in the United States.
Class of Recommendation and Level of Evidence
The Class of Recommendation (COR) indicates the strength of the recommendation, encompassing the
estimated magnitude and certainty of benefit in proportion to risk. The Level of Evidence (LOE) rates the
quality of scientific evidence that supports the intervention on the basis of the type, quantity, and
consistency of data from clinical trials and other sources (Table 1) (P-4–P-6).

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Glenn N. Levine, MD, FACC, FAHA
Chair, ACC/AHA Task Force on Clinical Practice Guidelines

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Table 1. Applying Class of Recommendation and Level of Evidence to Clinical Strategies, Interventions,
Treatments, or Diagnostic Testing in Patient Care* (Updated August 2015)

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1. Introduction
1.1. Methodology and Evidence Review
The recommendations listed in this guideline are, whenever possible, evidence-based. An initial
extensive evidence review, which included literature derived from research involving human subjects,
published in English, and indexed in MEDLINE (through PubMed), EMBASE, the Cochrane Library, the
Agency for Healthcare Research and Quality, and other selected databases relevant to this guideline,
was conducted from April 2014 to November 2014. Key search words included but were not limited to
the following: adult congenital heart disease, anesthesia, aortic aneurysm, aortic stenosis, atrial septal
defect, arterial switch operation, bradycardia, bicuspid aortic valve, cardiac catheterization, cardiac
imaging, cardiovascular magnetic resonance, cardiac reoperation, cardiovascular surgery, chest x-ray,
cirrhosis, coarctation of the aorta, congenital heart defects, congenitally corrected transposition of the
great arteries, contraception, coronary artery abnormalities, cyanotic congenital heart disease, dextro-
transposition of the great arteries, double inlet left ventricle, Ebstein anomaly, echocardiography,
Eisenmenger syndrome, electrocardiogram, endocarditis, exercise test, Fontan, heart catheterization,
heart defect, heart failure, infertility, l-transposition of the great arteries, medical therapy, myocardial
infarction, noncardiac surgery, patent ductus arteriosus, perioperative care, physical activity,
postoperative complications, pregnancy, preoperative assessment, psychosocial, pulmonary arterial
hypertension, hypoplastic left heart syndrome, pulmonary regurgitation, pulmonary stenosis, pulmonary
valve replacement, right heart obstruction, right ventricle to pulmonary artery conduit, single ventricle,
supravalvular pulmonary stenosis, surgical therapy, tachyarrhythmia, tachycardia, tetralogy of Fallot,
transplantation, tricuspid atresia, Turner syndrome, and ventricular septal defect. Additional relevant
studies published through January 2018, during the guideline writing process, were also considered by
the writing committee, and added to the evidence tables when appropriate. The final evidence tables,
included in the Online Data Supplement, summarize the evidence used by the writing committee to
formulate recommendations. References selected and published in this document are representative
and not all-inclusive.
As noted in the preamble, an independent ERC was commissioned to perform a formal
systematic review of critical clinical questions related to adult congenital heart disease (ACHD), the
results of which were considered by the writing committee for incorporation into this guideline.
Concurrent with this process, writing committee members evaluated study data relevant to the rest of
the guideline. The findings of the ERC and the writing committee members were formally presented and
discussed, and then recommendations were developed. The systematic review reports on “Medical
Therapy for Systemic Right Ventricles: A Systematic Review (Part 1) for the 2018 AHA/ACC Guideline for
the Management of Adults With Congenital Heart Disease” (S1.1-1) and “Interventional Therapy Versus
Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC
Guideline for the Management of Adults With Congenital Heart Disease” (S1.1-2) are published in
conjunction with this guideline.
1.2. Organization of the Writing Committee
The writing committee consisted of pediatric and adult congenital cardiologists, interventional
cardiologists, electrophysiologists, surgeons, and an advance practice nurse. The writing committee
included representatives from the ACC, AHA, and American Association for Thoracic Surgery (AATS),
American Society of Echocardiography (ASE), Heart Rhythm Society (HRS), International Society for Adult
Congenital Heart Disease (ISACHD), Society for Cardiovascular Angiography and Interventions (SCAI), and
the Society of Thoracic Surgeons (STS).

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1.3. Document Review and Approval
This document was reviewed by 3 official reviewers each nominated by the ACC and AHA, and 1 to 2
reviewers each from the AATS, ASE, HRS, ISACHD, SCAI, STS; and 32 individual content reviewers.
Reviewers’ RWI information was distributed to the writing committee and is published in this document
(Appendix 2).
This document was approved for publication by the governing bodies of the ACC and the AHA
and endorsed by the AATS, ASE, HRS, ISACHD, SCAI, and STS.
1.4. Scope of the Guideline
The 2018 ACHD guideline is a full revision of the “2008 ACC/AHA Guidelines for the Management of
Adults with Congenital Heart Disease” (S1.4-1), which was the first U.S. guideline to be published on the
topic. This revision uses the 2008 ACHD guideline as a framework and incorporates new data and
growing ACHD expertise to develop recommendations. Congenital heart disease (CHD) encompasses a
range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac
development but does not include inherited disorders that may have cardiac manifestations such as
Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as
patent foramen ovale. Valvular heart disease (VHD) may be congenital, so management overlaps with
the “2014 AHA/ACC Guidelines for the Management of Patients With Valvular Heart Disease” (S1.4-2),
particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the
diagnosis and treatment of congenital valve disease when it differs from acquired valve disease,
whether because of anatomic differences, presence of concomitant lesions, or differences to consider
given the relatively young age of patients with ACHD. This guideline is not intended to apply to children
(<18 years of age) with CHD or adults with acquired VHD, heart failure (HF), or other cardiovascular
diseases.
The prevalence of ACHD is growing because of the success of pediatric cardiology and congenital
cardiac surgery in diagnosing and treating congenital heart defects in children. Improved survival to
adulthood is most striking for those with the most severe disease, with survival to age 18 years now
expected for 90% of children diagnosed with severe CHD (S1.4-3–S1.4-5). Patients with ACHD are a
heterogeneous population, both in underlying anatomy and physiology, as well as surgical repair or
palliation. Consequently, although the prevalence of ACHD is increasing, the population of patients with
a given congenital abnormality or specific repair may be relatively small (S1.4-3, S1.4-6–S1.4-8).
Patients with CHD are not cured of their disease after successful treatment in childhood. Almost
all patients with ACHD will have sequelae of either their native CHD or its surgical repair or palliation,
although these sequelae can take decades to manifest. The heterogeneity of the population and the
long, symptom-free intervals constrain the ability to generate data applicable across the population of
ACHD or to adults with specific lesions or repairs. Despite the difficulty in studying ACHD populations,
there is a growing body of high-quality data in these patients to guide the care of this relatively “new”
population and, whenever feasible, these data were used to develop recommendations.
Recommendations are made based on the available data; however, when important clinical issues
lacked data, first principles, extrapolation from data in other populations, and expert consensus are
used to guide care. Patients with ACHD may have concomitant disease to which other existing guidelines
apply, such as coronary artery disease, HF, and arrhythmias. The data from acquired heart disease
populations may apply to some patients with ACHD, and those circumstances are acknowledged in these
recommendations and referenced accordingly.
Patients with ACHD who are cared for in ACHD centers have better outcomes than those cared
for in centers without ACHD expertise (S1.4-9), and this need for specialized care is noted throughout

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the guideline. These recommendations are intended to provide guidance to a wide variety of providers
caring for patients with ACHD, including general, pediatric, and ACHD cardiologists, as well as surgeons,
primary care providers, and other healthcare providers.
In developing the 2018 ACHD guideline, the writing committee reviewed previously published
guidelines and related scientific statements. Table 2 contains a list of publications and scientific
statements deemed pertinent to this writing effort; it is intended for use as a resource and does not
repeat existing guideline recommendations.
Table 2. Associated Guidelines and Statements
Title Organization
Publication Year
(Reference)
Guidelines
Syncope ACC/AHA/HRS 2017 (S1.4-10)
Supraventricular tachycardia ACC/AHA/HRS 2015 (S1.4-11)
Cardiopulmonary resuscitation and
emergency cardiovascular care—Part 8:
postcardiac arrest care
AHA 2015 (S1.4.12)
Non-ST-elevation acute coronary syndromes AHA/ACC 2014 (S1.4-13)
Perioperative cardiovascular evaluation and
noncardiac surgery
ACC/AHA 2014 (S1.4-14)
Atrial fibrillation AHA/ACC/HRS 2014 (S1.4-15)
Stable ischemic heart disease ACC/AHA/ACP/AATS/PCNA/SCAI/STS 2014 (S1.4-16),
2012 (S1.4-17)
Assessment of cardiovascular risk ACC/AHA 2014 (S1.4-18)
Blood cholesterol to reduce atherosclerotic
cardiovascular risk in adults
ACC/AHA 2014 (S1.4-19)
Overweight and obesity in adults AHA/ACC/TOS 2014 (S1.4-20)
Lifestyle management to reduce
cardiovascular risk
AHA/ACC 2014 (S1.4-21)
Valvular heart disease AHA/ACC 2017 (S1.4-22)
High blood pressure in adults ACC/AHA/AAPA/ABC/ACPM/AGS/
APhA/ASH/ASPC/NMA/PCNA
2017 (S1.4-23)
Aortic valve and ascending aorta STS 2013 (S1.4-24)
ST-elevation myocardial infarction ACC/AHA 2013 (S1.4-25)
Heart failure ACC/AHA/HFSA 2017 (S1.4-26)
Device-based therapy for cardiac rhythm
abnormalities
ACC/AHA/HRS 2012 (S1.4-27)
Coronary artery bypass graft surgery ACC/AHA 2011 (S1.4-28)
Percutaneous coronary intervention ACC/AHA/SCAI 2011 (S1.4-29)
Secondary prevention and risk reduction
therapy
AHA/ACC 2011 (S1.4-30)
Cardiovascular disease in women AHA/ACC 2011 (S1.4-31)
Grown-up congenital heart disease ESC 2010 (S1.4-32)
Thoracic aortic disease ACC/AHA/AATS/ACR/ASA/SCA/
SCAI/SIR/STS/SVM
2010 (S1.4-33)
Adult congenital heart disease CCS 2010 (S1.4-34)
Infective endocarditis ESC 2009 (S1.4-35)

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Scientific statements
Imaging for patients with transposition of
the great arteries
ASE 2016 (S1.4-36)
Cardiac chamber quantification by
echocardiography
ASE 2015 (S1.4-37)
Consensus on arrhythmia management in
ACHD
PACES/HRS 2014 (S1.4-38)
Imaging for patients with repaired tetralogy
of Fallot
ASE 2014 (S1.4-39)
Thoracic aortic disease CCS 2014 (S1.4-40)
Promotion of physical activity for children
and adults with CHD
AHA 2013 (S1.4-41)
Neurodevelopmental outcomes in children
with CHD
AHA 2012 (S1.4-42)
Pregnancy in women with heart disease ESC 2011 (S1.4-43)
Transition to adulthood for adolescents with
CHD
AHA 2011 (S1.4-44)
Pulmonary hypertension ACC/AHA 2009 (S1.4-45)
Prevention of infective endocarditis AHA 2007 (S1.4-46)
AATS indicates American Association for Thoracic Surgery; ABC, Association of Black Cardiologists; ACC, American College of
Cardiology; ACHD, adult congenital heart disease; ACP, American College of Physicians; ACPM, American College of Preventive
Medicine; ACR, American College of Radiology; AGS, American Geriatrics Society; AHA, American Heart Association; APhA,
American Pharmacists Association; ASA, American Stroke Association; ASE, American Society of Echocardiography; ASH,
American Society of Hypertension; ASPC, American Society of Preventive Cardiology; CCS, Canadian Cardiovascular Society;
CHD, congenital heart disease; ESC, European Society of Cardiology; HFSA, Heart Failure Society of America; HRS, Heart Rhythm
Society; NMA, National Medical Association; PACES, Pediatric and Congenital Electrophysiology Society; PCNA, Preventive
Cardiovascular Nurses Association; SCA, Society of Cardiovascular Anesthesiologists; SCAI, Society for Cardiovascular
Angiography and Interventions; SIR, Society of Interventional Radiology; STS, Society of Thoracic Surgeons; SVM, Society for
Vascular Medicine; and TOS, The Obesity Society.

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1.5. Abbreviations
Abbreviation Meaning/Phrase
3D 3-dimensional
AAOCA anomalous aortic origin of the coronary artery
ACHD adult congenital heart disease
AP anatomic and physiological
AR aortic regurgitation
ASD atrial septal defect
AVSD atrioventricular septal defect
BAV bicuspid aortic valve
CCT cardiac computed tomography
CCTGA congenitally corrected transposition of the great arteries
CHD congenital heart disease
CMR cardiovascular magnetic resonance
CoA coarctation of the aorta
CPET cardiopulmonary exercise test
CT computed tomography
CTA computed tomography angiography
d-TGA dextro-transposition of the great arteries
ECG electrocardiogram
ERC evidence review committee
GDMT guideline-directed management and therapy
HF heart failure
ICD implantable cardioverter-defibrillator
IE infective endocarditis
LV left ventricular
LVOT left ventricular outflow tract
PA pulmonary artery
PAH pulmonary arterial hypertension
PDA patent ductus arteriosus
PR pulmonary regurgitation
PS pulmonary stenosis
QoL quality of life
Qp:Qs pulmonary–systemic blood flow ratio
RCT randomized controlled trial
RV right ventricular
RVOT right ventricular outflow tract
SCD sudden cardiac death
SubAS subaortic stenosis
TEE transesophageal echocardiography
TGA transposition of the great arteries
TOF tetralogy of Fallot
TR tricuspid regurgitation
TTE transthoracic echocardiography
VHD valvular heart disease
VSD ventricular septal defect
VT ventricular tachycardia

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2. Background and Pathophysiology
2.1. Anatomic and Physiological Terms
The International Society for Nomenclature of Pediatric and Congenital Heart Disease (also known as the
Nomenclature Working Group) defined, codified, mapped, and archived examples of nomenclatures and
developed standards for terminology (S2.1-1– S2.1-5). The International Paediatric and Congenital
Cardiac Code (IPCCC) nomenclature for anatomic lesions and repairs is used in this guideline
(http://ipccc.net) (S2.1-6).
2.2. Severity of ACHD
In a patient with CHD, severity of disease is determined by native anatomy, surgical repair, and current
physiology. Prior documents, including the 2008 ACHD guideline (S2.2-1), relied primarily on anatomic
classifications to rank severity of disease. However, patients with the same underlying anatomy may
have very different repairs and experienced variable physiological consequences of those repairs. For
example, a patient with tetralogy of Fallot (TOF) after a valve-sparing primary repair may have excellent
biventricular function with normal exercise capacity and no arrhythmias, whereas another patient of the
same age with TOF may have had palliative shunting followed by a transannular patch repair resulting in
severe pulmonary regurgitation (PR) with right ventricular (RV) enlargement, biventricular dysfunction,
and ventricular tachycardia (VT). To categorize disease severity in CHD in a more comprehensive way,
the writing committee developed an ACHD Anatomic and Physiological (AP) classification system (Tables
3 and 4) that incorporates the previously described CHD anatomic variables as well as physiological
variables, many of which have prognostic value in patients with ACHD.
Table 3. Physiological Variables as Used in ACHD AP Classification
Variable Description
Aortopathy Aortic enlargement is common in some types of CHD and after some repairs.
Aortic enlargement may be progressive over a lifetime. There is no universally
accepted threshold for repair, nor is the role of indexing to body size clearly
defined in adults, as is done in pediatric populations. For purposes of
categorization and timing of follow-up imaging (S2.2-2–S2.2-4):
• Mild aortic enlargement is defined as maximum diameter 3.5–3.9 cm
• Moderate aortic enlargement is defined as maximum diameter 4.0–4.9
cm
• Severe aortic enlargement is defined as maximum diameter ≥5.0 cm
Arrhythmia Arrhythmias are very common in patients with ACHD and may be both the
cause and consequence of deteriorating hemodynamics, valvular dysfunction,
or ventricular dysfunction. Arrhythmias are associated with symptoms,
outcomes, and prognosis (S2.2-5–S2.2-8), thus are categorized based on
presence and response to treatment.
• No arrhythmia: No documented clinically relevant atrial or ventricular
tachyarrhythmias
• Arrhythmia not requiring treatment: Bradyarrhythmia, atrial or
ventricular tachyarrhythmia not requiring antiarrhythmic therapy,
cardioversion, or ablation
• Arrhythmia controlled with therapy:

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o Bradyarrhythmia requiring pacemaker implantation
o Atrial or ventricular tachyarrhythmia requiring antiarrhythmic
therapy, cardioversion, or ablation
o AF and controlled ventricular response
o Patients with an ICD
• Refractory arrhythmias:
o Atrial or ventricular tachyarrhythmia currently unresponsive to or
refractory to antiarrhythmic therapy or ablation
Concomitant VHD Severity defined according to the 2014 VHD guideline (S2.2-2).
• Mild VHD
• Moderate VHD
• Severe VHD
End-organ dysfunction Clinical and/or laboratory evidence of end-organ dysfunction (S2.2-9–S2.2-11)
including:
• Renal (kidney)
• Hepatic (liver)
• Pulmonary (lung)
Exercise capacity Patients with ACHD are often asymptomatic notwithstanding exercise
limitations demonstrated as diminished exercise capacity when evaluated
objectively (S2.2-12–S2.2-14). Thus, assessment of both subjective and
objective exercise capacity is important (see NYHA classification system
below). Exercise capacity is associated with prognosis (S2.2-15–S2.2-17).
• Abnormal objective cardiac limitation to exercise is defined as an
exercise maximum ventilatory equivalent of oxygen below the range
expected for the specific CHD anatomic diagnosis (S2.2-18).
• Expected norms for CPET values should take into account age, sex, and
underlying congenital diagnosis. Published studies with institution-
specific norms can be used as guides, bearing in mind variability among
institutional norms and ranges.
Hypoxemia/hypoxia/cyanosis See Section 3.16. for detailed definition of cyanosis.
• Hypoxemia is defined as oxygen saturation measured by pulse oximetry
at rest ≤90%.
• Severe hypoxemia is defined as oxygen saturation at rest <85%.
• In patients with normal or high hemoglobin concentrations, severe
hypoxemia will be associated with visible cyanosis (which requires ≥5
g/L desaturated hemoglobin to be appreciated).
• The terms cyanosis and hypoxemia (or hypoxia) are sometimes used
interchangeably. Such interchangeability would not apply; however, in
the presence of anemia, severe hypoxemia can be present without
visible cyanosis.
NYHA functional
classification system (S2.2-
19)
Class Functional Capacity
I Patients with cardiac disease but resulting in no limitation of physical
activity. Ordinary physical activity does not cause undue fatigue,
palpitation, dyspnea, or anginal pain.
II Patients with cardiac disease resulting in slight limitation of physical
activity. They are comfortable at rest. Ordinary physical activity results
in fatigue, palpitation, dyspnea, or anginal pain.
III Patients with cardiac disease resulting in marked limitation of physical
activity. They are comfortable at rest. Less than ordinary activity
causes fatigue, palpitation, dyspnea, or anginal pain.
IV Patients with cardiac disease resulting in inability to carry on any
physical activity without discomfort. Symptoms of HF or the anginal

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syndrome may be present even at rest. If any physical activity is
undertaken, discomfort increases.
Pulmonary hypertension Pulmonary hypertension is a broad term that encompasses pulmonary arterial
hypertension, which is pulmonary hypertension with increased pulmonary
vascular resistance. This document defines PH and PAH as they are used in the
field of pulmonary hypertension.
Pulmonary hypertension is defined as:
• Mean PA pressure by right heart catheterization ≥25 mm Hg.
PAH is defined as:
• Mean PA pressure by right heart catheterization ≥25 mm Hg and a
pulmonary capillary wedge pressure ≤15 mm Hg and pulmonary vascular
resistance ≥3 Wood units (S2.2-20)
Shunt (hemodynamically
significant shunt)
An intracardiac shunt is hemodynamically significant if:
• There is evidence of chamber enlargement distal to the shunt
• And/or evidence of sustained Qp:Qs ≥1.5:1
• An intracardiac shunt not meeting these criteria would be described as
small or trivial
Venous and arterial stenosis • Aortic recoarctation after CoA repair
• Supravalvular aortic obstruction
• Venous baffle obstruction
• Supravalvular pulmonary stenosis
• Branch PA stenosis
• Stenosis of cavopulmonary connection
• Pulmonary vein stenosis
ACHD indicates adult congenital heart disease; AF, atrial fibrillation; AP, anatomic and physiological; CHD, congenital heart
disease; CoA, coarctation of the aorta; CPET, cardiopulmonary exercise test; HF, heart failure; ICD, implantable cardioverter-
defibrillator; NYHA, New York Heart Association; PA, pulmonary artery; PAH, pulmonary arterial hypertension; Qp:Qs,
pulmonary–systemic blood flow ratio; and VHD, valvular heart disease.
Table 4. ACHD AP Classification
(CHD Anatomy + Physiological Stage = ACHD AP Classification)
CHD Anatomy*
I: Simple
Native disease
• Isolated small ASD
• Isolated small VSD
• Mild isolated pulmonic stenosis
Repaired conditions
• Previously ligated or occluded ductus arteriosus
• Repaired secundum ASD or sinus venosus defect without
significant residual shunt or chamber enlargement
• Repaired VSD without significant residual shunt or chamber
enlargement
II: Moderate Complexity
Repaired or unrepaired conditions
• Aorto-left ventricular fistula
• Anomalous pulmonary venous connection, partial or total
• Anomalous coronary artery arising from the pulmonary artery
• Anomalous aortic origin of a coronary artery from the opposite
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• AVSD (partial or complete, including primum ASD)
• Congenital aortic valve disease
• Congenital mitral valve disease
• Coarctation of the aorta
• Ebstein anomaly (disease spectrum includes mild, moderate,
and severe variations)
• Infundibular right ventricular outflow obstruction
• Ostium primum ASD
• Moderate and large unrepaired secundum ASD
• Moderate and large persistently patent ductus arteriosus
• Pulmonary valve regurgitation (moderate or greater)
• Pulmonary valve stenosis (moderate or greater)
• Peripheral pulmonary stenosis
• Sinus of Valsalva fistula/aneurysm
• Sinus venosus defect
• Subvalvar aortic stenosis (excluding HCM; HCM not addressed
in these guidelines)
• Supravalvar aortic stenosis
• Straddling atrioventricular valve
• Repaired tetralogy of Fallot
• VSD with associated abnormality and/or moderate or greater
shunt
III: Great Complexity (or Complex)
• Cyanotic congenital heart defect (unrepaired or palliated, all
forms)
• Double-outlet ventricle
• Fontan procedure
• Interrupted aortic arch
• Mitral atresia
• Single ventricle (including double inlet left ventricle, tricuspid
atresia, hypoplastic left heart, any other anatomic abnormality
with a functionally single ventricle)
• Pulmonary atresia (all forms)
• TGA (classic or d-TGA; CCTGA or l-TGA)
• Truncus arteriosus
• Other abnormalities of atrioventricular and ventriculoarterial
connection (i.e., crisscross heart, isomerism, heterotaxy
syndromes, ventricular inversion)
Physiological Stage
A
• NYHA FC I symptoms
• No hemodynamic or anatomic sequelae
• No arrhythmias
• Normal exercise capacity
• Normal renal/hepatic/pulmonary function
B
• NYHA FC II symptoms
• Mild hemodynamic sequelae (mild aortic enlargement, mild
ventricular enlargement, mild ventricular dysfunction)
• Mild valvular disease

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• Trivial or small shunt (not hemodynamically significant)
• Arrhythmia not requiring treatment
• Abnormal objective cardiac limitation to exercise
C
• NYHA FC III symptoms
• Significant (moderate or greater) valvular disease; moderate or
greater ventricular dysfunction (systemic, pulmonic, or both)
• Moderate aortic enlargement
• Venous or arterial stenosis
• Mild or moderate hypoxemia/cyanosis
• Hemodynamically significant shunt
• Arrhythmias controlled with treatment
• Pulmonary hypertension (less than severe)
• End-organ dysfunction responsive to therapy
D
• NYHA FC IV symptoms
• Severe aortic enlargement
• Arrhythmias refractory to treatment
• Severe hypoxemia (almost always associated with cyanosis)
• Severe pulmonary hypertension
• Eisenmenger syndrome
• Refractory end-organ dysfunction
*This list is not meant to be comprehensive; other conditions may be important
in individual patients.
ACHD indicates adult congenital heart disease; AP, anatomic and physiological;
ASD, atrial septal defect; AVSD, atrioventricular septal defect; CCTGA,
congenitally corrected transposition of the great arteries; CHD, congenital heart
disease; d-TGA, dextro-transposition of the great arteries; FC, functional class;
HCM, hypertrophic cardiomyopathy; l-TGA, levo-transposition of the great
arteries; NYHA, New York Heart Association; TGA, transposition of the great
arteries; and VSD, ventricular septal defect.
2.3. The ACHD AP Classification
The ACHD AP classification (Tables 3 and 4), newly elaborated in this guideline, is intended to capture
the complexity of ACHD anatomy and physiology, which are not always correlated. Certain anatomic
abnormalities of clinical importance are shared across diagnoses (e.g., aortic enlargement), which may
be found in patients with BAV, coarctation of the aorta (CoA), transposition of the great arteries, and
TOF, amongst others. In every patient, anatomic and physiological variables should be considered. In
using Tables 3 and 4, a patient should be classified based on the “highest” relevant anatomic or
physiological feature. For example, a normotensive patient with repaired CoA, normal exercise capacity,
and normal end-organ function would be ACHD AP classification IIA, whereas an otherwise similar
patient with ascending aortic diameter of 4.0 cm would be ACHD AP classification IIB, and if moderate
aortic stenosis were also present, the ACHD AP classification would be IIC.
Patients with ACHD may have baseline exercise limitations, cyanosis, end-organ dysfunction, or
other clinically important comorbidities related to their CHD. They are also at risk of HF, arrhythmias,
sudden cardiac death (SCD), and development or progression of cardiac symptoms such as dyspnea,
chest pain, and exercise intolerance. Concomitant valvular disease or aortic pathology may be present.

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There are growing data regarding the prognostic implications of these variables in patients with ACHD,
but not the abundance of data available for patients with acquired heart disease (S2.3-1–S2.3-16).
The variables forming part of the ACHD AP classification (Table 3) were selected because data
exist suggesting their importance in prognosis, management, or quality of life (QoL). As new data
become available, we expect changes in the relative weights attributed to the components of the ACHD
AP classification and perhaps new components, resulting in a scheme that ever more precisely tracks
overall severity of disease and need for more or less intensive follow-up and management.
Similar to the New York Heart Association (NYHA) classification of functional status, patients
may move from one ACHD AP classification to another over time. If clinical status worsens, the
classification will change to a higher severity group, but improvement in status, for example after an
intervention such as valve replacement or control of arrhythmia, can result in change to a lower severity
classification. Such movement among classes is unlike the AHA HF A to D classification (S2.3-17), in
which patients move in only one direction. This ACHD AP classification is used throughout this
document, particularly when considering follow-up visits and need for testing. As the ACHD AP
classification worsens because of changes in physiology (e.g., development of arrhythmias, HF, end-
organ disease), the nature and frequency of recommended follow-up visits and testing will also change,
adapting to the patient’s changing circumstance instead of depending solely on a description of
anatomic disease, which may not adequately discriminate physiological changes that alter severity over
time.
Some patients with ACHD may have substantial acquired comorbidities unrelated to CHD and, as
a consequence, their follow-up strategies might be more appropriately based on other existing
guidelines for acquired heart disease. For example, an 80-year-old patient who has a small atrial septal
defect (ASD), but whose symptoms are related to diastolic HF, chronic kidney disease caused by
hypertension and diabetes mellitus, and moderate aortic stenosis is well-suited to be followed according
to existing guidelines for those diseases, rather than according to the ACHD AP classification for the ASD.
Nevertheless, the added hemodynamic complexity brought by the ASD must be kept in mind.
Throughout this document, the ACHD AP classification is used to help guide resource utilization,
including ACHD consultation and routine diagnostic studies.
3. General Principles
See Online Data Supplements 1 and 2 for additional data supporting this section.
3.1. ACHD Program
Patients with complex CHD have generally better outcomes when cared for in an integrated,
collaborative, and multidisciplinary program (S3.1-1). Many medical issues in patients with ACHD involve
cardiac sequelae, and the diagnosis and management may require cardiac anesthesiologists,
electrophysiologists, and interventional cardiologists; imaging services such as cardiovascular magnetic
resonance (CMR)/cardiac computed tomography (CCT); and pulmonary hypertension services with
expertise in ACHD (Table 5). Appropriate specialty care must be available to address pregnancy,
acquired cardiovascular disease, and acute noncardiac illness complicating CHD, management of which
is frequently more complicated in patients with ACHD.

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Although individual providers may be community-based affiliates, ACHD programs are inpatient,
outpatient, and hospital-based with staffing and expertise available on-site or accessible when needed
(Table 5).
Table 5. Key Personnel and Services Recommended for ACHD Programs
Personnel
ACHD board-eligible/board-certified cardiologists
Congenital cardiac surgeons
Nurses/physician assistants/nurse practitioners
Cardiac anesthesiologists with CHD training/expertise
Multidisciplinary teams:
• High-risk obstetrics
• Pulmonary hypertension
• HF/transplant
• Genetics
• Hepatology
• Cardiac pathology
• Rehabilitation services
• Social services
• Psychological services
• Financial counselors
Services
Echocardiography, including TEE and intraoperative TEE*
CHD diagnostic and interventional catheterization*
CHD electrophysiology/pacing/ICD implantation*:
• Exercise testing
• Echocardiographic
• Radionuclide
• Cardiopulmonary
Cardiac imaging/radiology*:
• CMR
• CCT
• Nuclear medicine
Information technology:
• Data collection
• Database support
• Quality assessment review/protocols
*These modalities must be supervised/performed and interpreted by clinicians with
expertise and/or training in CHD.
ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CHD,
congenital heart disease; CMR, cardiovascular magnetic resonance; HF, heart failure; ICD,
implantable cardioverter-defibrillator; and TEE, transesophageal echocardiography.

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3.2. Access to Care
Recommendation for Access to Care
Referenced studies that support the recommendation are summarized in Online Data Supplement 3.
COR LOE Recommendation
I
B-NR 1. Physicians caring for patients with ACHD should support access to care by
a) assuring smooth transitions for adolescents and young adults from
pediatric to adult providers (S3.2-1, S3.2-2) (Level of Evidence: B-NR); and
b) promoting awareness of the need for lifelong specialized care through
outreach and educational programs (Level of Evidence: C-EO).
C-EO
Synopsis
As patients with ACHD grow beyond the pediatric age group, continued access to specialized
cardiovascular care presents several challenges:
• Lack of guided transfer from pediatric to adult care;
• Insufficient availability of ACHD programs;
• Inadequate insurance coverage;
• Deficient education of patients and caregivers regarding ACHD;
• Inadequate resources for patients with cognitive or psychosocial impairment;
• Lack of comprehensive case management; and
• Different needs for evaluation and management compared with adults with acquired
cardiovascular disease.
Recommendation-Specific Supportive Text
1. Many patients with CHD face gaps in care during and after adolescence (S3.2-2). Common reasons
include lack of knowledge regarding need for follow-up, inability to find specialized providers, insurance
issues, and feeling well (S3.2-1). Patients with gaps in care are more likely to develop medical problems
requiring intervention than those receiving continuous care (S3.2-3, S3.2-4). Canadian patients with CHD
in specialized care programs had lower mortality than those in centers without ACHD expertise (S3.2-5).
Improving transition programs and recognizing the importance of long-term care will hopefully improve
access to specialty care. Insurance barriers and lack of specialty providers for the large number of
patients are issues; thus, relationships with regulatory agencies to address these challenges are
important.
3.3. Delivery of Care
Recommendations for Delivery of Care
Referenced studies that support recommendations are summarized in Online Data Supplements 3, 4,
and 5.
COR LOE Recommendations
I B-NR
1. Patients with ACHD AP classification IB-D, IIA-D, and IIIA-D* should be
managed in collaboration with an ACHD cardiologist (S3.3-1).
I C-LD
2. Cardiac surgery, catheter-based interventional cardiac procedures, and
electrophysiological procedures involving congenital heart lesions in
patients with ACHD should be performed by operators with expertise in
CHD procedures and in collaboration with an ACHD cardiologist (S3.3-1,
S3.3-2).

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*See Tables 3 and 4 for details on the ACHD Anatomic and Physiological classification system.
1. Patients with ACHD, particularly those with more severe CHD, cared for in specialized centers have
lower mortality than those managed without specialized care (S3.3-1). Although clinical practice
guidelines can be helpful, many management decisions for patients with ACHD must be based on
insufficient data or care guidelines and require clinical experience often involving multiple members of
an ACHD team. Patients with complex anatomic and physiological forms of ACHD may need approaches
to evaluation and treatment that differ from those applicable to adults without ACHD who have valve
disease, HF, or arrhythmias.
From a practical perspective, it may be difficult to identify clinicians with expertise in ACHD, and
expertise in ACHD varies across medical and surgical specialties. Some specialties, such as cardiology and
congenital heart surgery, have defined ACHD fellowship training and board certification, whereas for
others, ACHD expertise is gained by focused experience during training and practice.
In 2012, the American Board of Medical Specialties approved ACHD as a subspecialty of internal
medicine (“adult”) cardiology and pediatric cardiology. Therefore, for cardiologists, one marker of ACHD
expertise is board eligibility/board certification in ACHD. There are expert ACHD clinicians who are not
board-certified, including those whose expertise was acquired before the development of formal
certification programs and those trained outside the United States who may also have different
pathways to achieve ACHD expertise. Expertise in the surgical management of patients with ACHD may
be identified through board eligibility/board certification in congenital heart surgery. There are expert
ACHD surgeons who are not board-certified, including those surgeons trained in other countries who are
not eligible for certification in the United States.
Specific ACHD training options are not generally available for cardiac anesthesiologists, but
many of them develop expertise through training in pediatric anesthesiology, cardiac anesthesiology,
mentoring, and practice experience. Other providers involved in the care of patients with ACHD (e.g.,
obstetricians, pulmonologists, radiologists, nurse practitioners, physician assistants) derive expertise
from training and/or practice. Individual providers may gain ACHD expertise in a specific area or
discipline, such as intraoperative transesophageal echocardiography (TEE) or interpretation of CMR.
2. Patients with ACHD who are undergoing invasive cardiovascular procedures in specialized ACHD
centers generally have better outcomes, including survival, than those managed in other care settings
(S3.3-2). Special attention is required to ensure appropriate periprocedural care, including identification
of procedure-related risk factors and availability of ancillary imaging (S3.3-3–S3.3-10).
Table 6 addresses delivery of care where circumstances of ACHD expertise may improve patient
outcomes.

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Table 6. Delivery of Care: Circumstances Where ACHD Expertise May Improve Outcomes
Circumstance Possible Solution Rationale Example
Care of patients in
the lowest ACHD AP
classification (IA)*
1. Face-to-face consultation
with an ACHD cardiologist.
2. Collaborative care planning
between an ACHD patient’s
general cardiologist or primary
care provider and an ACHD
cardiologist.
1. Patients in ACHD AP classification IA* are likely to
be asymptomatic and not require frequent routine
congenital cardiac care.
2. The very long-term outcomes of patients with
ACHD AP classification IA* lesions have not been
well described, although available data suggest that
patients with simple CHD have higher cardiac
mortality in long-term follow-up than age-matched
controls (S3.3-11).
3. Consultation with an ACHD cardiologist should
help to accurately assess the patient’s ACHD AP
class, provide information regarding potential long-
term outcomes, and reinforce signs and symptoms
that should prompt further evaluation.
Patients with small VSDs are thought to have
excellent long-term survival, although
complications (double-chamber RV, IE,
aortic valve prolapse and aortic
regurgitation) may manifest in adulthood;
consequently, patients with small VSDs
warrant lifelong follow-up (S3.3-12).
Cardiac imaging of
patients with ACHD
Imaging studies should be
performed and interpreted by
individuals with expertise in
CHD imaging.
1. The complexity and variability of lesions, repairs,
and sequelae in CHD constrain the use of standard
protocols and sequences and often require
modification of plans during acquisition of images,
as well as specialized skills in interpretation. Thus,
CHD expertise is helpful for optimal quality and
interpretation of cardiac imaging studies.
2. Use of a multimodality cardiac imaging approach
can be used for patients with ACHD, accounting for
patient-specific considerations, strengths and
weaknesses of each modality, institutional
resources, and expertise.
3. ACHD programs need a dedicated CMR service,
and CMR expertise is integral to an ACHD program,
as is expertise in ACHD CCT (S3.3-13, S3.3-14).
Although imaging of a patient with TOF may
seem straightforward because many have
familiar chamber and great vessel
relationships, there are nuances to
echocardiographic imaging of RV size and
function, PR severity, and/or location of
right ventricular outflow tract obstruction
that affect clinical care and are thus best
carried out by sonographers and
echocardiographers with appropriate
expertise. Similarly, expertise in congenital
CMR is important in evaluating patients with
TOF, as RV volumes and function are key
components in evaluation for timing of
pulmonary valve replacement (S3.3-15, S3.3-
16).
Electrophysiological
care of patients
with ACHD
Perform procedures in
electrophysiology laboratories
equipped for 3D mapping and
ablation and involve
specialists experienced in the
Examples of diagnostic questions best answered by
electrophysiological study:
a) evaluation of the conduction system in cases of
suspected postsurgical conduction abnormalities
b) evaluation of syncope
Bradyarrhythmia and tachyarrhythmias are
common in TGA with atrial switch patients
and may seem “straightforward,” but the
altered anatomy adds complexity to the
procedures and emphasizes the need for

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management of arrhythmias
in patients with ACHD.
c) diagnosis of the mechanism of supraventricular
tachycardia or wide complex tachycardia
d) programmed ventricular stimulation particularly
in patients following repair of TOF and its variants
(Section 4.4.1.) as well as preoperative assessment
of arrhythmia substrates that may be amenable to
operative intervention, such as an atrial maze
procedure for atrial arrhythmias. The latter
procedure is commonly used at the time of
conversion of atriopulmonary connection Fontan,
and may also be useful in other forms of repaired
CHD with postoperative atrial arrhythmias such as
TOF.
specialized equipment and expertise to
ensure the best chance for procedural
success. For example: 1) pacemaker
placement in a patient with TGA with atrial
switch can be challenging because of the
altered atrial anatomy and interatrial baffle
that will necessitate placement of an atrial
lead in the anatomic left atrium, often
scarred such that tissue amenable to pacing
is difficult to find; and 2) atrial flutter is a
common arrhythmia in TGA with atrial
switch, but the flutter circuit may be on the
systemic side of the interatrial baffle and
thus may require baffle puncture or
retrograde approach to effectively ablate
the circuit.
Diagnostic and
interventional
cardiac procedures,
including
electrophysiology
procedures
1. Perform procedure in a
hospital with cardiologists,
anesthesiologists, surgeons,
and other providers with
expertise in the management
of patients with ACHD.
2. Consultation with providers
with ACHD expertise may be
substituted if the procedure is
urgent such that timely
transfer is not feasible.
1. Patients with ACHD often have complex
underlying cardiac anatomy and physiology.
2. The data obtained and the interventions
performed during ACHD cardiac procedures are
difficult to sort out without specialized knowledge
of the CHD.
3. An ACHD program has additional resources such
as cardiac anesthesia, congenital cardiac surgery,
and specialty cardiac imaging, should the need for
those services arise during or after the procedure.
In patients with CHD, the presence of
anatomic and physiological complexity from
the specific defect or surgical palliation, may
change the overall care plan and procedural
decision-making. Procedures that may seem
straightforward, such as pacemaker
implantation or ASD closure, may be more
complex when accounting for the nuances
imparted by CHD.
Administration of
anesthesia for
invasive procedures
in patients with
ACHD AP
classification IB-D,
IIA-D, and IIIA-D*
1. Performed by, or in
collaboration with, an
anesthesiologist with
expertise in the management
of patients with ACHD.
2. If clinical urgency precludes
transfer, consultation with an
anesthesiologist with ACHD
expertise would be of benefit
to on-site providers who do
not have ACHD expertise.
1. ACHD-specific issues need to be addressed when
considering anesthesia, including underlying cardiac
physiology and hemodynamics, and the effects of
anesthetic medications and ventilation strategies.
2. Many patients with ACHD have had surgeries in
the past, which may have created or identified
airway or vascular access concerns. Patients with
ACHD can also have underlying restrictive and/or
obstructive lung disease that should be considered
(S3.3-17, S3.3-18).
The application of anesthesia for
laparoscopic procedures can be especially
challenging in Fontan patients. Significant
cardiovascular and respiratory alterations
may occur as a result of increased intra-
abdominal pressure and decreased venous
return. Abdominal insufflation may lead to
lower preload and hypotension, while at the
same time elevating systemic vascular
resistance and compromising cardiac
output. Elevations in pulmonary vascular

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resistance attributable to hypercarbia can be
caused by either direct carbon dioxide
absorption or hypoventilation (S3.3-19).
Patients with ACHD
and pulmonary
hypertension
1. Consultation with experts in
pulmonary hypertension and
ACHD to assist in the
interpretation of diagnostic
and invasive studies and
determine the best course of
management.
1. PAH imparts a poor prognosis compared with
CHD without PAH. Because of the complexity of PAH
in the setting of CHD, patients with ACHD benefit
from the expertise of both ACHD providers and
pulmonary hypertension providers (S3.3-20–S3.3-
28).
Management of PAH in patients with shunts
can be difficult. For example, in patients for
whom PAH treatment is expected to allow
subsequent closure of a shunt, cohort series
demonstrate progression of pulmonary
vascular resistance or late mortality if
defects with associated pulmonary vascular
resistance elevation beyond 2.5 Wood units
(≥4 Wood units/m
2
) or Qp:Qs ≥3 were closed
(S3.3-29, S3.3-30). The utility of acute
administration of pulmonary vasodilator
therapy as a marker of reversibility of
pulmonary vascular resistance remains
uncertain. “Treat-to-repair” strategies
involving use of PAH therapies to bring
pulmonary vascular resistance into a range
where repair can be considered have been
applied, but the utility of such strategies also
remains uncertain.
*See Tables 3 and 4 for details on the ACHD AP classification system.
3D indicates 3-dimensional; ACHD, adult congenital heart disease; AP, anatomic and physiological; ASD, atrial septal defect; CCT, cardiac computed tomography; CHD, congenital
heart disease; CMR, cardiovascular magnetic resonance; IE, infective endocarditis; PAH, pulmonary arterial hypertension; PR, pulmonary regurgitation; Qp:Qs, pulmonary–
systemic blood flow ratio; RV, right ventricle; TGA, transposition of the great arteries; TOF, tetralogy of Fallot; and VSD, ventricular septal defect.

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3.4. Evaluation of Suspected and Known CHD
Tools commonly used in the evaluation of adults with suspected or known acquired cardiovascular
disease are also valuable in the evaluation of patients with ACHD. Some tools (e.g., echocardiography)
are regularly used in the serial evaluation of patients with ACHD, whereas other tools (e.g., CMR and
CCT) may have more utility in the evaluation and management of patients with ACHD than in patients
with acquired cardiovascular disease (Tables 7 and 8). Cost and risk to patients can be minimized by
ensuring studies are acquired and interpreted by centers and providers with CHD expertise.
3.4.1. Electrocardiogram
Recommendations for Electrocardiogram
I C-EO
1. A standard 12-lead electrocardiogram (ECG) is recommended in adults
with CHD with serial assessment depending on the specific ACHD AP
classification or when symptoms develop or worsen.
I C-EO
2. Ambulatory electrocardiographic monitoring should be performed in
patients with CHD who are at risk of tachyarrhythmia, bradyarrhythmia
or heart block, or when symptoms possibly of arrhythmic origin develop.
1. The ECG is an essential part of a complete cardiovascular evaluation of a patient with ACHD, similar to
elements of the physical examination. Regardless of anatomic diagnosis, it is important to obtain an ECG
at baseline for comparison to any subsequently obtained ECG, because an abnormal baseline ECG is
expected in many forms of CHD, particularly those who have undergone surgical repair. A follow-up ECG
is recommended in specific lesions and in the setting of new or worsening congestion or low cardiac
output syndrome (Table 7).
2. Asymptomatic arrhythmias seen in patients with ACHD may be associated with development of
symptoms and increased risk of death, and are more common in particular lesions or repairs.
Bradyarrhythmias or tachyarrhythmias may occur, with some requiring treatment even when
asymptomatic. For example, sinus node dysfunction is common in patients with atrial switch repairs of
transposition of the great arteries (TGA), whereas complete heart block is seen in patients with
congenitally corrected transposition of the great arteries (CCTGA) or late after atrioventricular septal
defect (AVSD) repair, especially in those patients with transient postoperative heart block (S3.4.1-1–
S3.4.1-3). Some of these events have occurred as late as 15 years after surgery. The atrioventricular
node is typically displaced inferiorly in AVSD, which is associated with relative hypoplasia of the left
anterior fascicle (S3.4.1-4). Atrial tachyarrhythmias are common in atrial switch repairs of TGA, Fontan
repairs, and Ebstein anomaly (S3.4.1-5–S3.4.1-7). Thus, baseline and periodic screening for
asymptomatic arrhythmias with ambulatory electrocardiographic monitoring is advised to ensure that
asymptomatic arrhythmias that would warrant a change in therapy are not present (S3.4.1-8),
acknowledging the limitations of monitoring over short periods of time. Any symptoms of arrhythmia
should prompt investigation to establish an accurate diagnosis and direct subsequent therapy.

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Table 7. Use of ECGs in ACHD Evaluation
• Identification of sinus bradycardia or junctional rhythm in
patients at risk of sinus node dysfunction (especially after the
Mustard, Senning, Glenn, or Fontan procedure)
• Identification of clinically inapparent intra-atrial re-entry
tachycardia in patients who have had atriotomy
• Identification of atrioventricular block in patients at risk for
progression of atrioventricular conduction system disease
(especially CCTGA)
• Evaluation of rhythm in patients with pacemakers
• Measurement of QRS duration in patients after repair of TOF and
as part of CRT evaluation
• Preoperatively to compare with postoperative ECGs in patients
undergoing heart surgery and noncardiac surgery
• Postoperatively to identify arrhythmias (e.g., atrial ectopic
tachycardia, atrial flutter, AF, junctional ectopic tachycardia,
atrioventricular block)
• Diagnosis of Wolff-Parkinson-White Syndrome in patients with
Ebstein anomaly
• Initial evaluation of suspected acute coronary syndromes
ACHD indicates adult congenital heart disease; AF, atrial fibrillation; CCTGA,
congenitally corrected transposition of the great arteries; CRT, cardiac
resynchronization therapy; ECG, electrocardiogram; and TOF, tetralogy of Fallot.
3.4.2. Ionizing Radiation Principles
Recommendation for Ionizing Radiation Principles
I B-NR
1. Strategies to limit and monitor radiation exposure are recommended
during imaging of patients with ACHD, with studies not involving ionizing
radiation chosen whenever appropriate (S3.4.2-1–S3.4.2-4).
1. Low-dose ionizing radiation is a known carcinogen, and certain levels of exposure similar to medical
exposure have been associated with later malignancy (S3.4.2-5, S3.4.2-6). Patients with ACHD have
multiple potential exposures to low-dose ionizing radiation throughout their lifetimes from cardiac
catheterizations, computed tomographic (CT) scans, nuclear perfusion scans, stress tests, and chest x-
rays. It remains unclear whether there is an increased risk of malignancy among patients with ACHD, but
the exposure levels from multiple procedures are in the range of concern. Every effort should be made
to use tests without radiation whenever possible or to select protocols with the lowest possible doses of
radiation compatible with securing the needed clinical information.
3.4.3. Echocardiography
Recommendations for Echocardiography
Referenced studies that support recommendations are summarized in Online Data Supplement 7.
I B-NR
1. Intraoperative TEE is recommended to guide surgical repair of CHD in
adults (S3.4.3-1).

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I C-EO
2. Patients with ACHD should undergo transthoracic echocardiography
(TTE) for initial assessment, with timing of serial assessment based on
anatomic and physiological severity and clinical status.
1. A large retrospective study has shown that the routine use of intraoperative TEE has a substantial
impact on patient care, leading to alteration of planned procedure or revision of the initial repair in 14%
of cases and was also determined to be cost-effective (S3.4.3-1).
2. For patients with ACHD in whom abnormalities and changes that may be identified on
echocardiography (e.g., valvular or ventricular function or pulmonary pressures) commonly influence
management decisions; echocardiography is an indispensable tool in the initial and serial follow-up
evaluation. TTE is also valuable in the initial and serial evaluation of patients without symptoms or
changes in examination (Table 8).
3.4.4. CMR Imaging
Recommendations for CMR Imaging
I B-NR
1. In patients with ACHD who have or who are at risk of developing RV
enlargement and dysfunction, serial CMR is recommended for
quantitative assessment of RV size and function (S3.4.4-1–S3.4.4-3).
IIa C-LD
2. CMR can be useful in the initial evaluation and serial assessment of
selected patients with CHD based on anatomic complexity and clinical
status (S3.4.4-1, S3.4.4-2, S3.4.4-4–S3.4.4-10).
1. CMR plays a valuable role in assessment of RV size and function, because it provides data that are
reproducible and more reliable than data obtained with alternative imaging techniques (S3.4.4-1–S3.4.4-
4). Real-time 3-dimensional (3D) echocardiography is an emerging technique that shows some promise
for replacing CCT and even CMR for serial studies, especially when focusing on ventricular volumes and
intracardiac structures only, and if reasonably complete data sets can be obtained (S3.4.4-11).
2. CMR has unique value in the assessment and serial follow-up of patients with ACHD, because it offers
unrestricted access to the heart and great vessels noninvasively and without ionizing radiation. The
complexity and variability of lesions, repairs, and sequelae in CHD constrain the use of standard
protocols and sequences, and often require modification of plans during acquisition of images, as well as
specialized skills in interpretation (S3.4.4-12, S3.4.4-13). Thus, a dedicated CMR service is integral to an
ACHD program (S3.4.4-4, S3.4.4-5). CMR can provide exquisite anatomic detail and unique physiological
information in many forms of CHD. It has a particularly important role in the assessment of extracardiac
cardiovascular defects (e.g., CoA, aortic aneurysm, and abnormalities of the thoracic arterial and venous
anatomy and connections) (S3.4.4-6, S3.4.4-7). The elucidation of uncommon, complex forms and
variations of CHD is routinely facilitated by a CMR study (S3.4.4-5). Contraindications to CMR are
common in patients with ACHD, so they should be sought and confirmed. However, the high value of
serial CMR has encouraged modification of newer pacemakers, leads, and other devices and imaging
protocols to facilitate imaging in an expanding subset of patients with ACHD who have had previous
instrumentation. If a contraindication is confirmed, alternative forms of imaging, especially CCT, can
obtain much of the information otherwise obtained from CMR and some unique information not

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provided by CMR (S3.4.4-14). However, CCT has the disadvantage of substantial patient exposure to
ionizing radiation, especially when serial studies are contemplated over a lifetime (S3.4.4-9). Real-time
3D echocardiography shows promise for replacing CCT and even CMR for serial studies, especially when
focusing on ventricular volumes and intracardiac structures only, and if reasonably complete data sets
can be obtained (Tables 8 and 9) (S3.4.4-11, S3.4.4-15).
Table 8. Circumstances Where CMR, CCT, TEE, and/or Cardiac Catheterization May be Superior to TTE
CCT indicates cardiac computed tomography; CMR, cardiovascular magnetic resonance; PA, pulmonary artery; RV, right
ventricular; TEE, transesophageal echocardiography; and TOF, tetralogy of Fallot.
Table 9. Comparison of Imaging Modalities Useful in ACHD Evaluation
Radiation
Exposure
Relative
Cost
Ventricular
Volumes/Function
Valvular
Structure/
Function
Coronary
Anatomy
and
Course
Extracardiac
Vascular
Anatomy
Echocardiography No $ ++ +++ +/- +/-
CMR No $$ +++ ++ ++* +++
CCT Yes $$ +* + +++ +++
Cardiac
catheterization
Yes $$ + ++ +++ ++
*In specific gated imaging protocols.
$ indicates less expensive; $$, more expensive; +/-, possible value; +, good; ++, very good; and +++, excellent.
ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; and CMR, cardiovascular magnetic
resonance.
3.4.5. Cardiac Computed Tomography
Recommendation for Cardiac Computed Tomography
IIa C-LD
1. CCT imaging can be useful in patients with ACHD when information that
cannot be obtained by other diagnostic modalities is important enough
to justify the exposure to ionizing radiation (S3.4.5-1, S3.4.5-2).
1. The most important disadvantage of CCT (including CT angiography) as an imaging technique is the
associated exposure to ionizing radiation. This is especially problematic in patients with ACHD in whom
serial assessments are contemplated over a lifetime (S3.4.5-1). Gating CCT to the ECG allows image
• Assessment of RV size and function in repaired TOF, systemic right ventricles, and other conditions
associated with RV volume and pressure overload (S3.4.4-1, S3.4.4-3)
• Identification of anomalous pulmonary venous connections (S3.4.4-16)
• Serial assessment of thoracic aortic aneurysms, especially when the dilation might extend beyond the
echocardiographic windows (S3.4.4-7)
• Accurate assessment of PA pressure and pulmonary vascular resistance
• Assessment for recoarctation of the aorta
• Sinus venosus defects
• Vascular rings
• Evaluation of coronary anomalies
• Quantification of valvular regurgitation

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acquisition during multiple phases of the cardiac cycle, thereby providing cine imaging and the ability to
select phases of the cycle of specific interest (usually end-systole and end-diastole), at the cost of
increased radiation dose. Electrocardiographic gating is generally unnecessary when the focus is
assessment of extracardiac vascular structures, which can consequently be imaged using substantially
lower doses of ionizing radiation. Ongoing development of protocols and equipment that reduce
radiation exposure are welcome advances (S3.4.5-2).
3.4.6. Cardiac Catheterization
Recommendations for Cardiac Catheterization
I C-LD
2. Cardiac catheterization (hemodynamic and/or angiographic) in patients
with ACHD AP classification II and III, or interventional cardiac
catheterization in patients with ACHD AP classification I to III should be
performed by, or in collaboration with, cardiologists with expertise in
ACHD (S3.4.6-1–S3.4.6-4).
IIa B-NR
3. In patients with a low or intermediate pretest probability of coronary
artery disease (CAD), use of CT coronary angiography is reasonable to
exclude significant obstructive CAD when cardiac catheterization has
significant risk or because of patient preference (S3.4.6-5–S3.4.6-9).
1. Cardiac catheterization remains a standard tool when diagnosis, prognosis, or management require a)
more precise definition of anatomy than is achievable via advanced noninvasive imaging (e.g., structures
with low flow or those shielded from other techniques), b) calculation of pressures and resistances, or c)
physiological or anatomic simulation to allow additional calculation or anatomic visualization. Cardiac
catheterization can provide unique information not reliably available from other diagnostic modalities
(e.g., direct pressure measurement in a vessel or chamber, determination of pulmonary artery (PA)
pressures and resistance, and optimal imaging of vessels in which flow is compromised). Procedures
should be planned with appreciation of the anatomy and physiology likely to be encountered, including
sequelae and residua of prior surgery and interventions.
The expansion of interventional catheter techniques has dramatically expanded possibilities for
interventional treatment for an increasing number of conditions. Operators require specialized training
and expertise in ACHD. In addition, catheterization laboratories specially equipped with devices and
tools used in ACHD intervention are needed and personnel trained in their use. Such equipment and
expertise differ from those found in catheterization laboratories devoted primarily to diagnostic
catheterization and coronary interventions.
2. For patients at low or intermediate risk of obstructive coronary disease, CT coronary angiography can
be an alternative to cardiac catheterization for assessing coronary artery course and patency.

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3.4.7. Exercise Testing
Recommendations for Exercise Testing
IIa B-NR
1. In patients with ACHD, cardiopulmonary exercise testing (CPET) can be
useful for baseline functional assessment and serial testing (S3.4.7-1,
S3.4.7-2).
IIa C-LD
2. In symptomatic patients with ACHD, a 6-minute walk test can be useful
to objectively assess symptom severity, functional capacity, and response
to therapy (S3.4.7-3, S3.4.7-4).
1. Patients with ACHD often overestimate their physical capabilities and underreport limitations. In
contrast to patients with acquired heart disease, patients with ACHD may never have experienced
”normal” function. Decline in physical capacity may occur imperceptibly over many years (S3.4.7-1,
S3.4.7-2). Consequently, tools more precise than patient history are necessary for evaluation and serial
follow-up of functional capacity. CPET provides objective, reproducible, and repeatable assessment of
the cardiovascular, respiratory, and muscular systems and has been shown to have prognostic value in
patients with a wide variety of ACHD conditions (S3.4.7-1).
2. In severely impaired patients with ACHD, or those who cannot complete CPET for other reasons, the
6-minute walk test provides a more limited set of data, which nevertheless has prognostic value beyond
history alone (S3.4.7-3, S3.4.7-4).
3.5. Transition Education
Recommendation for Transition Education
I B-NR
1. Clinicians caring for patients with CHD should deliver developmentally
appropriate transition education to adolescent and young patients with
CHD, and to their families/support network (S3.5-1, S3.5-2).
1. Preparing a patient for independent cardiac care is an ongoing process that should start in early
adolescence if not sooner (S3.5-3) and may extend beyond 18 years of age in many patients. The
recommendation and goals for transition and transition education have been described and include
verbal, written, and experiential efforts to teach patients and families about their specific heart disease,
expectations, and concerns regarding CHD, as well as skills to navigate the healthcare system as an adult
(S3.5-4). Lack of education about the need for transition and lifelong cardiac care leads to gaps in care
that can result in increased hospitalizations, need for urgent intervention, and increased morbidity
(S3.5-5, S3.5-6). A structured approach to transition education improves health related knowledge and
self-management (S3.5-1, S3.5-2). This education is a continual process that includes after transfer to an
ACHD care provider (S3.5-4).

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3.6. Exercise and Sports
Recommendations for Exercise and Sports
I C-LD
1. Clinicians should assess activity levels at regular intervals and counsel
patients with ACHD about the types and intensity of exercise appropriate
to their clinical status (S3.6-1–S3.6-9).
IIa C-LD
2. CPET can be useful to guide activity recommendations for patients with
ACHD (S3.6-10, S3.6-11).
IIa B-NR
3. Cardiac rehabilitation can be useful to increase exercise capacity in
patients with ACHD (S3.6-12, S3.6-13).
Synopsis
Historically, guidelines for physical activity among patients with CHD have focused on restriction, rather
than promotion of activity (S3.6-14, S3.6-15). Because of fears of adverse events such as SCD or aortic
dissection, recommendations derived from those that apply to competitive sports (S3.6-16) have been
applied to recreational activities despite the absence of evidence on the risk or safety of moderate
activity. The 2015 “Eligibility and Disqualification Recommendations for Competitive Athletes With
Cardiovascular Abnormalities: Task Force 4: Congenital Heart Disease” (S3.6-14) does work toward
encouraging participation and shared decision-making with patients regarding competitive sports
participation. Most patients with ACHD can safely engage in regular, moderate physical activity. A few
conditions, such as systemic ventricular systolic dysfunction, systemic ventricular outflow tract
obstruction, hemodynamically significant arrhythmias, or aortic dilation, warrant more cautious
recommendations (S3.6-17).
1. Physical activity is widely recognized as being beneficial to the physical and mental health of those
who participate (S3.6-2–S3.6-4). There is conflicting evidence regarding physical activity levels in
patients with CHD, with some suggesting the tendency for less activity (S3.6-5, S3.6-9) and a greater
prevalence of obesity (S3.6-1) than in the general population. Studies describe the beneficial effects and
safety of exercise programs for patients across the spectrum of CHD (S3.6-18, S3.6-19). Activity
recommendations should be individualized based on the patient’s clinical status and their interests
(S3.6-20).
2. There is evidence that exercise capacity varies among congenital heart defects, with declining capacity
(generally) as complexity increases (S3.6-10, S3.6-11). Knowledge of the typical exercise capacity for
patients with a specific lesion is important when making appropriate activity recommendations (S3.6-
10). Self-directed activity is usually at 40% to 60% of maximal exercise capacity, whereas fitness training
occurs at 60% to 80% of maximal capacity (S3.6-20). Exercise capacity is defined in relation to maximal
oxygen consumption. The writing committee recognizes that not all ACHD centers will have the
resources to conduct CPET, which is the preferred method of evaluation. If CPET cannot be performed,
other exercise tests using an established treadmill or bicycle ergometer protocol are an acceptable
alternative for assessing exercise capacity, recognizing that valuable information may be unavailable
compared with CPET.
3. As with other populations of cardiac patients, inactivity leads to reduced exercise performance.
Regular exercise and cardiac rehabilitation may improve exercise capacity and HF symptoms, and ought
to be encouraged (S3.6-6, S3.6-7, S3.6-21, S3.6-22).

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3.7. Mental Health and Neurodevelopmental Issues
Recommendations for Mental Health and Neurodevelopmental Issues
I B-NR
1. Patients with ACHD should be evaluated for depression and anxiety
(S3.7-1–S3.7-3).
IIa B-NR
2. Referral for mental health evaluation and treatment is reasonable in
patients with ACHD (S3.7-1–S3.7-4).
IIb B-NR
3. Neurodevelopmental or neuropsychological testing may be considered in
some patients with ACHD to guide therapies that enhance academic,
behavioral, psychosocial, and adaptive functioning (S3.7-5–S3.7-9).
Synopsis
Mental health and neurodevelopmental issues are common in patients with ACHD and may significantly
affect QoL. Neurodevelopmental abnormalities are more frequently seen in children who have complex
disease, complex surgical repairs, and other characteristics (S3.7-10–S3.7-12). There is extensive
literature in the pediatric population on the frequency and importance of neurodevelopmental
abnormalities, However, many adults may not have been evaluated as children in accordance with
current diagnostic and treatment strategies (S3.7-13, S3.7-14). Neurodevelopmental disorders, such as
impairment of cognition, social skills and communication, and attention disorders, are often
underrecognized even though appropriate diagnosis, treatment, and rehabilitation may be beneficial in
optimizing function and QoL. An AHA scientific statement describes the common neurodevelopmental
disorders affecting children with CHD and may inform neurodevelopmental issues related to adults with
CHD (S3.7-13).
1. Anxiety and depression are underrecognized in the ACHD population. Point-of-care assessment with
simple questions about anxiety and depression should be included in the symptom review.
2. Anxiety and depression are prevalent among patients with ACHD. Self-reported symptoms are
incomplete to identify the existence of mood disorders. Structured professional psychological evaluation
can identify up to 50% more patients with mood disorders (S3.7-1).
3. Although there is limited evidence on neurodevelopmental and neuropsychological issues in patients
with ACHD, there is increasing evidence of the neurodevelopmental impact of CHD and surgery in
childhood (S3.7-6, S3.7-8, S3.7-9). It is likely that this impact will persist into adulthood and may
manifest in lower educational and occupational achievement. This is particularly evident in patients with
genetic conditions such as 22q11 deletion and trisomy 21.
3.8. Endocarditis Prevention
Patients with ACHD have an increased risk of developing infective endocarditis (IE) (S3.8-1, S3.8-2). The
most common pathogens responsible for IE include Streptococcus viridans, Staphylococcus species, and
Enterococcus species. Despite advances in antimicrobial therapy and surgical techniques, IE remains a
condition associated with significant morbidity and mortality. Numerous guidelines are available with
recommendations on the prevention and diagnosis of IE (S3.8-3–S3.8-5). These guidelines include
consistent descriptions of the patients at highest risk of adverse effects from endocarditis. Antibiotic

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prophylaxis continues to be recommended for patients with high-risk characteristics, which are often
found in patients with ACHD (S3.8-2). These patients include:
• Those with previous IE;
• Patients with prosthetic valves (biological and mechanical, surgical and transcatheter);
• Patients within 6 months of placement of prosthetic material;
• Patients with residual intracardiac shunts at the site of or adjacent to previous repair with
prosthetic material or devices; or
• Patients with uncorrected cyanotic heart disease.
See Online Data Supplement 15 for referenced studies.
3.9. Concomitant Syndromes
Recommendation for Concomitant Syndromes
IIa B-NR
1. Genetic testing for 22q11 deletions is reasonable for patients with
conotruncal cardiac defects (S3.9-1, S3.9-2).
Synopsis
Patients with genetic syndromes may have phenotypic manifestations and associated CHD as clinical
features of the genetic abnormality. An underlying chromosomal abnormality exists in at least 10% of
infants with CHD and may not have been previously tested in patients with ACHD (S3.9-3). Clinicians
caring for patients with ACHD should recognize the potential for undiagnosed genetic abnormalities that
may affect overall health (Table 10) and pursue appropriate evaluation.
1. Several forms of CHD may be associated with underlying genetic syndromes (Table 10). Some genetic
syndromes may not be phenotypically apparent in adults, and prior childhood genetic workup may not
be readily available; therefore, genetic syndromes may be missed in patients with ACHD. Many of these
syndromes may have important clinical comorbidities, including but not limited to learning disabilities,
psychiatric conditions, and reproductive disorders. Up to 5% of children born with CHD have DiGeorge
syndrome (22q11.2 deletion), the congenital heart defects most commonly associated being those of
conotruncal origin. DiGeorge syndrome is an autosomal dominant condition. Therefore, genetic testing
is reasonable for patients with ACHD with conotruncal defects for recognition and management of
comorbidities and for counseling on the potential risk of recurrence in offspring (S3.9-4, S3.9-5).
Table 10. Underlying Genetic Syndromes Commonly Associated With CHD (S3.9-4, S3.9-6)
Syndrome
Genetic
Abnormality Clinical Features
Common Cardiac
Findings
DiGeorge syndrome
(velocardiofacial
syndrome)
22q11.2 deletion Thymic and parathyroid hypoplasia,
immunodeficiency, low-set ears,
hypocalcemia, speech and learning
disorders, renal anomalies, psychiatric
disease
25%–75% have CHD, depending on
age studied (S3.9-7, S3.9-8)
IAA type B, aortic arch
anomalies, truncus
arteriosus, TOF
Down syndrome Trisomy 21 Developmental disability, ASD, VSD, AVSD, TOF

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characteristic facial features,
hypotonia, palmar crease
40%–50% have CHD
Holt–Oram
syndrome (S3.9-9)
TBX5 Upper limb skeletal abnormalities
75% have CHD
ASD, VSD, MV disease
Klinefelter
syndrome
47 XXY Tall stature, hypoplastic testes,
delayed puberty, developmental
disability
50% have CHD
PDA, ASD, MV prolapse
Noonan syndrome
(S3.9-10)
PTPN11, KRAS,
SOS1 RAF1, NRAS,
BRAF, MAP2K1
Facial anomalies, webbed neck, chest
deformity, short stature, lymphatic
abnormalities, bleeding abnormalities
80% have CHD
PS, ASD, HCM
Turner syndrome 45X Short stature, webbed neck,
lymphedema, primary amenorrhea
30% have CHD
Risk of aortic dissection
Coarctation, BAV, aortic
stenosis, hypoplastic left
heart, ascending
aortopathy
Williams syndrome 7q11.23 deletion Elfin face, social personality, hearing
loss, developmental delay, infantile
hypercalcemia
50%–80% have CHD
Supravalvar aortic
stenosis, peripheral PS
ASD indicates atrial septal defect; AVSD, atrioventricular septal defect; BAV, bicuspid aortic valve; CHD, congenital heart
disease; HCM, hypertrophic cardiomyopathy; IAA, interrupted aortic arch; MV, mitral valve; PDA, patent ductus arteriosus; PS,
pulmonary stenosis; TOF, tetralogy of Fallot; and VSD, ventricular septal defect.
3.10. Acquired Cardiovascular Disease
Patients with ACHD can acquire other cardiovascular diseases such as hypertension, atherosclerotic
coronary artery disease, vascular disease, stroke, and HF (S3.10-1–S3.10-3). The impact of acquired
heart disease is increasing as the lifespan of patients with ACHD extends. Myocardial infarction is one of
the leading contributing causes of death for late surviving adults with acyanotic CHD (S3.10-4). Major
adverse cardiac events, such as HF, percutaneous coronary intervention, coronary artery bypass graft
surgery, malignant arrhythmia, cardiac shock, and placement of an implantable cardioverter-defibrillator
(ICD), are also quite prevalent (S3.10-5, S3.10-6). Overall, cardiovascular reasons account for
approximately 77% of all deaths in patients with ACHD, with approximately half attributable to chronic
HF (S3.10-7). Evaluation for acquired cardiac conditions is warranted in patients with risk factors,
although results of testing (e.g., stress perfusion studies) should account for preexisting abnormalities
caused by CHD, recognizing prior interventions can mimic abnormalities otherwise suggestive of
acquired heart disease (S3.10-8).
In patients with ACHD, prevention and treatment of conditions predisposing to acquired
cardiovascular disease such as diabetes mellitus, obesity, hypertension, dyslipidemia, and/or similar
comorbidities are important. Given the increased risk of acquired cardiovascular disease with age,
promoting a healthy lifestyle is important in all patients with ACHD, although there are not data
demonstrating the effects of risk reduction on clinical outcomes specific to the ACHD population.
Emphasizing the importance of daily physical activity according to functional capacity, and decreasing
sedentary behavior as appropriate for the patient’s clinical status is essential when counseling patients
with congenital heart defects (S3.10-9). Interestingly, most patients with ACHD lead healthier lifestyles
compared with control patients (S3.10-10), suggesting that this patient population may be receptive to
advice and may continue to benefit from recommendations about diet, activity, and modifiable risk
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3.11. Noncardiac Medical Issues
Recommendation for Noncardiac Medical Issues
I C-LD
1. Patients with ACHD at risk for hepatitis C should be screened and
vaccinated for viral hepatitis and treated as appropriate (S3.11-1).
1. Patients with ACHD are at risk of hepatitis C because of blood exposure during cardiac surgery.
Hepatitis screening is warranted especially in those with exposure to blood products before universal
screening for hepatitis C, which began in 1992. Hepatitis vaccination and/or consultation with a
hepatologist should also be offered where appropriate, particularly in patients with ACHD with
concomitant liver disease (e.g., Fontan patients).
3.12. Noncardiac Surgery
Recommendations for Noncardiac Surgery
I C-LD
1. Optimization before and close surveillance after invasive procedures,
regardless of the complexity of the anatomic defect or type of procedure
is beneficial for patients with ACHD (S3.12-1–S3.12-4).
I B-NR
2. In patients with ACHD AP classification IB-D, IIA-D, and IIIA-D* noncardiac
surgical and interventional procedures should be performed in a hospital
with or in consultation with experts in ACHD when possible (S3.12-1,
S3.12-3, S3.12-5–S3.12-9).
Synopsis
Patients with ACHD may have greater operative risk than patients without ACHD. The ”2014 ACC/AHA
Guideline on Perioperative Cardiovascular Evaluation and Management of Patients Undergoing
Noncardiac Surgery” (S3.12-10) may be applied; however, those guidelines may not apply directly. One
must remain cognizant that there are differences in cardiac issues commonly present in patients with
ACHD, such as mechanisms for ventricular dysfunction, type and mechanisms of arrhythmia, and the
probability of coronary artery disease. The 2014 guideline (S3.12-10) was developed primarily with
evidence and experience derived from, and related to, patients with acquired heart disease. Thus, the
evidence supporting recommendations regarding risk indices and management strategies may not apply
to many patients with ACHD.
1. A checklist of issues to consider in the assessment and management of patients with ACHD
undergoing noncardiac surgery is presented in Table 11. Patients with ACHD may present with
nonroutine and unusual physiological challenges (e.g., those related to fluid balance in the setting of
single ventricle or the impact of vascular resistances on shunts in cyanotic patients) (S3.12-2–S3.12-4).

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Heightened surveillance may mandate extended postoperative intensive or other high-acuity care
(S3.12-2).
2. Case series and analysis of administrative databases confirm that surgical procedures in patients with
ACHD carry greater risk than in patients without ACHD (S3.12-1–S3.12-3, S3.12-6, S3.12-8, S3.12-11–
S3.12-13). Risk relates to the specific type of ACHD, surgical procedure, urgency of intervention, and
availability of specialized resources (S3.12-1, S3.12-3–S3.12-6, S3.12-8, S3.12-14). Noncardiac surgery is
usually accomplished without substantial morbidity or mortality, but even minor surgery can be
complicated in patients with ACHD. Surgery that is low risk in the general population may be associated
with higher risk in the ACHD population (S3.12-1, S3.12-6). Patients with ACHD may present with
nonroutine and unusual physiological challenges (e.g., those related to fluid balance in the setting of
single ventricle or the impact of vascular resistances on shunts in cyanotic patients) (S3.12-2, S3.12-4).
When possible, patients with ACHD, especially those with complex disease (ACHD AP
classification II and III) and/or whose disease has progressed (stages B, C, D) (Tables 3 and 4), should
receive preoperative evaluation and surgery or other nonsurgical intervention within an ACHD program.
Because the inability to access resources or urgent conditions may preclude transfer or timely
consultation, collaboration with members of the multidisciplinary ACHD team may be helpful. Clear
processes for timely consultation and support are needed to manage the physiological challenges
presented by patients with ACHD related to fluid balance, vascular resistance, and shunts (S3.12-3,
S3.12-4). A checklist of issues to consider in assessment and management of patients with ACHD
undergoing noncardiac surgery is presented in Table 11.
Table 11. ACHD Management Issues for Noncardiac Surgery
Clarify CHD diagnosis
Clarify prior procedures, residua, sequelae, and current status,
including ACHD AP classification
Be aware that history obtained from only the patient and
family may be faulty or incomplete
Obtain and review old records to ensure accurate
understanding of past procedures and clinical course
Complete additional investigations required to define ACHD
AP classification
Develop management strategies to minimize risk and optimize
outcome
Factors associated with increased risk of perioperative morbidity
and mortality (S3.12-12):
• Cyanosis
• Congestive HF
• Poor general health
• Younger age
• Pulmonary hypertension
• Operations on the respiratory and nervous systems
• Complex CHD
• Urgent/emergency procedures
Issues to consider:
• Endocarditis prophylaxis
• Complications related to underlying hemodynamics
• Abnormal venous and/or arterial anatomy affecting venous
and arterial access
• Persistent shunts

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• Valvular disease
• Arrhythmias, including bradyarrhythmias
• Erythrocytosis
• Pulmonary vascular disease
• Meticulous line care (also consider air filters for intravenous
lines) to reduce risk of paradoxic embolus in patients who
are cyanotic because of right-to-left shunts
• Adjustment of anticoagulant volume in tubes for some
blood work in cyanotic patients
• Prevention of venous thrombosis
• Monitoring of renal and liver function
• Periprocedure anticoagulation
• Possible need for nonconventional drug dosing
• Increased prevalence of hepatitis C infection because of
prior procedures and remote blood transfusions
• Developmental disability
ACHD indicates adult congenital heart disease; AP, anatomic and
physiological; CHD, congenital heart disease; and HF, heart failure.
3.13. Pregnancy, Reproduction, and Sexual Health
3.13.1. Pregnancy
Recommendations for Pregnancy
I C-LD
1. Women with CHD should receive prepregnancy counseling with input
from an ACHD cardiologist to determine maternal cardiac, obstetrical and
fetal risks, and potential long-term risks to the mother (S3.13.1-1–
S3.13.1-4).
I C-LD
2. An individualized plan of care that addresses expectations and
contingencies should be developed for and with women with CHD who
are pregnant or who may become pregnant and shared with the patient
and all caregivers (S3.13.1-2, S3.13.1-3).
I B-NR
3. Women with CHD receiving chronic anticoagulation should be counseled,
ideally before conception, on the risks and benefits of specific
anticoagulants during pregnancy (S3.13.1-5, S3.13.1-6).
I B-NR
4. Women with ACHD AP classification IB-D, IIA-D, and IIIA-D* should be
managed collaboratively during pregnancy by ACHD cardiologists,
obstetricians, and anesthesiologists experienced in ACHD (S3.13.1-2,
S3.13.1-7, S3.13.1-8).
I C-EO
5. In collaboration with an ACHD cardiologist to ensure accurate
assessment of pregnancy risk, patients at high risk of maternal morbidity
or mortality, including women with pulmonary arterial hypertension
(PAH), Eisenmenger syndrome, severe systemic ventricular dysfunction,
severe left-sided obstructive lesions, and/or ACHD AP classification ID,
IID, IIID* should be counseled against becoming pregnant or be given the
option of terminating pregnancy.
I B-NR 6. Men and women of childbearing age with CHD should be counseled on

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the risk of CHD recurrence in offspring (S3.13.1-9).
IIa B-NR
7. Exercise testing can be useful for risk assessment in women with ACHD
AP classification IC-D, IIA-D, and IIIA-D* who are considering pregnancy
(S3.13.1-10, S3.13.1-11).
IIa B-NR
8. When either parent has CHD, it is reasonable to perform fetal
echocardiography (S3.13.1-12, S3.13.1-13).
*See Tables 3 and 4 for the ACHD AP classification system.
Synopsis
Most data regarding cardiac and obstetric risk to women with CHD during pregnancy derive from
retrospective case series (S3.13.1-2–S3.13.1-5, S3.13.1-8, S3.13.1-10, S3.13.1-12, S3.13.1-14–S3.13.1-
20). Many women with CHD considering pregnancy may have received inconsistent guidance regarding
pregnancy risks (S3.13.1-21). Several risk scores have been developed to risk-stratify women with heart
disease desiring pregnancy (S3.13.1-2, S3.13.1-7), and a prospective validation study suggests that the
World Health Organization classification is the most accurate prediction model (S3.13.1-11). Although
many women with CHD tolerate the hemodynamic changes of pregnancy, others may face significant
immediate or late risks of pregnancy including volume overload, arrhythmias, progressive cardiac
dysfunction, and death. Cardiac medications may need to be adjusted during pregnancy and counseling
provided to discuss the options for and potential impact of those changes. Some specific complications
may be more common in women with certain types of CHD, such as hypertension, which is more
common in women with coarctation (S3.13.1-22, S3.13.1-23). The offspring of patients with ACHD have
an increased risk of CHD and other events such as prematurity (S3.13.1-24). All women with CHD should
receive appropriate counseling regarding contraception choices. A multidisciplinary team that includes
ACHD specialists and maternal-fetal medicine obstetricians with expertise in caring for women with
heart disease is appropriate for achieving optimal outcomes.
1. Prepregnancy counseling allows for an individualized risk assessment. This will include discussing
maternal risks for pregnancy, delivery, and postpartum period, and medications that may be teratogenic
and require alternative therapies (e.g., angiotensin-converting enzyme inhibitors/angiotensin-receptor
blockers). Additionally, counseling should include a discussion related to fetal risk in regard to CHD
transmission and overall risk to the health of the fetus. ACHD cardiologists are valuable in accurately
assessing pregnancy risks. Risk may be overestimated or underestimated by providers without expertise
in CHD and pregnancy, leading to patients’ receiving inaccurate recommendations on risks of pregnancy,
risks of delivery, and the type of delivery (e.g., the incorrect notion that most women with CHD require
cesarean delivery for cardiac reasons).
2. This care plan should address maternal cardiac risks on the basis of the individual patient’s anatomy
and physiology. Clear documentation is important so that all providers are well aware of the risks and
expected outcomes, including risk of maternal volume shifts, arrhythmias, labor and delivery plan, and
need for maternal cardiac monitoring when indicated. Contingency plans for anticipated complications
related to the presence of CHD should also be developed.
3. Chronic anticoagulation during pregnancy is associated with increased risk of maternal bleeding and
thrombotic events as well as a higher risk of fetal loss, and in the case of warfarin, the risk of
teratogenicity (S3.13.1-5, S3.13.1-14). The choice of specific anticoagulant must balance maternal well-
being and risks for mother and fetus, and should be individualized. Patients with mechanical valves
should be treated according to GDMT (S3.13.1-25).

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4. The hemodynamic changes of pregnancy, labor, and delivery can result in hemodynamic
decompensation for some women with CHD (S3.13.1-1, S3.13.1-7, S3.13.1-15, S3.13.1-24). Management
involving the expertise of ACHD, maternal-fetal medicine, and anesthesiology should help anticipate and
mitigate some of the potentially detrimental maternal or fetal outcomes.
5. Women at high risk include, but are not limited to, those diagnosed with cardiac conditions that meet
World Health Organization maternal cardiac risk classification IV (S3.13.1-26).
a. PAH of any cause
b. Severe systemic ventricular dysfunction: LV ejection fraction <30% and/or NYHA III–IV symptoms
c. Severe left heart obstruction
d. Severe native coarctation (S3.13.1-16, S3.13.1-27, S3.13.1-28)
These patients have an extremely high risk of maternal mortality or severe morbidity, and if pregnant,
the option of pregnancy termination should be discussed
6. Prepregnancy counseling regarding the risk of CHD recurrence in offspring provides helpful
information to parents to inform decision-making regarding family planning and delivery options, and
should allow adequate time dedicated to answering important questions from the parents.
7. CPET performed before conception can predict maternal and neonatal outcomes in pregnant women
with CHD. A blunted heart rate response to exercise in women with CHD is associated with a higher risk
of maternal cardiac and neonatal adverse events (S3.13.1-11).
8. If the patient with CHD or their partner is pregnant, there is an increased risk of CHD in the offspring
and fetal echocardiography can be useful in defining whether CHD is present, and if so, help to
determine the course of action at the time of delivery. There are data to suggest a prenatal diagnosis
improves neonatal survival, although selection bias (e.g., preoperative deaths, family preference) is a
limitation for many studies, so benefit has been more difficult than expected to prove (S3.13.1-13,
S3.13.1-29, S3.13.1-30).
3.13.2. Contraception
Recommendations for Contraception
I C-LD
1. Women of childbearing potential with CHD should be counseled about
the risks associated with pregnancy and appropriate contraceptive
options (S3.13.2-1–S3.13.2-3).
III:
Harm
B-NR
2. Estrogen-containing contraceptives are potentially harmful for women
with CHD who are at high risk of thromboembolic events (e.g., cyanosis,
Fontan physiology, mechanical valves, prior thrombotic events, PAH)
(S3.13.2-4, S3.13.2-5).
Synopsis
The use of contraceptive agents should be balanced against the risks of pregnancy in every woman with
CHD after menarche (S3.13.2-6). There are no data on the safety of various contraceptive techniques in
patients with ACHD.

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1. The individualized benefits and risks of each contraceptive therapy must be determined based on the
patient’s anatomy and physiology in consultation with a gynecologist. This counseling should include the
expected failure rates of contraceptive options and the anticipated maternal and fetal risks of
unplanned pregnancy, with these issues revisited on a regular basis.
Contraceptive choices include combined hormonal (estrogen/progesterone) contraception,
progesterone-only agents, intrauterine devices, barrier methods, and permanent sterilization. Low-dose
combination oral contraceptive (≤20 mcg of ethinyl estradiol) is an option except in women who are at
increased risk of thrombosis (S3.13.2-4). Medroxyprogesterone acetate is a less effective method of
contraception, and the potential for fluid retention must be considered (S3.13.2-5). Intrauterine devices
are highly effective methods of contraception; however, women may experience vasovagal reactions at
the time of implant. Tubal ligation is generally safe with recognized risks associated with anesthesia and
abdominal insufflation. An efficacious option is a vasectomy for the male partner; however, the long-
term prognosis of the female patient with CHD must be considered and discussed openly. In the case of
unplanned pregnancy with desire for termination, the morning-after pill (levonorgestrel) is safe for
women, but acute fluid retention is a risk to be considered.
2. Women with CHD who are at high risk of thrombosis include those with cyanosis, Fontan physiology,
mechanical valves, prior thrombotic events, and PAH. In women who are at high risk of thrombosis and
who receive warfarin, there are no data on which to base a recommendation or counseling as to
whether it is safe to use estrogen-containing contraception. It is unclear whether the use of warfarin
offsets adequately the additional risk of thrombosis related to pregnancy in high-risk patients.
3.13.3. Infertility Treatment
Menstrual cycle disorders are not uncommon in women with CHD. In small case series of women with
CHD, various causes for infertility were documented including primary and secondary amenorrhea,
oligomenorrhea, and uterine anomalies (S3.13.3-1, S3.13.3-2). In more complex forms of CHD (e.g., the
population with Fontan palliation), the prevalence of primary amenorrhea may be as high as 40%
(S3.13.3-2). Menarche occurs at an older age in these women than in the general population (S3.13.3-2).
Women with CHD also have higher rates of spontaneous abortion and miscarriage (S3.13.3-3–S3.13.3-5).
The prevalence of infertility in men with CHD is unknown. Each patient with ACHD should be counseled
regarding the potential for infertility and referral to a specialized reproductive endocrinologist when
appropriate, although there is little specific guidance for women based on types of CHD. Alternative
options for family planning including assisted reproductive technologies and adoption is appropriate,
and risks versus benefits of all options are addressed during counseling.
3.13.4. Sexual Function
Sexuality is an important element of QoL. Although there are data that sexual function is a concern in
both women and men, there is minimal evidence on the prevalence of sexual concerns among adults
with CHD and far less to guide interventions (S3.13.4-1).
Concerns with sexual health are present in 20% to 40% of men with CHD (S3.13.4-2–S3.13.4-4).
Erectile dysfunction is reported by up to 42% of men with CHD (S3.13.4-1, S3.13.4-3). Men with CHD
report being in sexual relationships significantly less often than the general population (S3.13.4-1,
S3.13.4-4). Among men with CHD who report sexual health concerns, there is a high level of
psychological distress and diminished QoL (S3.13.4-1, S3.13.4-2, S3.13.4-4, S3.13.4-5). The ACHD
provider should be mindful of this often-unspoken concern and create an environment in which the
patient feels comfortable addressing concerns about their sexuality.

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3.14. Heart Failure and Transplant
3.14.1. Heart Failure
Recommendation for Heart Failure
I C-LD
1. Consultation with ACHD and HF specialists is recommended for patients
with ACHD and HF or severe ventricular dysfunction (S3.14.1-1–S3.14.1-
4).
Synopsis
HF is a significant issue in patients with ACHD. It is common, associated with morbidity and mortality,
and is anticipated to increase in prevalence. However, despite the clinical importance of HF in patients
with ACHD and efforts to study the effects of medication and device therapy in these patients, there are
no data to support treatment recommendations. For patients with biventricular physiology, systemic left
ventricular (LV) dysfunction, no repairable residual hemodynamic abnormalities, and persistent HF
symptoms, standard GDMT is ostensibly preferable to no treatment. However, expectations of its
benefit should be tempered, and risk may be different in patients with acquired CVD, because CHD
patients have not been included in the trials by which those guidelines were developed.
1. HF is common in patients with ACHD and is associated with increased morbidity and mortality
(S3.14.1-1–S3.14.1-4). There are many causes of HF symptoms that may be reversible, including valve
dysfunction, shunts, arrhythmias, venous obstruction, and systolic and/or diastolic ventricular
dysfunction, which require evaluation and treatment when possible. Unlike acquired HF, and despite the
clinical importance of HF in ACHD, data to support a treatment recommendations including typical HF
medical therapy (e.g., angiotensin-converting enzyme inhibitors, angiotensin-receptor blockers, beta
blockers, and aldosterone antagonists) (S3.14.1-5) are limited in patients with ACHD (S3.14.1-6–S3.14.1-
22). HF in patients with ACHD is multifactorial and may manifest as variable response to
pharmacotherapy. Advanced HF therapies may be technically difficult or considered too late in the
course. Thus, timely evaluation by ACHD and HF specialists is crucial to optimal care of such patients.
3.14.2. Heart Transplant
Because of the prevalence of HF among patients with CHD, heart transplantation is increasingly being
considered as a therapeutic option. Data on proper timing of transplantation are limited, particularly for
individual lesions. Larger studies based on transplant databases do not allow for analysis based on the
type of CHD (S3.14.2-1–S3.14.2-4). Currently, patients with ACHD may have fewer mechanical
circulatory devices (e.g., ventricular-assist devices), which may lower their listing status and hence
potential for organ receipt (S3.14.2-1, S3.14.2-2, S3.14.2-4–S3.14.2-7).
Although specific criteria for timing of referral for transplantation are desirable, universal
recommendations cannot be made based on current data. Generally, published data show that
immediate and early posttransplantation risk is higher in ACHD than in acquired heart disease because
of increased perioperative mortality (S3.14.2-2). However, once beyond the perioperative period,
patients with ACHD do as well as or better than those with acquired heart disease, with expected 10-
year survival equivalent to or better than that of patients without ACHD (S3.14.2-2–S3.14.2-4, S3.14.2-6,
S3.14.2-7). Risks for poor outcomes include single ventricle anatomy, anatomic complexity, protein-

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losing enteropathy, or high titers of panel reactive antibodies (S3.14.2-8, S3.14.2-9). The current
allocation system puts patients with ACHD at a disadvantage. Rather than priority dictated by the usual
accepted risk markers, patients with ACHD are often listed by “exception,” a process that requires the
clinician to argue that the patient warrants higher priority than would be evident by applying the used
risk markers. There is also significant mortality for patients with ACHD while on the waitlist (S3.14.2-10,
S3.14.2-11). Surgical alternatives to transplantation exist for some patients with CHD (e.g., valve
replacement, shunt closure), but these patients are at high risk of perioperative mortality (S3.14.2-12).
Ideally, providers will consider early referral to a transplant center with expertise in ACHD
transplantation when transplantation becomes a relevant clinical consideration. Additionally, it is
advisable to consider options for transplantation or ventricular assist device as a backup before other
high-risk surgery is pursued.
3.14.3. Multiorgan Transplant
Recognizing the vulnerability of many organ systems in patients with CHD, multiorgan transplantation is
often considered, although infrequently performed. Multiorgan transplantation requires a
multidisciplinary and comprehensive approach with thoughtful planning and communication among
practitioners.
Multiorgan transplantation may be performed as sequential operations or as a single operation.
Typically, simultaneous multiorgan procedure in patients with CHD will be heart-lung transplantation for
conditions that result in irreversible pulmonary hypertension such as Eisenmenger syndrome. (S3.14.3-
1, S3.14.3-2). Fewer than 100 heart-lung transplants are performed internationally each year, with a
median survival of 3.3 years and 10-year survival of 32% (S3.14.3-3). Survival is worse for heart-lung
recipients than single-organ heart or lung recipients possibly, in part, because of longer wait times
(S3.14.3-4).
The occurrence of simultaneous heart-liver transplantation is an option in patients with severe
right-sided HF and in single ventricle patients after Fontan palliation. Given the recognized vulnerability
of the liver to injury in Fontan patients and the fact that heart alone transplantation outcomes have
been poor in patients with concomitant liver dysfunction, transplant centers may favor heart-liver
transplantation in those with cirrhosis, but this policy is not universal. Fewer than 15 such procedures
are performed annually in the United States, and approximately 20% of patients are referred because of
underlying CHD (S3.14.3-5, S3.14.3-6). Consequently, experience with these procedures is limited
(S3.14.3-5, S3.14.3-7), and heterogeneity makes generalizability difficult. Data are insufficient to support
recommendations. For all patients, survival mimics that for liver transplantation alone with 1-, 2-, and 5-
year survival at 84%, 74%, and 72%, respectively (S3.14.3-6). Outcomes in Fontan patients with or
without cirrhosis are not necessarily different in those who receive heart transplantation alone (S3.14.3-
5, S3.14.3-8). Multicenter data gathering on patients considered for multiorgan transplantation are
needed to inform future recommendations for these therapies.
3.15. Palliative Care
Recommendation for Palliative Care
IIa B-NR
1. Discussion of end-of-life issues and advance directives can be beneficial
for patients with ACHD or their surrogates (S3.15-1–S3.15-3).

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1. Patients with ACHD sometimes have significant morbidities not amenable to effective medical or
surgical treatment and may be best managed using the consultative expertise of palliative care
specialists. Accurate predictions of prognosis in ACHD are difficult, and patients commonly receive
aggressive treatments during their terminal admission (S3.15-4). There is a discrepancy between
patient-reported interest in discussing advanced directives and physician-reported discussions, with
more patients interested in such discussions than recognized by providers (S3.15-1, S3.15-2). Early
discussion of advance planning is favored by nearly twice as many patients as physicians (S3.15-3). Early
discussion of end-of-life issues is consistent with patient-centered care and patient satisfaction and can
facilitate palliative care. Although discussing end-of-life options would seem appropriate for all patients,
there are circumstances (e.g., cultural or cognitive) when those conversations may not be appropriate.
Similarly, although the goal is not to wait to discuss end-of-life until death is imminent, such discussion
may not have the same benefit for young patients who are clinically well with low-risk disease. Thus, it is
important to always have and encourage the option to discuss end-of-life issues, but timing of
conversation is individualized.
3.16. Cyanosis
The definition of cyanosis is “blueish discoloration of the skin and/or mucous membranes resulting from
inadequate oxygenation of the blood.” Generally, for cyanosis to be visible, at least 5 g/L of unsaturated
hemoglobin in tissue is needed (S3.16-1). Anemia may result in hypoxemia that is not manifest as
cyanosis. In this guideline, “cyanosis” is used as a generic term to identify hypoxemia caused by right-to-
left shunting of blood, but not all hypoxemic patients will be visibly cyanotic at all times.
Cyanotic heart disease encompasses a widely heterogeneous group; therefore, an individualized
approach is needed for each patient according to the clinical details.
Secondary erythrocytosis (a physiological increase in red blood cell mass in response to
hypoxemia) and polycythemia (a neoplastic proliferation of hematopoietic cells including the red blood
cell line) are fundamentally different conditions that require different treatments. In secondary
erythrocytosis, the patient’s own homeostatic processes generally direct achievement of an optimal
level of red cell mass, estimated by hemoglobin and hematocrit (S3.16-2).
Iron deficiency is frequently encountered in cyanotic individuals (S3.16-3). In addition to
contributing to symptoms, iron deficiency causes a reduction of hemoglobin without a proportional
change in hematocrit and thus compromises systemic oxygen transport without lowering viscosity
(S3.16-3). Symptoms mimic those of hyperviscosity. Consequences of iron deficiency may include stroke
and myocardial ischemia (S3.16-4–S3.16-6), although published findings are inconsistent. Iron deficiency
requires assessment of serum iron, ferritin, and transferrin levels, because mean corpuscular volume is
not a reliable screening test (S3.16-7). Limited data suggest that treatment of transferrin saturation
<20% with iron supplementation until iron stores are replete can be done safely (S3.16-8).
Although there is an exponential relationship between viscosity and hematocrit, available data
do not justify a cut point for a ”safe” hematocrit (S3.16-3). There is no clear correlation between
viscosity, iron deficiency, and a patient’s symptoms or clinical condition (S3.16-3). The nature and cause
of hyperviscosity symptoms are not well understood. The severity and frequency of symptoms of
hyperviscosity do not correlate with measured hematocrit. Phlebotomy is, therefore, rarely necessary in
patients with secondary erythrocytosis, and routine phlebotomy is not supported by data. Patients with
suspected hyperviscosity need to be rehydrated either with oral fluids or intravenous normal saline
solution as a first-line therapy, evaluated for iron deficiency, and treated if appropriate. Phlebotomy

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(with equal volume fluid replacement) is sometimes performed in special cases wherein, after adequate
hydration, hematocrit remains higher than the patient’s baseline and symptoms persist, or there is
evidence of end-organ damage attributable to hyperviscosity (e.g., myocardial ischemia, transient
ischemic attack/stroke) (S3.16-9, S3.16-10).
Observational studies in cyanotic individuals have shown evidence of altered synthesis and
function of clotting factors that may contribute to both hypo- and hypercoagulability (S3.16-11, S3.16-
12), and thrombosis and bleeding (particularly epistaxis or hemoptysis) have been described in patients
with Eisenmenger syndrome, which may be life-threatening (S3.16-13–S3.16-15). These disparate trends
preclude developing universally applicable recommendations, including use of antiplatelet or
anticoagulant therapy in these patients (S3.16-16). Similarly, there is not a clear role for preoperative
phlebotomy to improve coagulation properties.
Cyanotic heart disease is a multisystem disorder. Manifestations, in addition to those already
discussed, include renal dysfunction, gout, infections, and osteoarthropathy. Alterations can be found of
myocardial (S3.16-17, S3.16-18), cerebral (S3.16-19), and retinal blood flow (S3.16-20), and kidney
function (S3.16-21). Providers should recognize multiorgan susceptibility and avoid treatments that may
have adverse noncardiac effects. Additional practices that may contribute to effective management of
cyanotic patients are listed in Table 12.
Table 12. Specific Management Practices for Cyanotic CHD
• Recording clinical oxygen saturation at rest (>5 min) rather than immediately after effort (e.g., walking
into a clinic examination room).
• Meticulous intravenous care to avoid air or particulate matter, which may include use of air/particulate
filters on all intravenous access lines, when feasible, and careful de-airing of all lines.
• Cerebral imaging for any new headache or neurologic sign to assess for possible cerebral abscess,
hemorrhage, or stroke.
• Measurement of serum uric acid and treatment with allopurinol in a patient with a history of gout.
• Supplemental oxygen as needed for symptom relief but not to a target oxygen saturation level and not if
there is no demonstrable symptomatic benefit.
• Avoidance of or cautious use of therapies that may reduce the patient’s hypoxia-mediated drive to
ventilation, such as narcotics or, in rare circumstances, excess supplemental oxygen (S3.16-22).
• Anesthesia by providers with expertise in anesthesia for patients with ACHD for any noncardiac surgery.
• Non-estrogen–containing birth control for women of child-bearing potential (intrauterine device may be a
preferred option). Avoidance of birth control entirely is not a safe, acceptable option.
• Patients can travel safely on commercial airlines without undue risk (S3.16-23). Preflight simulation
testing or mandated supplemental oxygen are not usually indicated, although adequate hydration and
movement during the flight are appropriate.
• Measurement of coagulation parameters (e.g., activated partial thromboplastin time, international
normalized ratio, thrombin time) in a patient with an elevated hematocrit >55% requires adjustment of
anticoagulant volume in the blood collection vials to account for reduced plasma volume in the draw
(S3.16-24).
ACHD indicates adult congenital heart disease and CHD, congenital heart disease.

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3.17. Pharmacological Therapy for ACHD
Patients with ACHD are commonly excluded from clinical trials, and there are few data to guide
pharmacological therapies. Although it may be tempting to extrapolate from management guidelines
developed for patients without CHD (e.g., HF guidelines) (S3.17-1), treatments may not have the same
benefit in the heterogeneous population of patients with ACHD and in some cases may cause harm. The
evaluation of new symptoms in a patient with ACHD must be tailored to the patient’s anatomy, surgical
repair, and physiology. Before considering pharmacological therapies, evaluation for residual shunts,
baffle stenosis, valvular or conduit dysfunction, and collateral vessels, any of which may be amenable to
interventions, is an important consideration.
The literature documenting pharmacological therapies for patients with ACHD is limited to small
studies with limited duration of drug administration and follow-up. Additionally, the endpoints used are
often surrogate markers that have not been validated for clinical decision-making, and studies are also
often underpowered. However, studies in patients with ACHD do exist and evaluate conventional
pharmacological therapy, especially for HF and for arrhythmia, including beta blockers, angiotensin-
converting enzyme inhibitors, angiotensin-receptor blockers, and aldosterone antagonists, although
results vary (S3.17-2–S3.17-9).
Pharmacological therapies in patients with ACHD are often directed to specific conditions (i.e.,
beta blockers for arrhythmia treatment). However, there are limited data examining the benefits of beta
blockers in specific ACHD populations. Results from a small study indicate that beta-blocker therapy may
have potential to improve functional class in patients with a systemic right ventricle and a pacemaker
(S3.17-2). Angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers have also been
assessed in small studies in specific ACHD populations in which no significant benefit on ventricular
function or exercise capacity has been proven (S3.17-6–S3.17-8). Data from 1 small trial with a short
follow-up interval in patients with a systemic right ventricle suggest that eplerenone may be associated
with reduced myocardial fibrosis, as assessed by imaging (S3.17-3).
Some pharmacological therapies affecting the pulmonary vasculature (e.g., endothelin-receptor
antagonists and phosphodiesterase type-5 [PDE-5] inhibitors) have a beneficial effect on long-term
outcomes in patients with Eisenmenger syndrome (S3.17-10). Similarly, there are limited data on the use
of pulmonary vasodilator therapy in Fontan patients, in whom the pulmonary vascular resistance may
be abnormal (S3.17-11–S3.17-13). Because of the lack of data, clinical recommendations regarding
pharmacological therapy for patients with ACHD are unsupported. Individualized care is needed,
recognizing the potential benefits and risks of the therapy relative to patient-specific anatomic and
physiological issues.
4. Specific Lesions
4.1. Shunt Lesions

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4.1.1. Atrial Septal Defect
Recommendations for Atrial Septal Defect
Referenced studies that support recommendations are summarized in Online Data Supplement 26
and the systematic review report (S4.1.1-1).
Diagnostic
I C-EO
1. Pulse oximetry at rest and during exercise is recommended for
evaluation of adults with unrepaired or repaired ASD with residual shunt
to determine the direction and magnitude of the shunt.
I B-NR
2. CMR, CCT, and/or TEE are useful to evaluate pulmonary venous
connections in adults with ASD (S4.1.1-2–S4.1.1-4).
I B-NR
3. Echocardiographic imaging is recommended to guide percutaneous ASD
closure (S4.1.1-5, S4.1.1-6).
Therapeutic
I B-NRSR
4. In adults with isolated secundum ASD causing impaired functional
capacity, right atrial and/or RV enlargement, and net left-to-right shunt
sufficiently large to cause physiological sequelae (e.g., pulmonary–
systemic blood flow ratio [Qp:Qs] ≥1.5:1) without cyanosis at rest or
during exercise, transcatheter or surgical closure to reduce RV volume
and improve exercise tolerance is recommended, provided that systolic
PA pressure is less than 50% of systolic systemic pressure and pulmonary
vascular resistance is less than one third of the systemic vascular
resistance (S4.1.1-7–S4.1.1-12).
I B-NR
5. Adults with primum ASD, sinus venosus defect or coronary sinus defect
causing impaired functional capacity, right atrial and/or RV enlargement
and net left-to-right shunt sufficiently large to cause physiological
sequelae (e.g., Qp:Qs ≥1.5:1) without cyanosis at rest or during exercise,
should be surgically repaired unless precluded by comorbidities,
provided that systolic PA pressure is less than 50% of systemic pressure
and pulmonary vascular resistance is less than one third of the systemic
vascular resistance (S4.1.1-13, S4.1.1-14).
IIa C-LDSR
6. In asymptomatic adults with isolated secundum ASD, right atrial and RV
enlargement, and net left-to-right shunt sufficiently large to cause
physiological sequelae (e.g., Qp:Qs 1.5:1 or greater), without cyanosis at
rest or during exercise, transcatheter or surgical closure is reasonable to
reduce RV volume and/or improve functional capacity, provided that
systolic PA pressure is less than 50% of systemic pressure and pulmonary
vascular resistance is less than one third systemic resistance (S4.1.1-7–
S4.1.1-10, S4.1.1-12).
IIa C-LD
7. Surgical closure of a secundum ASD in adults is reasonable when a
concomitant surgical procedure is being performed and there is a net
left-to-right shunt sufficiently large to cause physiological sequelae (e.g.,
Qp:Qs 1.5:1 or greater) and right atrial and RV enlargement without
cyanosis at rest or during exercise (S4.1.1-15–S4.1.1-18).
IIb B-NR
8. Percutaneous or surgical closure may be considered for adults with ASD
when net left-to-right shunt (Qp:Qs) is 1.5:1 or greater, PA systolic

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pressure is 50% or more of systemic arterial systolic pressure, and/or
pulmonary vascular resistance is greater than one third of the systemic
resistance (S4.1.1-19, S4.1.1-20).
III:
Harm
C-LD
9. ASD closure should not be performed in adults with PA systolic pressure
greater than two thirds systemic, pulmonary vascular resistance greater
than two thirds systemic, and/or a net right-to-left shunt (S4.1.1-21,
S4.1.1-22).
Synopsis
ASDs are common and may occur as a consequence of different anatomic defects, including secundum
ASD, primum ASD, sinus venosus defect (not properly a defect in the atrial septum but considered in this
section), and coronary sinus septal defect. Left-to-right shunting may result in right heart enlargement
and RV dysfunction and, in a minority of patients, PAH. Some patients may have right-to-left shunting or
paradoxical embolism, and some may develop arrhythmias. Percutaneous device or surgical closure are
the mainstays of therapy in those with hemodynamic or clinical consequences of the defect. Severe PAH
is a contraindication to closure, and its presence must be accurately excluded before closure (S4.1.1-21–
S4.1.1-23).
ASD may occur with other congenital cardiac abnormalities. In some circumstances, such as in
patients with Ebstein anomaly and pulmonary stenosis (PS) or right HF, the physiology related to the
ASD is substantially more complex, and ASD closure could result in clinical deterioration. Therefore,
these recommendations regarding ASD address only isolated ASDs and not ASD associated with complex
CHD.
The “Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A
Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With
Congenital Heart Disease” (S4.1.1-1) has additional data and analyses. The results from the question
“are outcomes in asymptomatic patients with unoperated secundum ASD and RV dilatation improved
after percutaneous or surgical closure?” and the writing committee’s review of the totality of the
literature were used to frame decision-making. Recommendations that are based on a body of evidence
that includes the systematic review conducted by the ERC are denoted by the superscript SR (e.g., LOE
B-RSR
).
See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and
other procedures in these patients; Section 3.4 for recommendations on diagnostic evaluation; Section
4.4.6 for evaluation and management of severe PAH and Eisenmenger syndrome; and Figure 1 for a
diagnostic and treatment algorithm for secundum ASD. See Table 13 for routine follow-up and testing
intervals.
1. Pulse oximetry is useful in defining shunt direction at rest and with exercise, which will help guide
decisions regarding therapeutic options. Pulse oximetry at rest and with exercise may identify patients
with increased pulmonary arterial resistance and shunt reversal. In a subset of patients with resting
systemic oxygen saturation >90%, a decrease in oxygen saturation with activity to <90% may occur,
emphasizing the importance of performing resting and ambulatory pulse oximetry assessment.
2. TTE has limited use in assessment of anomalous pulmonary venous connections in adults with ASD.
Moreover, the poor visualization of the superior and posterior atrial septum by TTE in adults may
require testing with other imaging modalities to clearly define septal anatomy. TEE is excellent for
visualization of the entire atrial septum as well as pulmonary venous connections. Anomalous right

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upper and middle lobe pulmonary venous connections often occur in combination with superior sinus
venosus defect; TEE is excellent for visualization of this combination but may not visualize other
anomalous pulmonary venous connections. Cross-sectional imaging with CMR or CCT is ideal for
delineating pulmonary venous connections, particularly those that are associated with veins that may be
difficult or impossible to image by echocardiography (e.g., innominate vein or vertical vein). CMR has
the advantages of not involving ionizing radiation and ability to quantify degree of shunting.
3. It is considered standard of care to use echocardiographic imaging to guide closure of interatrial
communications. TEE and intracardiac echocardiography are the most widely studied and used
modalities for guidance of ASD closure. Defect size, defect morphology, atrial rim adequacy, pulmonary
venous anomalies, and left atrial appendage thrombus can all be evaluated using TEE. Echocardiography
is also used to determine sizing either by balloon diameter producing complete occlusion of the defect
(”stop flow” diameter) or by direct visualization and measurement using intracardiac echocardiography.
Echocardiography can assess for pericardial effusion and for thrombi on wires or devices. TTE has also
been studied for guiding percutaneous ASD closure but is not widely used for this purpose.
4. Cardiac catheterization is performed at the time of transcatheter ASD closure. Provided noninvasive
imaging is of sufficiently high quality to estimate pulmonary artery pressures and shunt magnitude, not
every patient with an ASD requires a diagnostic catheterization before surgical closure. However, a
diagnostic catheterization may be necessary to determine detailed hemodynamics for decision-making
or to clarify discrepant or inconclusive noninvasive imaging data. Patients with reduced functional
capacity presumed caused by hemodynamically important secundum ASD (moderate or large left-to-
right shunt and evidence of right heart volume overload in the absence of significant PAH) benefit from
surgical or transcatheter closure of the secundum ASD (S4.1.1-8, S4.1.1-10). Patients who do not
undergo ASD closure have worse long-term outcomes, including more atrial arrhythmias, reduced
functional capacity, and eventually greater degrees of PAH. Older adults should be evaluated for left
atrial hypertension resulting from diastolic dysfunction, which may cause similar symptoms but could
result in clinical worsening after ASD closure because of further increase in left atrial pressures when
blood from the relatively restrictive and higher pressure left atrium can no longer decompress into the
lower pressure right atrium. Cyanosis with exercise typically occurs in association with poor RV diastolic
compliance and hemodynamics with exercise, and the ASD acts as a “pop-off” to maintain cardiac
output. However, exercise-induced cyanosis is not an absolute contraindication to ASD closure, because
there are rare cases of either streaming or directed tricuspid regurgitation (TR) leading to right-to-left
shunting with exercise not related to abnormal RV diastolic pressures that may allow for closure after
expert evaluation. Data are most compelling that closure improves functional status, although some
descriptive studies support improved long-term outcomes after closure as well (S4.1.1-7–S4.1.1-12).
5. Available percutaneously deployed ASD closure devices are approved for closure of secundum-type
defects. Primum, sinus venosus, and coronary sinus ASDs should be closed surgically because of the
absence of appropriate rims for percutaneous device placement and the proximity of the
atrioventricular valves and conduction system to the closure device. Congenital heart surgeons are
trained in the nuances of repair of such defects, including common association with anomalous
pulmonary venous connection and abnormalities of the atrioventricular valves (S4.1.1-24, S4.1.1-25).
6. Patients who do not undergo ASD closure have worse long-term outcomes, including more atrial
arrhythmias, reduced functional capacity, and eventually greater degrees of PAH (S4.1.1-7–S4.1.1-10,
S4.1.1-12). However, concomitant diseases may influence the anticipated benefit of ASD closure in
ameliorating symptoms and improving functional capacity, and it has not been clearly demonstrated
that ASD closure in asymptomatic adults prevents long-term complications. Data suggest that ASD
closure improves functional capacity but, in patients with normal functional capacity, the long-term

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benefit of ASD closure is less clear (S4.1.1-1, S4.1.1-9). Pending further study, it is reasonable to close an
ASD that is hemodynamically important in the absence of significant PAH. Older adults should be
evaluated for left atrial hypertension resulting from diastolic dysfunction that may cause symptoms
simulating those from an ASD alone, in whom ASD closure could result in clinical worsening because of
further increase in left atrial pressure because the relatively restrictive and higher pressure left atrium
can no longer decompress into the lower pressure right atrium. Concomitant tricuspid annuloplasty can
be of benefit in patients with moderate or more TR, as the additional volume load may adversely affect
RV remodeling.
7. If surgical treatment is necessary for other congenital or acquired cardiac conditions and the patient
has a secundum ASD, it is reasonable to perform ASD closure at the time of surgery. When there is
moderate or greater TR, tricuspid valve repair may improve RV remodeling.
8. To evaluate the patient with PAH and ASD, ensure the shunt remains left to right despite elevated
pulmonary vascular resistance and/or pulmonary pressure and that pulmonary pressure and PVR are
accurately measured. In this circumstance, data derived from invasive hemodynamic assessment are
important in clarifying the appropriate course of action. The exclusion of patients with severe PAH from
ASD closure may eventually be obviated by PA vasodilator and remodeling therapy with prostaglandins,
endothelin blockers, and PDE-5 inhibitors. Because of the complexity of the hemodynamics in such
patients, collaboration between ACHD and pulmonary hypertension providers is important.
Pretreatment with PAH therapies and pulmonary arterial remodeling agents, with a demonstrated
reduction in pulmonary arterial resistance of >20%, portends a favorable prognosis after ASD closure
(S4.1.1-26).
9. Morbidity and mortality are prohibitively high when surgical repair is attempted in patients with open
shunts, such as ASD when Eisenmenger syndrome is present (S4.1.1-21, S4.1.1-22).
Table 13. ASD: Routine Follow-Up and Testing Intervals
Frequency of Routine Follow-Up
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
Outpatient ACHD cardiologist 36–60 24 6–12 3–6
ECG 36–60 24 12 12
TTE 36–60 24 12 12
Pulse oximetry As needed As needed Each visit Each visit
Exercise test† As needed As needed 12–24 6–12
†6-minute walk test or CPET, depending on the clinical indication.
ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE,
transthoracic echocardiogram.

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Figure 1. Secundum ASD
*Combination therapy with bosentan and PDE-5 inhibitor if symptomatic improvement does not occur with either alone.
ACHD indicates adult congenital heart disease; ASD, atrial septal defect; PAH, pulmonary artery hypertension; PASP, pulmonary
artery systolic pressure; PDE-5, phosphodiesterase type-5 inhibitors; PH, pulmonary hypertension; and Qp:Qs, pulmonary–
systemic blood flow ratio.
4.1.2. Anomalous Pulmonary Venous Connections
Recommendations for Anomalous Pulmonary Venous Connections
Diagnostic
I B-NR
1. CMR or CTA is recommended for evaluation of partial anomalous
pulmonary venous connection (S4.1.2-1–S4.1.2-4).
IIa B-NR
2. Cardiac catheterization can be useful in adults with partial anomalous
pulmonary venous connection to further define hemodynamics (S4.1.2-5,
S4.1.2-6).
Therapeutic
I B-NR
3. Surgical repair is recommended for patients with partial anomalous
pulmonary venous connection when functional capacity is impaired and

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RV enlargement is present, there is a net left-to-right shunt sufficiently
large to cause physiological sequelae (e.g., Qp:Qs ≥1.5:1), PA systolic
pressure is less than 50% systemic pressure, and pulmonary vascular
resistance is less than one third of systemic resistance (S4.1.2-5).
I B-NR
4. Repair of partial anomalous pulmonary venous connection is
recommended at the time of closure of a sinus venosus defect or ASD
(S4.1.2-7).
I B-NR
5. Repair of a scimitar vein is recommended in adults when functional
capacity is impaired, evidence of RV volume overload is present, there is
a net left-to-right shunt sufficiently large to cause physiological sequelae
(e.g., Qp:Qs ≥1.5:1), PA systolic pressure is less than 50% systemic
pressure and pulmonary vascular resistance is less than one third
systemic (S4.1.2-5, S4.1.2-8, S4.1.2-9).
IIa B-NR
6. Surgery can be useful for right- or left-sided partial anomalous
pulmonary venous connection in asymptomatic adults with RV volume
overload, net left-to-right shunt sufficiently large to cause physiological
sequelae (e.g., Qp:Qs ≥1.5:1), pulmonary pressures less than 50%
systemic and pulmonary vascular resistance less than one third systemic
(S4.1.2-5).
IIa B-NR
7. Surgery can be useful for repair of a scimitar vein in adults with evidence
of RV volume overload, with Qp:Qs 1.5:1 or greater (S4.1.2-5, S4.1.2-9).
Synopsis
Abnormal connection between a pulmonary vein and systemic vein will result in volume overload of the
right heart, with a physiological effect similar to that of an ASD. However, in the absence of an
associated ASD, anomalous pulmonary venous connection differs in that there is no potential for right-
to-left shunting, and the magnitude of the left-to-right shunt is not exacerbated by the development of
acquired left heart disease. The most common anomalous pulmonary venous connection is of the right
upper pulmonary vein to the superior vena cava (S4.1.2-10), which may be associated with a sinus
venosus defect. Other abnormal connections include right pulmonary vein(s) to the inferior vena cava
(often via a so-called ”scimitar vein” and associated with sequestration of the right lower lobe), left
upper pulmonary vein(s) to the left innominate vein, and right upper pulmonary vein(s) connecting high
on the superior vena cava. Long-term sequelae of anomalous pulmonary venous connections reflect the
impact of right heart volume overload and are similar to the sequelae of ASDs. Surgical repair can be
challenging as low-velocity venous flow imparts risk of thrombosis of the surgically operated vein.
other procedures in these patients; Section 3.4 for recommendations on diagnostic evaluation; and
Section 4.4.6 for evaluation and management of severe PAH and Eisenmenger syndrome.
1. Cross-sectional imaging with CMR or CTA is ideal for delineating pulmonary venous connections. CMR
has the advantage of not using ionizing radiation and may also quantify the degree of shunting.
Echocardiography is an important part of the evaluation and may identify the anomalous veins (S4.1.2-
11), particularly in patients with excellent acoustic windows; however, CMR and CTA are superior for
evaluating extracardiac vascular anatomy.
2. In higher-risk patients, invasive hemodynamic assessment can be useful for direct measurement of
pressures, quantification of shunt magnitude, and measurement of pulmonary arterial resistance and

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responsiveness to pulmonary vasodilator therapy. Invasive hemodynamic assessment is especially
important in adult patients who are being considered for surgical correction.
3. It is unusual for a single anomalous pulmonary venous connection of only 1 pulmonary lobe to result
in a sufficient volume load to justify surgical repair. However, if a patient has symptoms referable to the
shunt, there is >1 anomalous vein, and a moderate or large left-to-right shunt, then surgical repair is
associated with a reduction in RV size and PA pressure (S4.1.2-5). Pulmonary hypertension is a risk for
adverse outcomes with surgery.
4. Surgery usually involves intracaval baffling into the left atrium, Warden procedure (S4.1.2-12), or
direct reimplantation of the anomalous pulmonary vein directly into the left atrium.
5. Surgical repair of a scimitar vein includes direct reimplantation of the scimitar vein into the left
atrium, conduit placement to the left atrium, or intracaval baffling. This surgery can be technically
challenging with a greater risk of postoperative vein thrombosis than is associated with more common
and simpler anomalous pulmonary vein abnormalities (S4.1.2-10). Pulmonary hypertension is associated
with poor outcomes.
6. It is unusual for a single anomalous pulmonary venous connection from only one pulmonary lobe to
result in a sufficient volume load to justify surgical repair. However, if there is >1 anomalous vein and a
moderate or large left-to-right shunt, then surgical repair is associated with a reduction in RV size and
PA pressure and can be useful (S4.1.2-5).
7. Surgical repair of a scimitar vein includes direct reimplantation of the scimitar vein into the left
atrium, side-to-side anastomosis of the scimitar vein to the left atrium and closure of its connection to
the inferior vena cava or intracaval baffling. This surgery can be technically challenging with a greater
risk of postoperative vein thrombosis than is associated with simpler anomalous pulmonary vein
abnormalities (S4.1.2-10).
4.1.3. Ventricular Septal Defect
Recommendations for Ventricular Septal Defect
Therapeutic
I B-NR
1) Adults with a VSD and evidence of left ventricular volume overload and
hemodynamically significant shunts (Qp:Qs ≥1.5:1) should undergo VSD
closure, if PA systolic pressure is less than 50% systemic and pulmonary
vascular resistance is less than one third systemic (S4.1.3-1).
IIa C-LD
2) Surgical closure of perimembranous or supracristal VSD is reasonable in
adults when there is worsening aortic regurgitation (AR) caused by VSD
(S4.1.3-1, S4.1.3-2).
IIb C-LD
3) Surgical closure of a VSD may be reasonable in adults with a history of IE
caused by VSD if not otherwise contraindicated (S4.1.3-3).
IIb C-LD
4) Closure of a VSD may be considered in the presence of a net left-to-right
shunt (Qp:Qs ≥1.5:1) when PA systolic pressure is 50% or more than
systemic and/or pulmonary vascular resistance is greater than one third
systemic (S4.1.3-4–S4.1.3-6).
III:
Harm
C-LD
5) VSD closure should not be performed in adults with severe PAH with PA
systolic pressure greater than two thirds systemic, pulmonary vascular

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resistance greater than two thirds systemic and/or a net right-to-left
shunt (S4.1.3-7–S4.1.3-9).
Synopsis
Ventricular septal defects (VSDs) initially create a volume load to the left heart, and the magnitude of
hemodynamic impact is directly related to the size of the shunt and afterload to the ventricles. Isolated
VSDs are the most commonly encountered form of CHD in the pediatric population (S4.1.3-10– S4.1.3-
14). Most isolated muscular and perimembranous VSDs are small and close spontaneously. The
spectrum of isolated residual VSDs encountered in the adult patient includes:
1. Small restrictive defects. The pulmonary vascular resistance is not significantly elevated and the
left-to-right shunt is small (Qp:Qs <1.5:1).
2. Large nonrestrictive defects in cyanotic patients who have developed Eisenmenger syndrome,
with pulmonary vascular resistance at systemic levels and shunt reversal (right-to-left).
3. Patients with moderately restrictive defects (Qp:Qs ≥1.5:1 and <2:1) who have not undergone
closure for some reason. These patients often have mild-to-moderate PAH.
4. Patients who have had their defects closed in childhood. These patients may have VSD patch
leaks.
4.4.6 for evaluation and management of severe PAH and Eisenmenger syndrome; Figure 2 for a
diagnostic and treatment algorithm for ventricular level shunt; and Table 14 for routine testing and
follow-up intervals.
1. In the absence of aortic valve prolapse and regurgitation or IE, small restrictive defects of the
muscular or membranous septum may be watched conservatively without need for operative
intervention. In a long-term follow-up registry, the overall survival rate was 87% for all patients with
unoperated VSD at 25 years (S4.1.3-1). For patients with small defects (Qp:Qs <1.5:1 and low PA
pressure), the survival rate was 96%. Patients with moderate and large defects fared worse with 25-year
survival of 86% and 61%, respectively. Those with Eisenmenger syndrome (cyanosis/hypoxemia caused
by reversal of shunt to right-to-left) had a much lower 25-year survival (42%). Larger defects may be
repaired but only in the absence of severe PAH and severely elevated pulmonary vascular resistance, the
presence of which incurs a high perioperative risk S4.1.3-15).
Life expectancy after VSD closure in an adult is not normal but has improved over the past 50
years. Transcatheter device occlusion of muscular and perimembranous VSD is feasible, and trials have
demonstrated a good safety and efficacy profile (S4.1.3-16, S4.1.3-17). VSD in adults is most commonly
either small, or large and associated with Eisenmenger syndrome; therefore, data regarding optimal
management of moderate VSD in adults are lacking because of relative infrequency of a
hemodynamically significant VSD for which closure is an option.
2. Small restrictive defects of the muscular or membranous septum may be managed by observation
without need for operative intervention. However, 6% of patients with small supracristal (subaortic) or
perimembranous defects may develop aortic valve prolapse and resultant AR that may be progressive
(S4.1.3-1, S4.1.3-2, S4.1.3-18). There is a paucity of data supporting the timing of VSD closure in patients
with AR. Ideally, the VSD is closed if AR is progressive to avoid the continued worsening of AR and the
need for aortic valve replacement. In the presence of a VSD, an aortic valve cusp (usually the right
coronary cusp) may prolapse and partially or completely close the VSD, often with associated AR. At the

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time of VSD closure, aortic valve repair may be performed in an effort to stabilize or improve AR. For
patients who meet GDMT criteria for aortic valve replacement, this may be performed concomitant with
VSD closure (S4.1.3-19).
3. In patients with unrepaired VSD, there is an increased risk of IE, typically involving the tricuspid and
pulmonic valves.
4. Early attempts at surgical closure of nonrestrictive VSD in patients with Eisenmenger syndrome were
associated with an unacceptably high risk of mortality, and the practice was quickly abandoned.
However, there are adult patients with large VSD and PAH who may benefit from closure of the VSD if
the net shunt is left-to-right either at baseline or with PAH therapies. The use of fenestrated devices and
fenestrated surgical patches in these patients leaves a small residual shunt to allow decompression of
the right heart (S4.1.3-5, S4.1.3-6). In theory, treatment of these patients with PAH therapies before
closure could improve outcomes.
5. Closure of nonrestrictive VSD in adults with Eisenmenger syndrome who do not demonstrate left-to-
right shunting and a decline in pulmonary vascular resistance with PAH therapies carries a high risk of
mortality and should not be performed (S4.1.3-7- S4.1.3-9).
Table 14. VSD: Routine Follow-Up and Testing Intervals
and Testing
Physiological Stage
A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
Outpatient ACHD cardiologist 36 24 6–12 3–6
ECG 36 24 12 12
TTE 36 24 12 12
†6-minute walk test or CPET, depending on the clinical circumstance.
ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; TTE, transthoracic
echocardiogram; and VSD, ventricular septal defect.

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Figure 2. Hemodynamically Significant Ventricular Level Shunt
*Combination therapy with bosentan and PDE-5 inhibitor, if symptomatic improvement does not occur with either alone.
ACHD indicates adult congenital heart disease; AR, aortic regurgitation; IE, infective endocarditis; LV, left ventricular; PAH,
pulmonary artery hypertension; PASP, pulmonary artery systolic pressure; PDE-5, phosphodiesterase type-5 inhibitors; PH,
pulmonary hypertension; Qp:Qs, pulmonary–systemic blood flow ratio; and VSD, ventricular septal defect.
4.1.4. Atrioventricular Septal Defect
Recommendations for Atrioventricular Septal Defect
Diagnostic
IIa C-EO
1. Cardiac catheterization can be useful in adults with atrioventricular
septal defect when pulmonary hypertension is suspected.
Therapeutic

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I C-LD
2. Surgery for severe left atrioventricular valve regurgitation is
recommended per GDMT indications for mitral regurgitation (S4.1.4-1–
S4.1.4-4).
I C-EO
3. Surgery for primary repair of atrioventricular septal defect or closure of
residual shunts in adults with repaired atrioventricular septal defect is
recommended when there is a net left-to-right shunt (Qp:Qs ≥1.5:1), PA
systolic pressure less than 50% systemic and pulmonary vascular
resistance less than one third systemic.
IIa C-EO
4. Operation for discrete LVOT obstruction in adults with atrioventricular
septal defect is reasonable with a maximum gradient of 50 mm Hg or
greater, a lesser gradient if HF symptoms are present, or if concomitant
moderate-to-severe mitral or AR are present.
IIb C-EO
residual shunts in adults with repaired atrioventricular septal defect may
be considered in the presence of a net left-to-right shunt (Qp:Qs ≥1.5:1),
if PA systolic pressure is 50% or more systemic, and/or pulmonary
vascular resistance is greater than one third systemic.
III:
Harm
C-LD
residual shunts in adults with repaired atrioventricular septal defect
should not be performed with PA systolic pressure greater than two
thirds systemic, pulmonary vascular resistance greater than two thirds
systemic, or a net right-to-left shunt (S4.1.4-5, S4.1.4-6).
Synopsis
AVSDs represent about 4% to 5% of congenital heart defects and include a primum ASD, inlet VSD, and
common atrioventricular valve. They can occur in several anatomic variations including partial AVSD
with only a primum ASD component and typically a cleft left atrioventricular valve, complete AVSD with
both ASD and VSD and a common atrioventricular valve, and transitional and intermediate AVSD with
incomplete atrial and VSDs and/or incomplete abnormalities of the common atrioventricular valve.
AVSD anatomy is also commonly described by the Rastelli classification (S4.1.4-7, S4.1.4-8). The Rastelli
classification describes anatomic variations of the superior bridging leaflet of the atrioventricular valve.
In addition to the Rastelli classification or other similar descriptors, the relative sizes of the ventricles as
balanced or unbalanced guide the type of repair (e.g., biventricular or single ventricle repair). This
section refers to patients with balanced AVSD and biventricular repair. AVSD also occurs in association
with other congenital lesions including TOF, CoA, and heterotaxy. There is also a strong association with
syndromes, most commonly trisomy 21 (Down syndrome).
From a management perspective, most adults with AVSD will have had surgical repair as
children. If those with complete AVSD (with large ASD and VSD) are not repaired early in life (typically <6
months of age), irreversible pulmonary vascular disease usually develops resulting in Eisenmenger
physiology, precluding complete repair. For those who underwent a surgical repair, long-term follow-up
is required to monitor for left atrioventricular valve regurgitation and stenosis, left ventricular outflow
tract (LVOT) obstruction attributable to the abnormal shape of the LVOT, and tachyarrhythmias and
bradyarrhythmias. Left atrioventricular valve regurgitation is the most common reason for later surgical
reintervention. There are few long-term follow-up studies of patients after AVSD repair in childhood, so
the most effective and efficient timing and type of surveillance are still being evaluated.
The atrioventricular node is typically displaced inferiorly in AVSD and is associated with relative
hypoplasia of the left anterior fascicle (S4.1.4-9). Late-onset complete heart block (as late as 15 years

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after surgery) has been noted after surgery in patients operated on for AVSD who were discharged from
the hospital with normal conduction, although more commonly seen in those patients with transient
postoperative heart block. Regular monitoring for symptoms and screening with an ECG are important
to evaluate for conduction abnormalities (S4.1.4-10).
4.1.1 for recommendations on primum ASD; Section 4.4.6 for evaluation and management of severe
PAH and Eisenmenger syndrome associated with AVSD; and Table 15 for routine testing and follow-up
intervals.
1. Invasive hemodynamic assessment still has an important role as a confirmatory tool and for the
evaluation of pulmonary vasoreactivity, which does carry prognostic significance for adults with shunts.
2. Although the left atrioventricular valve in an AVSD malformation is not anatomically the same as a
mitral valve, one can extrapolate the criteria for consideration of left atrioventricular valve surgery from
the VHD guideline for mitral regurgitation and mitral stenosis (S4.1.4-1). In extrapolating these criteria,
there are important potential differences in this patient population compared with those with acquired
mitral valve disease. There are anatomic differences in position of the annulus, papillary muscles and the
morphology of the LVOT, which is an anterior, narrow, and potentially obstructed structure, such that
congenital surgical expertise is needed. Patients with an AVSD have typically had at least 1 prior attempt
to repair the AVSD, have different risks of arrhythmia, and may have other anatomic lesions (e.g.,
subaortic stenosis [subAS]). In 1 meta-analysis of studies of adult left atrioventricular valve surgery in
patients with AVSD, the risk of needing a pacemaker was higher in those who underwent valve
replacement than in those who underwent repair (S4.1.4-2). In another single-center study, one third of
repaired patients required an additional reoperation (S4.1.4-3). When replacement is required, the
choice to use mechanical versus bioprosthetic valve is individualized, but a mechanical valve is usually
necessary because of the potential for LVOT obstruction from the struts of the bioprosthetic valve.
Nevertheless, valve repair is preferred to valve replacement when it is technically feasible.
3. There are no large studies on residual shunts in patients with AVSD, but extrapolating from
information on residual isolated ASD or isolated VSD, a moderate or large residual shunt is likely to
result in worsening clinical status over time and thus merits consideration of repair (S4.1.4-11–S4.1.4-
13). See Sections 4.1.1 and 4.1.3 for related considerations regarding ASD and/or VSD. Pulse oximetry at
rest and with ambulation may identify patients with increased pulmonary resistance and shunt reversal.
There is a subset of patients with resting systemic oxygen saturation >90% who will have a decrease in
oxygen saturation with activity to <90%, emphasizing the importance of performing resting and
ambulatory pulse oximetry assessment.
4. Patients with AVSD are at risk of LVOT obstruction because of the abnormal anatomy of the LVOT.
Surgical resection of LVOT obstruction in association with AVSD is reasonable when there is moderate-
to-severe obstruction or less obstruction but associated HF or mitral regurgitation or AR. In isolated
subAS studies, worse outcomes were revealed in patients with maximum gradients ≥50 mm Hg or with
gradients <50 mm Hg in association with symptoms of HF (S4.1.4-14–S4.1.4-17). Importantly the LVOT
obstruction in AVSD may not be discrete and, therefore, surgical repair may be more complex. When
evaluating patients with tunnel-like or complex LVOT obstruction, the peak Doppler gradients and
Bernoulli equation may inaccurately reflect the severity of obstruction, and cardiac catheterization may
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5. Patients with AVSD, particularly those with Down syndrome, are at high risk of developing pulmonary
vascular disease resulting in Eisenmenger syndrome (S4.1.4-18, S4.1.4-19). For those who continue to
have a net left-to-right shunt despite elevated PA pressures, closure of the defect may prevent
exacerbation of PAH. This is an unusual circumstance and decision-making requires collaboration with
ACHD and pulmonary hypertension providers.
6. Morbidity and mortality are prohibitively high when surgical repair is attempted in patients with open
shunts such as AVSD when Eisenmenger syndrome is present (S4.1.4-5, S4.1.4-6).
Table 15. AVSD: Routine Follow-Up and Testing Intervals
and Testing
Physiological Stage
A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 24–36 24 12 12
TTE 24–36 24 12 12
ACHD indicates adult congenital heart disease; AVSD, atrioventricular septal defect; CPET, cardiopulmonary exercise testing;
ECG, electrocardiogram; and TTE, transthoracic echocardiogram.
4.1.5. Patent Ductus Arteriosus
Recommendations for Patent Ductus Arteriosus
Diagnostic
I C-EO
1. Measurement of oxygen saturation should be performed in feet and both
hands in adults with a PDA to assess for the presence of right-to-left
shunting.
IIa C-EO
2. In addition to the standard diagnostic tools, cardiac catheterization can
be useful in patients with PDA and suspected pulmonary hypertension
(Section 3.5).
Therapeutic
I C-LD
3. PDA closure in adults is recommended if left atrial or LV enlargement is
present and attributable to PDA with net left-to-right shunt, PA systolic
pressure less than 50% systemic and pulmonary vascular resistance less
than one third systemic (S4.1.5-1–S4.1.5-3).
IIb B-NR
4. PDA closure in adults may be considered in the presence of a net left-to-
right shunt if PA systolic pressure is 50% or greater systemic, and/or
pulmonary vascular resistance is greater than one third systemic (S4.1.5-
3, S4.1.5-4).
III:
Harm
C-LD
5. PDA closure should not be performed in adults with a net right-to-left
shunt and PA systolic pressure greater than two thirds systemic or
pulmonary vascular resistance greater than two thirds systemic (S4.1.5-
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Synopsis
The ductus arteriosus is a vascular connection between the aorta and PA that is present in fetal life. It
typically closes shortly after birth but, in some people, it will remain patent. Patent ductus arteriosus
(PDA) is found in about 0.3% to 0.8% of term infants and is twice as common in females as males
(S4.1.5-6–S4.1.5-8). The clinical and physiological manifestations of the PDA are dependent on the size
of the vessel and the relative systemic and pulmonary vascular resistances. The PDA can range from a
small hemodynamically insignificant lesion that is not heard on auscultation to one that without
intervention is large enough to cause congestive HF and pulmonary hypertension. Many PDAs are now
closed in infancy or childhood with catheter-based or surgical approaches. For those whose ductus
remains patent in adulthood, catheter-based or surgical intervention consideration depends on the
symptoms and physiological expression of the lesion. Follow-up of these patients as adults is important
for all, although timing and testing will vary among individuals.
4.4.6 for recommendations on severe PAH (4.4.6.1) and Eisenmenger syndrome (4.4.6.2) associated with
PDA; and Table 16 for routine testing and follow-up intervals.
1. Because cyanosis caused by right-to-left shunting in PDA may manifest predominantly downstream
from the ductal insertion into the aorta, accurate assessment of oxygen saturation by oximetry and
assessment of cyanosis should be done in the feet and both hands. As with other types of shunts, pulse
oximetry with ambulation as well as at rest may identify patients with increased pulmonary arterial
resistance and dynamic shunt reversal induced by exercise. A subset of patients with resting systemic
oxygen saturation >90% will have a decrease in oxygen saturation with activity to <90%, emphasizing the
importance of performing resting and ambulatory pulse oximetry assessment.
2. Invasive hemodynamic assessment still has an important role as a confirmatory tool and for the
evaluation of pulmonary vasoreactivity, which carries prognostic significance (S4.1.5-1, S4.1.5-4).
3. When signs of volume overload are indicative of significant left-to-right shunt, closing the PDA is likely
to prevent further left atrial or LV enlargement, progression or development of PAH, and pulmonary
hypertension secondary to left HF and will possibly provide symptom relief if symptoms are present.
Closure is typically performed percutaneously with good success and minimal complications (S4.1.5-2).
Pulmonary blood flow and thus Qp:Qs can be difficult to calculate accurately because of differences in
right/left PA blood flow caused by the flow from the PDA. Invasive hemodynamics including pulmonary
vascular resistance are generally relied on for decision-making. Surgical closure can be performed but is
potentially hazardous in adults because of calcification and tissue fragility.
4. Even with elevated pulmonary pressure and elevated pulmonary vascular resistance, closure of a PDA
may improve clinical status in some patients with persistent left-to-right shunting and prevent further
progression of PAH (S4.1.5-3, S4.1.5-4). Consultation with ACHD and pulmonary hypertension providers
is important given the low frequency of this circumstance and the complexity of decision-making.
5. Morbidity and mortality are high when closure of a shunt is attempted in patients with Eisenmenger
physiology with elevated pulmonary pressure and net right-to-left shunting (S4.1.5-5).
Table 16. PDA: Routine Follow-Up and Testing Intervals
Frequency of Routine Follow-Up Physiological Physiological Physiological Physiological

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and Testing Stage A* (mo) Stage B* (mo) Stage C* (mo) Stage D* (mo)
ECG 36–60 24 12 12
TTE 36–60 24 12 12
Pulse oximetry† As needed As needed Each visit Each visit
Exercise test‡ As needed As needed 12–24 6–12
†Upper and lower extremity.
‡6-minute walk test or CPET, depending on the clinical indication.
ACHD indicates adult congenital heart disease; ECG, electrocardiogram; CPET, cardiopulmonary exercise test; PDA, patent
ductus arteriosus; and TTE, transthoracic echocardiogram.
4.2. Left-Sided Obstructive Lesions
4.2.1. Cor Triatriatum
Recommendations for Cor Triatriatum
Diagnostic
I B-NR
1. Adults presenting with cor triatriatum sinister should be evaluated for
other congenital abnormalities, particularly ASD, VSD, and anomalous
pulmonary venous connection (S4.2.1-1).
IIa B-NR
2. In adults with prior repair of cor triatriatum sinister and recurrent
symptoms, it is reasonable to evaluate for pulmonary vein stenosis
(S4.2.1-2).
Therapeutic
I B-NR
3. Surgical repair is indicated for adults with cor triatriatum sinister for
symptoms attributable to the obstruction or a substantial gradient across
the membrane (S4.2.1-3)
Synopsis
Cor triatriatum occurs when a membrane divides either the left atrium (sinister), or right atrium
(dexter). Cor triatriatum sinister is usually associated with other congenital malformations, specifically
ASD, VSD, or anomalous pulmonary venous connection (partial or total) (S4.2.1-1–S4.2.1-4). The left
atrial appendage is invariably in the same chamber as the mitral valve, separated from the pulmonary
veins by the membrane. Supravalvular mitral stenosis is typically caused by a fibrous ring on the atrial
side of the mitral valve, separating the mitral valve from both the left atrial appendage and the
pulmonary veins. The finding will have similar physiology to cor triatriatum and similar indications for
intervention. It can be associated with an abnormal mitral valve that may also require intervention.
Supravalvular mitral stenosis often comprises one part of a more complex sequence of serial left-sided
inflow and outflow obstructions (i.e., Shone complex).
other procedures in these patients; and Section 3.4 for recommendations on diagnostic evaluation.
1. Cor triatriatum sinister is a membrane spanning the left atrium. Surgery has been largely successful
with relatively few early or late deaths, which are usually attributable to associated congenital

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abnormalities (S4.2.1-4). The gradient across the defect at the time of surgery was at least 8 mm Hg
(mean 17 mm Hg; range 8 to 40 mm Hg) (S4.2.1-3). After repair, recurrence of stenosis is not expected.
Although pulmonary vein stenosis has been demonstrated before and after surgery (S4.2.1-2), it is not
usually progressive over time and has not been associated with PAH.
2. Pulmonary venous stenosis has been demonstrated before and after surgery, but it is not usually
progressive over time and has not been associated with PAH.
3. Although risks of isolated cor triatriatum sinister surgery is low, it should be performed when there is
evidence of a substantial gradient. In 1 series, the mean gradient at the time of surgical repair was at
least 8 mm Hg (S4.2.1-3). It is conceivable that on occasion, clinical circumstances (i.e., symptoms,
arrhythmia) would warrant intervention in patients with lower gradients.
4.2.2. Congenital Mitral Stenosis
Recommendation for Congenital Mitral Stenosis
I B-NR
1. Adults with congenital mitral stenosis or a parachute mitral valve should
be evaluated for other left-sided obstructive lesions (S4.2.2-1, S4.2.2-2).
Synopsis
Congenital mitral valve disease may be anatomically complex and is often accompanied by other lesions.
Indications for intervention in mitral stenosis are described in the 2014 VHD guideline (S4.2.2-3) and
apply to those patients with congenital mitral stenosis. Balloon mitral valvuloplasty is rarely, if ever,
indicated or effective in congenital mitral stenosis.
Table 17 for routine testing and follow-up intervals.
1. Parachute mitral valve is most commonly found in the presence of other congenital abnormalities
such as the components of Shone complex. Recurrence and progression of the various associated lesions
are expected, subsequent surgeries are common, and mortality may be associated with other defects
(S4.2.2-2). Therefore, these patients require follow-up at a center where such abnormalities can be
followed and future interventions considered. Choices and techniques for valve repair or replacement
are based on consideration of coexisting abnormalities including the likelihood of future surgery.
Table 17. Congenital Mitral Stenosis: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 24 24 12 12
TTE 24 24 12 12
Exercise test† As needed 24 24 12

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4.2.3. Subaortic Stenosis
Recommendations for Subaortic Stenosis
Diagnostic
IIb C-LD
1. Stress testing for adults with LVOT obstruction to determine exercise
capacity, symptoms, electrocardiographic changes, or arrhythmias may
be reasonable in the presence of otherwise equivocal indications for
intervention (S4.2.3-1, S4.2.3-2).
Therapeutic
I C-EO
2. Surgical intervention is recommended for adults with subAS, a maximum
gradient 50 mm Hg or more and symptoms attributable to the subAS.
I C-LD
3. Surgical intervention is recommended for adults with subAS and less
than 50 mm Hg maximum gradient and HF or ischemic symptoms, and/or
LV systolic dysfunction attributable to subAS (S4.2.3-3).
IIb C-LD
4. To prevent the progression of AR, surgical intervention may be
considered for asymptomatic adults with subAS and at least mild AR and
a maximum gradient of 50 mm Hg or more (S4.2.3-4–S4.2.3-6).
Synopsis
SubAS may occur as a discrete membrane below the aortic valve in the LVOT, as a longer tunnel-like
obstruction, as a consequence of chordal attachments in patients with abnormalities such as AVSD, or
because of surgical repairs involving VSD baffled to a transposed aorta, such as seen in the Rastelli
operation. SubAS may occur in isolation or as part of a suite of abnormalities. In adults with Shone
complex or its variants, subAS may be one of several LV obstructive lesions, including variants of
congenital mitral stenosis, supravalvular mitral stenosis, valvular aortic stenosis, supravalvular aortic
stenosis, and CoA (S4.2.3-7).
SubAS tends to recur, particularly when initial resection is needed in childhood. Surgical repair
for subAS carries a 10% to 15% risk of complete heart block (S4.2.3-6). SubAS may be first diagnosed in
adulthood and may be confused with hypertrophic obstructive cardiomyopathy when LV hypertrophy of
sufficient severity has developed such that the subaortic membrane is less evident on imaging.
The recommendations in this guideline apply to subAS caused by a discrete membrane or
tunnel-like obstruction. Similar principles may apply to more complex causes of subAS, but insufficient
data exist to support recommendations for more complex lesions, and extrapolation needs to take the
additional anatomic complexity into account.
Turbulent flow created distal to the subaortic obstruction may cause barotrauma to the
adjacent aortic valve leaflets and result in progressive AR, which may itself become clinically significant.
Resection of the subaortic obstruction ideally delays or prevents the eventual need for aortic valve
replacement, and concomitant aortic valve repair could also help delay the need for aortic valve
replacement in these cases.

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1. Exercise stress testing may be reasonable in the assessment of exercise capacity, stress-induced
arrhythmias, and ischemia in patients with subAS and may be considered as an adjunct to
echocardiographic imaging.
2. Patients with symptomatic subAS should attain symptomatic improvement from surgical relief of the
obstruction. In some cases, concomitant AVR may be needed, if indicated according to GDMT.
3. Patients with depressed LV systolic function and severe subAS may not manifest a resting gradient of
≥50 mm Hg. In this population, evaluation and decisions regarding surgical relief of LVOT obstruction can
be extrapolated from the existing aortic stenosis data and should be considered as per the 2014 VHD
guideline (S4.2.3-8). Additionally, patients with preserved LV systolic function but poor LV compliance
may present with signs or symptoms of HF and a resting maximum gradient <50 mm Hg. These patients
may benefit from surgical relief of LVOT obstruction. Patients with evidence of resting or stress-induced
ischemia in the absence of obstructive coronary artery disease and in the presence of moderate subAS
(maximum gradient >30 mm Hg and <50 mm Hg) may benefit from surgical relief of subAS (S4.2.3-9).
4. Discrete subAS tends to be progressive with age, and patients with a resting maximum gradient ≥50
mm Hg are more likely to have progressive subAS and concomitant moderate or severe aortic valve
regurgitation (S4.2.3-4). Therefore, surgical intervention may be considered in the asymptomatic patient
with severe subAS. Tunnel-type subAS, which is often associated with a small aortic valve annulus, is
associated with worse long-term outcomes and a higher risk of recurrence after surgical resection
compared with subAS caused by a discrete membrane (S4.2.3-5). Surgical intervention on patients with
asymptomatic subAS (maximum gradient ≥50 mm Hg) with preserved LV ejection fraction may delay
progression of, or improve the degree of, aortic valve regurgitation. SubAS in adults may progress more
slowly than in children, and although mild AR is common, it may not be progressive in medium-term
follow-up (S4.2.3-10).
Table 18. Subaortic Stenosis: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 24 24 12 12
TTE 24 24 12 12
ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; SubAS, subaortic
stenosis; and TTE, transthoracic echocardiogram.
4.2.4. Congenital Valvular Aortic Stenosis
Recommendations for Congenital Valvular Aortic Stenosis
Diagnostic
I B-NR
1. Adults with bicuspid aortic valve should be evaluated for coarctation of
the aorta by clinical examination and imaging studies (S4.2.4-1).
IIa B-NR 2. It is reasonable to screen first-degree relatives of patients with bicuspid

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aortic valve or unicuspid aortic valve with echocardiography for valve
disease and aortopathy (S4.2.4-2–S4.2.4-4).
Therapeutic
IIb B-NR
3. In adults with bicuspid aortic valve stenosis and a noncalcified valve with
no more than mild AR meeting indications for intervention per GDMT
(S4.2.4-5), it may be reasonable to treat with balloon valvuloplasty
(S4.2.4-6).
Synopsis
Indications for aortic valve replacement according to the 2014 VHD guideline (S4.2.4-5) generally apply.
Recommendations above deal with issues specific to congenital aortic valve disease, which includes
BAV, as well as unicuspid aortic valve and aortic stenosis caused by hypoplastic aortic annulus. The
underlying anatomy must be taken into account in patients with congenital aortic stenosis, as
intervention may need to include annular enlarging procedures and other surgical techniques not
commonly used in valvular aortic stenosis. These patients are often young adults, for whom lifestyle
considerations such as athletic endeavors, employment, and childbearing may influence the type of
intervention.
See Section 3.4 for recommendations on diagnostic evaluation; and Table 19 for routine testing and
follow-up intervals.
1. CoA has a male-to-female ratio of 1.5:1 (S4.2.4-7–S4.2.4-13). A BAV is present in 50% to 70% of cases
of CoA. Given the association of these abnormalities, evaluation of patients with BAV for CoA is
warranted.
2. BAV is the most prevalent congenital cardiac abnormality with an estimated prevalence of 4.6 per
1,000 live births, and is 1.5 times more prevalent in males than females (S4.2.4-7–S4.2.4-13). Most cases
are spontaneous; however, familial inheritance may occur in an autosomal dominant pattern with
variable penetrance. On echocardiographic screening, 1 study reports the prevalence of asymptomatic
BAV in first-degree relatives of patients is 9%, and 32% of first-degree relatives without a BAV will have
an abnormal aorta (S4.2.4-4).
3. Calcification of the aortic valve in adults necessitates that most patients who require therapy for
aortic stenosis will require aortic valve replacement per GDMT (S4.2.4-5). However, young patients with
congenitally abnormal valves and relatively little calcification may be candidates for balloon
valvuloplasty. Balloon valvuloplasty may improve the degree of stenosis and symptoms in patients with
mobile noncalcified BAV stenosis. In general, the valves that would be amenable to successful balloon
valvuloplasty are found in young patients, who are often <25 years of age. Restenosis will occur over
time and in a relatively short time in some patients. Balloon valvuloplasty of calcified BAV is associated
with decreased efficacy and an increased risk of AR (S4.2.4-14, S4.2.4-15). Although transcatheter
interventions for aortic stenosis are increasingly commonly performed in older adults and, thus, there
are increasing numbers of interventional cardiologists technically skilled at balloon aortic valvuloplasty
and transcatheter aortic valve replacement, the differences in anatomy and patient population
necessitate collaboration with an ACHD cardiologist for younger patients.

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Table 19. Congenital Aortic Stenosis: Routine Follow-Up and Testing Intervals*
Stage Frequency of Echocardiogram
Progressive (Stage B) Every 3–5 y (mild severity, Vmax 2.0–2.9 m/s)
Every 1–2 y (moderate severity, Vmax 3.0–3.9 m/s)
Severe (Stage C) Every 6–12 mo (Vmax ≥4.0 m/s)
Aortic dilation >4.5 cm Every 12 mo (echocardiogram, MRI or CT)
*Modified from existing GDMT for valvular heart disease (S4.2.4-5).
CT indicates computed tomography; GDMT, guideline-directed management and therapy; MRI,
magnetic resonance imaging; and Vmax, maximum velocity.
4.2.4.1. Turner Syndrome
Recommendations for Turner Syndrome
Diagnostic
I B-NR
1. Women with Turner syndrome should be evaluated for bicuspid aortic
valve, coarctation of the aorta, and enlargement of the ascending aorta
(S4.2.4.1-1).
Therapeutic
IIa B-NR
2. Prophylactic replacement of the aortic root or ascending aorta in adults
with Turner syndrome is reasonable when the aortic diameter is 2.5
cm/m2
or greater (S4.2.4.1-2).
Synopsis
The management of valve dysfunction is generally as directed by the 2014 VHD guideline (S4.2.4.1-3).
Aortopathy is a commonly associated condition, and frequently involves the mid-ascending aorta, which
may not be reliably seen on TTE. Measurement of aortic dimensions with magnetic resonance
angiography and CCT has not been standardized, and clinicians should be wary of comparisons of
reported diameters between modalities. Side-by-side comparisons are more reliable for detecting
changes over time. Baseline and routine serial measurements of the aortic size are useful, with imaging
interval determined by the indexed size and rate of progression. Pregnancy in Turner syndrome, which
often requires assisted reproductive technology, is associated with an increased risk of aortic dissection,
especially if there is a preexisting abnormality of the aortic valve or aorta (S4.2.4.1-4).
1. Women with Turner syndrome are at substantial risk of BAV, CoA, and aortic enlargement, which can
result in morbidity and mortality if left untreated. Therefore, evaluation is necessary to help decide what
interventions may be necessary and provide accurate risk assessment for exercise, pregnancy, or other
considerations that could be influenced by aortic pathology.
2. Because of case series reporting dissection at smaller aortic diameters than in non-Turner aortopathy,
prophylactic surgery is reasonable at lower diameters, particularly if rapid dilation is present.
Measurements must take into account the patient's stature either by indexing to body surface area
utilizing Turner-specific normative data or by using ratio of aortic area to body height (S4.2.4.1-5–
S4.2.4.1-7).

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4.2.4.2. Aortopathies
Several CHD subtypes and/or repairs are associated with enlargement of the aorta. The management of
these varies by condition, as some are perceived to have a stronger association with aortic dissection or
rupture than others, although the true natural history of most is unknown. There is wide heterogeneity
of timing of surgical referral, which makes interpretation of longitudinal studies problematic.
BAV is the most common CHD, is associated with aortopathy, and is of high concern for aortic
complications, as discussed in other guideline statements (S4.2.4.2-1–S4.2.4.2-3). Although in many
published series of aortic dissection, BAV patients account for a higher proportion of dissections than
expected from prevalence of BAV in the general population alone, the risk of dissection or rupture
amongst all BAV patients is less clear. The largest population study reported a 0.5% risk of aortic rupture
or dissection after a mean of 16 years of follow-up (S4.2.4.2-4), although 11% underwent elective aortic
surgery. Risk factors for aortic complications were age and an enlarged aorta at baseline. Frequency of
dissection in BAV disease is higher in adults with Turner syndrome.
A dilated neoaortic root after a Ross procedure is not uncommon, although only a single
dissection has been reported (S4.2.4.2-5). Because of this, it is generally believed that prophylactic root
replacement strategies based on sinus of Valsalva diameters can be less aggressive after a Ross
procedure than in a native BAV patient, but practice patterns vary. Most patients with a Ross repair had
underlying congenitally abnormal aortic valves (BAV or unicuspid aortic valve) and, therefore, are at risk
of the ascending aortic dilation typical of those abnormalities. Thus, in addition to the dilation at the
sinuses of Valsalva associated with the Ross repair, dilation of the native ascending aorta above the
sinotubular junction can also occur.
Although patients with conotruncal abnormalities (TOF, dextrotransposition of the great arteries
[d-TGA] after arterial switch (S4.2.4.2-6–S4.2.4.2-8), pulmonary atresia with VSD, truncus arteriosus)
commonly have aortic diameters of 40 mm to 50 mm, aortic complications are extremely rare (only 6
published case reports) (S4.2.4.2-9–S4.2.4.2-14). Therefore, there is no strong justification for empiric
prophylactic surgery in such patients based solely on aortic diameter. Watchful observation has often
been recommended unless surgery is being undertaken for other indications (S4.2.4.2-15). However,
there are rare patients who develop substantially greater aortic enlargement and for whom prophylactic
surgery may have more of a role. Risk factor management such as control of hypertension is important.
There are no RCTs evaluating the efficacy of medical therapy to reduce the rate of progression of aortic
dilation or incidence of aortic dissection in this population.
4.2.5. Supravalvular Aortic Stenosis
Recommendations for Supravalvular Aortic Stenosis
Diagnostic
I C-LD
1. Aortic imaging using TTE, TEE, CMR, or CTA is recommended in adults
with Williams syndrome or patients suspected of having supravalvular
aortic stenosis (S4.2.5-1).
I C-LD
2. Coronary imaging is recommended in patients with Williams syndrome
and supravalvular aortic stenosis presenting with symptoms of coronary
ischemia (S4.2.5-2–S4.2.5-4).

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Therapeutic
I B-NR
3. Surgical repair is recommended for adults with supravalvular aortic
stenosis (discrete or diffuse) and symptoms or decreased LV systolic
function deemed secondary to aortic obstruction (S4.2.5-5–S4.2.5-8).
I C-LD
4. Coronary artery revascularization is recommended in symptomatic adults
with supravalvular aortic stenosis and coronary ostial stenosis (S4.2.5-4,
S4.2.5-9).
Synopsis
Supravalvular aortic stenosis is a relatively rare condition overall but is seen commonly in patients with
Williams syndrome or homozygous familial hypercholesterolemia. The stenotic ridge tends to occur
distal to the coronary artery orifices at the sinotubular junction. In addition to pressure load physiology
similar to other causes of LVOT obstruction, coronary abnormalities can occur, including significant
coronary ostial stenosis resulting in risk of SCD and anesthesia risk (S4.2.5-10–S4.2.5-14). Unlike subAS
or valvular aortic stenosis, the coronary arteries are exposed to the higher pressure generated by the
supravalvular obstruction.
1. TTE with Doppler imaging is useful in deriving peak and mean pressure gradients across the area of
supravalvular aortic stenosis from apical, suprasternal, and right parasternal views; however,
visualization of the full extent of supravalvular aortic stenosis with TTE is limited. TEE is superior in this
regard, and 3D TEE allows excellent visualization of the narrowed ascending aorta. CMR and CTA provide
comprehensive and detailed images of supravalvular aortic stenosis and are used with echocardiography
in the assessment of patients before and after repair (S4.2.5-15).
2. Impaired coronary perfusion may occur because of varying degrees of aortic valve leaflet adhesion to
the narrowed sinotubular junction or because of fibrotic thickening in the area immediately surrounding
the coronary ostia. This causes ostial stenosis with restriction in diastolic filling of the coronary arteries;
the left coronary is most frequently involved. TEE with Doppler can be used in the assessment of
proximal coronary patency and to search for flow turbulence. CMR can also be used in assessing the
coronary ostia. Electrocardiographic-gated CT coronary angiography or invasive selective coronary
angiography provides excellent visualization of the coronary arterial anatomy.
3. Supravalvular aortic stenosis is usually a progressive problem with a progressive increase in LV systolic
pressure resulting in exertional symptoms and, if the stenosis is severe, eventual decreases in LV systolic
function.
4. Impaired coronary perfusion may occur because of varying degrees of aortic valve leaflet adhesion to
the narrowed sinotubular junction with restriction in diastolic filling of the coronary arteries; the left
coronary is most frequently involved. Surgical coronary revascularization is recommended for patients
with symptoms of coronary ischemia.

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Table 20. Supravalvular Aortic Stenosis: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 24 24 12 12
TTE† 24 24 12 12
CMR‡/CCT§ 36–60 36–60 36–60 36–60
Exercise test║ As needed 24 24 12
†RouƟne TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise.
‡CMR may be indicated for assessment of aorƟc anatomy. Baseline study is recommended with periodic follow-up CMR, with
frequency of repeat imaging determined by anatomic and physiological findings.
§If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.
║6-minute walk test or CPET, depending on the clinical indication.
ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance;
CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.
4.2.6. Coarctation of the Aorta
Recommendations for Coarctation of the Aorta
Diagnostic
I B-NR
1. Initial and follow-up aortic imaging using CMR or CTA is recommended in
adults with coarctation of the aorta, including those who have had
surgical or catheter intervention (S4.2.6-1–S4.2.6-3).
I C-EO
2. Resting blood pressure should be measured in upper and lower
extremities in all adults with coarctation of the aorta.
IIa C-LD
3. Ambulatory blood pressure monitoring in adults with coarctation of the
aorta can be useful for diagnosis and management of hypertension
(S4.2.6-4).
IIb B-NR
4. Screening for intracranial aneurysms by magnetic resonance angiography
or CTA may be reasonable in adults with coarctation of the aorta (S4.2.6-
5, S4.2.6-6).
IIb C-LD
5. Exercise testing to evaluate for exercise-induced hypertension may be
reasonable in adults with coarctation of the aorta who exercise (S4.2.6-4,
S4.2.6-7).
Therapeutic
I B-NR
6. Surgical repair or catheter-based stenting is recommended for adults
with hypertension and significant native or recurrent coarctation of the
aorta (S4.2.6-1, S4.2.6-2, S4.2.6-8–S4.2.6-12).
I C-EO
7. GDMT is recommended for treatment of hypertension in patients with
coarctation of the aorta (S4.2.6-13).
IIb B-NR
8. Balloon angioplasty for adults with native and recurrent coarctation of
the aorta may be considered if stent placement is not feasible and
surgical intervention is not an option (S4.2.6-14).

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Synopsis
CoA typically occurs near the ductal remnant and left subclavian artery. Hypertension is the most
common sequela of CoA, whether repaired or unrepaired. BAV is commonly associated with CoA and is
present in more than half of CoA patients (S4.2.6-15–S4.2.6-21). Intracranial aneurysms may occur.
Ascending aortic aneurysms are often found in those with BAV, and aneurysms are seen at the site of
coarctation repair in the descending thoracic aorta or arch. Dissection can occur, presumably more likely
in the setting of poorly controlled hypertension. Even with excellent repair, hypertension remains
common and predisposes to later myocardial infarction, stroke, and HF.
1. Complications of CoA repair include recoarctation, aneurysm, pseudoaneurysm, and dissection. Long-
term follow-up after successful surgical intervention for CoA reveals that 11% of patients may require
reintervention for restenosis, visualized by CMR or CTA (if CMR is contraindicated or there is a history of
stent therapy) and supported by physical examination findings (S4.2.6-1). Although evidence of
recoarctation can be found on clinical examination and echocardiography, aneurysms near the site of
repair may not be well seen by echocardiography. Patients who have undergone surgical patch repair
are at an increased risk of developing aneurysms that can be evaluated by CMR or CTA. After successful
transcatheter intervention with stenting or balloon angioplasty, follow-up CMR or CTA imaging is
recommended to evaluate for long-term complications (e.g., aneurysm formation, stent fracture, or
stent migration) (S4.2.6-1). The same CMR or CTA study will also evaluate the ascending aorta, which
may become aneurysmal over the years of follow-up.
2. Unoperated adults with CoA almost invariably present with systemic arterial hypertension measured
in the upper extremities. Brachial and femoral pulse timing and amplitude evaluation on physical
examination reveals a delay or decrease in amplitude of the femoral pulse. Upper and lower extremity
noninvasive blood pressure measurement is recommended in all patients with unoperated or
operated/intervened CoA.
3. Upper body systemic hypertension is prevalent in patients with unoperated coarctation and may be
present in up to one third of patients who have undergone operative or transcatheter intervention
(S4.2.6-2). Systemic hypertension may not consistently be identifiable at rest; therefore, ambulatory
blood pressure monitoring can be useful in identifying and appropriately managing patients with
ambulatory hypertension.
4. Multiple studies have demonstrated an increased frequency of intracranial aneurysm in adults with
CoA. Approximately 10% of patients with CoA have intracranial aneurysms identified on magnetic
resonance angiography or CTA. Increasing age has been identified as a risk factor. Many such identified
aneurysms are small; however, the expected outcome and ideal management of such aneurysms are
not clear. Providers and patients should be aware of management uncertainties when considering
routine screening for aneurysms (S4.2.6-22). Additionally, there are some data suggesting that
intracranial aneurysms are not commonly found in children and teenagers with CoA (S4.2.6-23),
reinforcing the possibility that coarctation alone may not be sufficient for development of intracranial
aneurysm, and other factors, such as hypertension and/or age, play a role in development and
progression of aneurysms.

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5. Despite successful surgical repair or transcatheter intervention, hypertension can persist and may not
be identified during resting blood pressure measurement. Up to 80% of patients with prior CoA
intervention manifest an abnormally elevated upper extremity exercise blood pressure response, and
peak blood pressure is correlated with increased LV mass (S4.2.6-24). Moreover, restenosis of the
previously repaired or stented region may be identified by increased peak blood pressure response,
increased upper to lower extremity blood pressure gradient with exercise, and increased Doppler
velocity across the coarctation site during exercise TTE.
6. Significant native or recurrent aortic coarctation has been defined as follows: upper extremity/lower
extremity resting peak-to-peak gradient >20 mm Hg or mean Doppler systolic gradient >20 mm Hg;
upper extremity/lower extremity gradient >10 mm Hg or mean Doppler gradient >10 mm Hg plus either
decreased LV systolic function or AR; upper extremity/lower extremity gradient >10 mm Hg or mean
Doppler gradient >10 mm Hg with collateral flow (S4.2.6-2, S4.2.6-8, S4.2.6-12). This should be coupled
with anatomic evidence for CoA, typically defined by advanced imaging (CMR, CTA). The best evidence
to proceed with intervention for CoA includes systemic hypertension, upper extremity/lower extremity
blood pressure gradient and echocardiography Doppler gradient as defined above, and anatomic
evidence of CoA. Multiple factors help determine whether surgery or stenting is optimal, including
anatomic features such as proximity of native coarctation to head and neck vessels or concomitant
aneurysm, and, if stenting, whether a covered stent is needed.
7. The long-term complications of CoA are generally related to chronic upper body systemic
hypertension, therefore, systemic hypertension should be identified by resting, ambulatory, or exercise
blood pressure assessment and medical treatment should follow GDMT (S4.2.6-13, S4.2.6-25)
8. Balloon angioplasty alone is associated with a higher rate of intimal tears and aneurysm formation
compared with stent placement.
Table 21. CoA: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A*(mo)
Physiological
Stage B*(mo)
Physiological
Stage C*(mo)
Physiological
Stage D*(mo)
ECG 24 24 12 12
TTE† 24 24 12 12
CMR‡/CCT§ 36–60 36–60 12–24 12–24
Exercise test║ 36 24 24 12
‡CMR may be indicated for assessment of aorƟc size and aorƟc arch/coarctaƟon repair site anatomy. Baseline study is
recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological
findings.
§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status–post-stent therapy for coarctation of
the aorta; the frequency should be weighed against radiation exposure.
║6-minute walk test or CPET, depending on the clinical indication.
ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance
imaging; CoA, coarctation of the aorta; CPET, cardiopulmonary exercise; ECG, electrocardiogram; and TTE, transthoracic
echocardiogram.
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4.3.1. Valvular Pulmonary Stenosis
Recommendations for Valvular Pulmonary Stenosis
I B-NR
1. In adults with moderate or severe valvular pulmonary stenosis and
otherwise unexplained symptoms of HF, cyanosis from interatrial right-
to-left communication, and/or exercise intolerance, balloon
valvuloplasty is recommended (S4.3.1-1–S4.3.1-4).
I B-NR
2. In adults with moderate or severe valvular pulmonary stenosis and
otherwise unexplained symptoms of HF, cyanosis, and/or exercise
intolerance who are ineligible for or who failed balloon valvuloplasty,
surgical repair is recommended (S4.3.1-1, S4.3.1-5–S4.3.1-8)
IIa C-EO
3. In asymptomatic adults with severe valvular pulmonary stenosis,
intervention is reasonable.
Synopsis
Valvular PS is one of the most common congenital heart defects, estimated to occur in up to 7% of
children born with CHD (S4.3.1-9–S4.3.1-11). Some common findings associated with isolated valvular PS
include a dilated main PA and dysplastic valve cusps. Surgical or catheter-based intervention depends on
degree of obstruction, RV pressure and function, and associated symptoms. Patients with isolated
pulmonary valve stenosis (native or recurrent after an intervention) require ongoing cardiac follow-up
and monitoring for evidence of progressive valve stenosis or regurgitation, RV hypertrophy, HF, and
arrhythmias (S4.3.1-12). Patients with mild native pulmonary valve stenosis (Table 22) have a reassuring
natural history, and intervention is not usually necessary. Patients with severe PS (Table 22) usually
require intervention in childhood with a good prognosis into adulthood (S4.3.1-6). Patients with
moderate stenosis (Table 22) have more variable histories, with some having received surgical or
catheter intervention in childhood or adulthood and some not. Patients with moderate PS, whether
native or postintervention, have a good long-term outcome, although some will go on to require an
intervention in adulthood because of progressive PS or, commonly, significant PR as a sequela of earlier
intervention.
Pulmonary atresia with intact ventricular septum is a rare congenital heart lesion that is
associated with varying degrees of RV hypoplasia and tricuspid valve hypoplasia in addition to
pulmonary valve atresia. Adults with pulmonary atresia with intact ventricular septum followed various
surgical pathways in childhood, either biventricular repair, 1 1/2 ventricular repair, Fontan procedure,
transplant, or shunt palliation (S4.3.1-13). Adults with history of pulmonary atresia with intact
ventricular septum have a high incidence of need for reintervention and management of atrial
arrhythmias (S4.3.1-14, S4.3.1-15). Restrictive RV physiology is common in adults with history of
pulmonary atresia with intact ventricular septum and may be associated with substantial ventricular
fibrosis (S4.3.1-16) and RV-dependent coronary circulation.
other procedures in these patients; Section 3.4 for recommendations on diagnostic evaluation; Table 23
for routine testing and follow-up intervals; and Figure 3 for a diagnostic and treatment algorithm for
isolated PR after repair of PS.

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1. In patients with moderate or severe isolated pulmonary valve stenosis, pulmonary balloon
valvuloplasty is safe and effective in reducing the pulmonary valve gradient and improving symptoms in
most patients
2. Surgical valvotomy is usually sufficient, particularly when the pulmonary annulus is not hypoplastic.
Pulmonary valve replacement may be necessary when there is marked dysplasia of the pulmonary valve
or significant hypoplasia of the annulus.
3. Relief of a severely stenotic pulmonary valve in an asymptomatic patient will reduce the RV pressure
and the possibility of potential sequelae. As in symptomatic patients, the procedure can be performed
by surgery or interventional catheterization with low morbidity and mortality. If intervention is deferred,
careful follow-up to evaluate for symptoms, decline in exercise capacity, worsening RV function, or
development of cyanosis is important and may prompt reconsideration of intervention.
Table 22. Severity of RVOT Obstruction
Mild Peak gradient <36 mm Hg (peak velocity
<3 m/s)
Moderate Peak gradient 36–64 mm Hg (peak velocity
3–4 m/s)
Severe Peak gradient 64 mm Hg (peak velocity >4
m/s); mean gradient >35 mm Hg
Estimations of RV systolic pressure by TR velocity is part of the
echocardiographic assessment of RV obstruction, as Doppler
measurements across the RV obstruction itself may be unreliable.
RV indicates right ventricular; RVOT, right ventricular outflow tract;
and TR, tricuspid regurgitation.
Table 23. Valvular PS: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 36–60 24 12 12
TTE 36–60 24 12 12
†6-minute walk test or CPET, depending on clinical indication.
ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; PS, pulmonary
stenosis; and TTE, transthoracic echocardiogram.
4.3.1.1. Isolated PR After Repair of PS
Recommendations for Isolated PR After Repair of Pulmonary Stenosis
I C-EO
1. In symptomatic patients with moderate or greater PR resulting from
treated isolated pulmonary stenosis, with RV dilation or RV dysfunction,
pulmonary valve replacement is recommended.
I C-EO
2. For asymptomatic patients with residual PR resulting from treatment of
isolated pulmonary stenosis with a dilated right ventricle, serial follow-
up is recommended.
IIb C-EO 3. In asymptomatic patients with moderate or greater PR resulting from

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treatment of isolated pulmonary stenosis with progressive RV dilation
and/or RV dysfunction, pulmonary valve replacement may be
reasonable.
Synopsis
Although many patients with valvular PS do not require intervention, some have PS that is severe
enough to warrant intervention, often in infancy or childhood. PS can be alleviated either by surgical
valvotomy or with balloon valvuloplasty. Either surgical or catheter intervention may result in
hemodynamically important PR that can result in symptoms, RV enlargement, and/or dysfunction
requiring pulmonary valve replacement.
1. Patients with isolated PS who have previously undergone an intervention on the pulmonary valve
require ongoing clinical follow-up and monitoring of PR, RV size and function, and functional capacity.
This may include echocardiography, CPET, and advanced imaging. The right ventricle in patients with PR
after intervention for PS may be smaller than in patients with TOF; however, patients with PR may have
evidence of decreased RV ejection fraction or decreased exercise capacity. Pulmonary valve
replacement can improve symptoms for patients with symptoms that are attributable to moderate or
greater PR, and can improve RV size and/or RV function if there is RV dilation or decreased RV ejection
fraction.
2. PR resulting from treatment of isolated PS may have progressive impact on RV size and function, and
may result in symptoms, such that pulmonary valve replacement would be considered. Serial follow-up
for clinical evaluation, CPET, and imaging to evaluate for symptoms, exercise intolerance attributable to
PR, and/or RV dilation or RV dysfunction will allow appropriate timing of intervention if needed.
3. There are no data to suggest appropriate timing for pulmonary valve replacement in the presence of
RV dilation, but it is likely inappropriate to directly extrapolate the data applicable to patients with TOF
(S4.3.1.1-1). However, RV dilation or dysfunction should improve, or at least not progress further, if the
volume overload from PR is alleviated by pulmonary valve replacement. Thus, although specific RV size
criteria are not available for these patients to determine timing of pulmonary valve replacement,
patients with progressively worsening RV size or function presumably represent a subset of patients for
whom valve replacement could be beneficial.

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Figure 3. Isolated PR After Repair of PS
*Significant PR causes RV dilation. If a patient has moderate or greater PR and normal RV size, most likely the estimation of PR
severity is inaccurate or there may be restrictive RV physiology, which would warrant further investigation.
†Symptoms may include dyspnea, chest pain, and/or exercise intolerance referable to PR or otherwise unexplained.
ACHD indicates adult congenital heart disease; CPET, cardiopulmonary exercise test; PR, pulmonary regurgitation; PS,
pulmonary stenosis; and RV, right ventricular.

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4.3.2. Branch and Peripheral Pulmonary Stenosis
Recommendations for Branch and Peripheral PS
Diagnostic
I B-NR
1. For adults with peripheral or branch PS, ongoing surveillance is
recommended (S4.3.2-1, S4.3.2-2).
Therapeutic
IIa B-NR
2. In adults with peripheral or branch PA stenosis, PA dilation and stenting
can be useful (S4.3.2-2, S4.3.2-3).
Synopsis
Pulmonary branch and peripheral PS can be isolated, occur as part of a constellation of right ventricular
outflow tract (RVOT) obstruction, or be found in association with a syndrome (e.g., Noonan, Alagille,
Williams, maternal rubella exposure). Intervention decisions are typically based on symptoms,
distribution of pulmonary blood flow, RV function, and RV systolic pressure. TTE is a good modality to
obtain RV pressure and function but does not adequately image the peripheral pulmonary arteries.
Alternative imaging (e.g., CMR, CCT) can visualize anatomic obstructions and branch PA anatomy. In
addition, CMR and pulmonary perfusion testing can quantify relative pulmonary blood flow.
1. Cardiac follow-up and imaging may include evaluation of RV pressure; quantifying relative pulmonary
blood flow and imaging for evidence of residual lesions or PA obstruction or aneurysm at sites of prior
intervention; and in-stent stenosis and/or stent fracture (the latter often best seen by fluoroscopy).
Stenting of branch PA stenosis is effective in reducing the pressure gradients, but patients often require
further intervention (S4.3.2-2). In-stent stenosis with a reduction in the ipsilateral pulmonary blood flow
is seen in approximately 25% of patients after percutaneous PA angioplasty and stent placement, more
common in patients with abnormal pulmonary arteries, such as those with TOF or Williams syndrome
(S4.3.2-1). Regular surveillance and imaging, with intervention as required, may prevent the
development of RV hypertension and its sequelae (S4.3.2-1).
2. Balloon angioplasty or stenting of a peripheral PA is effective in reducing pressure gradients and
improving pulmonary blood flow. Indications for pulmonary angioplasty or stenting include symptoms
attributed to the decreased pulmonary blood flow, focal narrowing, abnormal differential perfusion,
and/or elevated RV pressure. The decision for intervention with PA angioplasty or stenting includes
assessment of clinical symptoms, imaging, and discussion with an ACHD interventional cardiologist.
Table 24. Branch and Peripheral PS: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 24–36 24 12 12
TTE† 24–36 24 12 12

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CMR‡/CCT§ 36–60 36–60 24–36 24–36
‡CMR may be indicated for assessment of branch PS. Baseline study is recommended with periodic follow-up CMR, with
frequency of repeat imaging determined by anatomic and physiological findings.
§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status and post-stent therapy for peripheral PS;
the frequency should be weighed against radiation exposure.
║6-minute walk test or CPET, depending on clinical indication.
imaging; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; PS, pulmonary stenosis; and TTE, transthoracic
echocardiogram.
4.3.3. Double-Chambered Right Ventricle
Recommendations for Double-Chambered Right Ventricle
I C-LD
1. Surgical repair for adults with double-chambered right ventricle and
moderate or greater outflow obstruction is recommended in patients
with otherwise unexplained symptoms of HF, cyanosis, or exercise
limitation (S4.3.3-1–S4.3.3-3) (Table 22).
IIb C-LD
2. Surgical repair for adults with double-chambered right ventricle with a
severe gradient may be considered in asymptomatic patients (S4.3.3-3,
S4.3.3-4) (Table 22).
Synopsis
Double-chambered right ventricle is uncommon in adults. Hypertrophied muscle bundles develop in the
RV cavity, creating RVOT obstruction (S4.3.3-5, S4.3.3-6). It is commonly associated with a VSD. Double-
chambered right ventricle can be missed on TTE if not sought specifically, and alternative imaging or
cardiac catheterization is often required to confirm the diagnosis and establish the hemodynamic
impact.
1. Surgery typically involves transatrial or transventricular resection of obstructing muscle bundles and
VSD closure if present. Occasionally, patch enlargement of RVOT may be necessary to adequately relieve
obstruction.
2. VSD is often present and may communicate with the higher or lower pressure chamber in the right
ventricle, with resulting differences in shunt direction and flow characteristics. In patients with a severe
gradient through the right ventricle, the VSD may be associated with right-to-left shunting if proximal to
the obstruction, or associated with left-to-right shunting if distal. Exercise testing performed in a
subjectively asymptomatic patient will often be abnormal. Patients may benefit from repair of both the
VSD and outflow obstruction, especially if exercise capacity is decreased.

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Table 25. Double-Chambered Right Ventricle: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 24–36 24 12 12
TTE 24–36 24 12 12
†6-minute walk test or CPET, depending on clinical indication.
4.3.4. Ebstein Anomaly
Recommendations for Ebstein Anomaly
Diagnostic
IIa B-NR
1. In adults with Ebstein anomaly, CMR can be useful to determine
anatomy, RV dimensions, and systolic function (S4.3.4-1, S4.3.4-2).
IIa B-NR
2. In adults with Ebstein anomaly, TEE can be useful for surgical planning if
TTE images are inadequate to evaluate tricuspid valve morphology and
function (S4.3.4-1).
IIa B-NR
3. Electrophysiological study with or without catheter ablation can be
useful in the diagnostic evaluation of adults with Ebstein anomaly and
ventricular preexcitation but without supraventricular tachycardia
(S4.3.4-3, S4.3.4-4).
IIa B-NR
4. In adults with Ebstein anomaly, electrophysiological study (and catheter
ablation, if needed) is reasonable before surgical intervention on the
tricuspid valve even in the absence of preexcitation or supraventricular
tachycardia (S4.3.4-5).
Therapeutic
I B-NR
5. Surgical repair or reoperation for adults with Ebstein anomaly and
significant TR is recommended when one or more of the following are
present: HF symptoms, objective evidence of worsening exercise
capacity, progressive RV systolic dysfunction by echocardiography or
CMR (S4.3.4-6–S4.3.4-10).
I C-LD
6. Catheter ablation is recommended for adults with Ebstein anomaly and
high-risk pathway conduction or multiple accessory pathways (S4.3.4-3,
S4.3.4-11, S4.3.4-12).
IIa B-NR
7. Surgical repair or reoperation for adults with Ebstein anomaly and
significant TR can be beneficial in the presence of progressive RV
enlargement, systemic desaturation from right-to-left atrial shunt,
paradoxical embolism, and/or atrial tachyarrhythmias (S4.3.4-11, S4.3.4-
13, S4.3.4-14).
IIb B-NR
8. Bidirectional superior cavopulmonary (Glenn) anastomosis at time of
Ebstein anomaly repair may be considered for adults when severe RV
dilation or severe RV systolic dysfunction is present, LV function is

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preserved, and left atrial pressure and LV end diastolic pressure are not
elevated (S4.3.4-6, S4.3.4-15).
Synopsis
Ebstein anomaly is an uncommon congenital heart defect occurring in about 0.005% of live births
(S4.3.4-16–S4.3.4-18). It is a malformation of the tricuspid valve and the right ventricle and varies in
severity, including babies who do not survive infancy, asymptomatic adults diagnosed incidentally in the
sixth and seventh decades of life, and many variations in severity between those extremes. Ebstein
anomaly can occur with other defects including ASD, VSD, and PS. A patent foramen ovale, otherwise
usually considered normal, may have significant impact in Ebstein anomaly. Accessory pathways and
arrhythmias are relatively common. Patient surveillance and management varies depending on age,
severity of the lesion, and associated abnormalities including HF, cyanosis, and arrhythmias. Surveillance
includes echocardiographic and other advanced imaging to assess RV size and function, rhythm
assessment, pulse oximetry, and stress testing. Treatments include medical and surgical therapy for
patients with manifest symptoms as well as catheter-based structural and electrophysiological
interventions when indicated.
1. Deciphering the anatomy and size of the right atrium and right ventricle in Ebstein anomaly is often
difficult using echocardiography alone, particularly in adults. Data obtained from CMR can inform clinical
care and surgical planning or decision-making, because CMR data correlate well with intraoperative
findings.
2. Two-dimensional and 3D TEE can better define the anatomy and function of the tricuspid valve before
surgery and provide valuable information in planning surgical repair.
3. Approximately one third of adults with Ebstein anomaly and ventricular preexcitation have multiple
accessory pathways, associated with a high risk of SCD. Adults with Ebstein anomaly also have a high
prevalence of atrial tachyarrhythmia (S4.3.4-3, S4.3.4-4). In the setting of ventricular preexcitation, atrial
tachyarrhythmia may expose the patient to a higher risk of lethal ventricular arrhythmia. In patients
with clinical supraventricular tachycardia, management is according to existing GDMT (S4.3.4-19). A
Pediatric & Congenital Electrophysiology Society (PACES)/HRS expert consensus document provides
additional information on the management of arrhythmias (S4.3.4-20).
4. Concealed accessory pathways are common in Ebstein anomaly and may coexist with manifest
accessory pathways. In addition, preexcitation may be present but difficult to appreciate on the surface
ECG. Tricuspid valve surgery can hinder transcatheter access to right-sided accessory pathways and the
slow pathway in AV node reentry, such that it may be reasonable to assess for arrhythmia substrates
and proceed with catheter ablation if identified, before surgery.
5. Data demonstrate that delay of surgery until HF or RV systolic dysfunction ensues is associated with
poorer outcomes; surgery before either of those develops is recommended (S4.3.4-6, S4.3.4-7, S4.3.4-
10). Ebstein anomaly is understood as not just valve disease but also a myopathic process.
Consequently, threshold for operation may be different than in other RV volume-loading lesions,
because there is more concern regarding the capacity of the myopathic Ebstein right ventricle to
tolerate a volume load. Also, there are cohort series of Ebstein patients to inform decisions (S4.3.4-6,
S4.3.4-7, S4.3.4-10). Surgical repair generally consists of tricuspid valve repair (preferred when feasible)

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or replacement, selective plication of atrialized right ventricle, reduction atrioplasty, arrhythmia surgery,
and/or closure of atrial level shunt. Surgery may result in improvement of symptoms and functional
ability, and prevent or delay worsening symptoms.
6. Adults with Ebstein anomaly and ventricular preexcitation often have multiple accessory pathways,
which are associated with a higher risk of SCD. Surgical interruption of accessory pathways is largely
reserved for patients who have failed attempts at catheter ablation. High-risk pathways are those with
an increased risk of SCD, largely related to VF resulting from rapidly conducting AF. Definition and
discussion of high-risk pathways is beyond the scope of these guidelines but can be found elsewhere,
such as the “PACES/HRS Expert Consensus Statement on the Recognition and Management of
Arrhythmias in ACHD” (S4.3.4-21).
7. Systemic desaturation and arrhythmias are frequently signs of worsening hemodynamics, progressive
TR, or worsening RV function. Surgery for the tricuspid valve as well as closure of the ASD or stretched
patent foramen ovale and arrhythmia surgery can be beneficial. When arrhythmia surgery is required, it
typically involves a modified right atrial maze procedure. In the presence of AF, the addition of a left
atrial Cox Maze III procedure can be beneficial to reduce the risk of recurrent AF.
8. The use of the bidirectional cavopulmonary shunt is much more common in children than in adults.
When it is applied in the adult, it is usually reserved for patients with severe RV dysfunction with
concern that the right ventricle will not tolerate supporting the entirety of stroke volume (S4.3.4-6,
S4.3.4-15). Preoperative catheterization to determine hemodynamics and feasibility of applying the
bidirectional cavopulmonary shunt becomes progressively more important in older patients, particularly
those with longstanding hypertension with LV hypertrophy, which can lead to diastolic dysfunction and
elevated pulmonary pressures.
Table 26. Ebstein Anomaly: Routine and Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 12–24 12 12 12
CXR As needed As needed 12–24 12–24
TTE† 12–24 12–24 12 12
Pulse oximetry 24 12 Each visit Each visit
Holter monitor As needed As needed 24 12–24
CMR‡/CCT§ 60 36 24–36 12–24
Exercise test║ 36 24–36 24 12
‡CMR may be indicated for assessment of right ventricular size and funcƟon. Baseline study is recommended with periodic
follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.
§CCT may be used if CMR is not feasible; the frequency should be weighed against radiation exposure.
imaging; CPET, cardiopulmonary exercise test; CXR, chest x-ray; ECG, electrocardiogram; and TTE, transthoracic
echocardiogram.

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4.3.5. Tetralogy of Fallot
Recommendations for TOF
(See Section 4.3.6. for recommendations regarding evaluation and management of right ventricle–to-
PA conduits.)
Diagnostic
I B-NR
1. CMR is useful to quantify ventricular size and function, pulmonary valve
function, pulmonary artery anatomy, and left heart abnormalities in
patients with repaired TOF (S4.3.5-1).
I B-NR
2. Coronary artery compression testing is indicated before right ventricle–to-
PA conduit stenting or transcatheter valve placement in repaired TOF
(S4.3.5-2).
IIa B-NR
3. Programmed ventricular stimulation can be useful to risk-stratify adults
with TOF and additional risk factors for SCD (S4.3.5-3–S4.3.5-8).
IIa C-EO
4. In patients with repaired TOF, cardiac catheterization with angiography, if
indicated, is reasonable to assess hemodynamics when adequate data
cannot be obtained noninvasively in the setting of an arrhythmia, HF,
unexplained ventricular dysfunction, suspected pulmonary hypertension or
cyanosis.
Therapeutic
I B-NR
5. Pulmonary valve replacement (surgical or percutaneous) for relief of
symptoms is recommended for patients with repaired TOF and moderate
or greater PR with cardiovascular symptoms not otherwise explained
(S4.3.5-9–S4.3.5-11).
IIa B-NR
7. Pulmonary valve replacement (surgical or percutaneous) is reasonable for
preservation of ventricular size and function in asymptomatic patients with
repaired TOF and ventricular enlargement or dysfunction and moderate or
greater PR (S4.3.5-1, S4.3.5-9, S4.3.5-12–S4.3.5-14).
IIa B-NR
8. Primary prevention ICD therapy is reasonable in adults with TOF and
multiple risk factors for SCD (S4.3.5-15–S4.3.5-17).
IIb C-EO
9. Surgical pulmonary valve replacement may be reasonable for adults with
repaired TOF and moderate or greater PR with other lesions requiring
surgical interventions.
IIb C-EO
10. Pulmonary valve replacement, in addition to arrhythmia management,
may be considered for adults with repaired TOF and moderate or greater
PR and ventricular tachyarrhythmia.
Synopsis
Long-term survival after surgery for TOF continues to improve. However, residual hemodynamic and
electrophysiological abnormalities are common in adulthood. Adults with repaired TOF face an
increased risk of arrhythmias, exercise intolerance, HF, and death beginning in early adulthood (S4.3.5-1,
S4.3.5-18–S4.3.5-20). Surgical repair of TOF has evolved over time, with relief of the RVOT obstruction
usually involving infundibulotomy, resection of obstructive muscle bundles, and the use of a patch to
enlarge the pathway from the right ventricle to the pulmonary arteries. These procedures result in scar
tissue and create a dyskinetic and often aneurysmal area in the RVOT. Residual RVOT stenosis, branch
PA stenosis, residual ASD or VSD, TR, RV dilation and dysfunction, aortic dilation, AR, and LV dysfunction

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are some of the anatomic and functional abnormalities encountered in patients with repaired TOF. The
most common hemodynamic sequela of TOF repair is PR. Current evidence confirms that adults with
repaired TOF are at risk of severe PR, RV dilation and dysfunction, LV dysfunction and electromechanical
dyssynchrony, all of which contribute to adverse clinical outcomes late after TOF repair (S4.3.5-1, S4.3.5-
20–S4.3.5-24). Despite intense interest and numerous publications on pulmonary valve replacement in
adults with repaired TOF, optimal timing for this intervention remains uncertain, and most studies have
focused on preoperative RV volumes that would result in normalization of postoperative RV volumes
(S4.3.5-9, S4.3.5-14, S4.3.5-25–S4.3.5-27). In adults with repaired TOF, prevalence rates for atrial and
ventricular arrhythmias have been estimated to be 20% and 15%, respectively, with steep increases
after 45 years of age (S4.3.5-28). The incidence of SCD after surgical repair of TOF is approximately 2%
per decade (S4.3.5-18, S4.3.5-21, S4.3.5-24, S4.3.5-29, S4.3.5-30). Currently, factors associated with SCD
in patients with TOF have largely been identified from observational, predominantly retrospective
studies. Despite numerous studies that identified factors associated with malignant ventricular
arrhythmias and SCD, risk stratification remains imperfect.
Primary prevention ICDs should generally be considered in patients who otherwise meet
standard qualifying criteria (i.e., LV ejection fraction ≤35% with NYHA class II or III symptoms) (S4.3.5-
31–S4.3.5-33). There may be a role for primary prevention ICDs in selected adults with TOF who have
additional risk factors for SCD but would not meet standard criteria otherwise.
other procedures in these patients; Section 3.4 for recommendations on diagnostic evaluation; Figure 4
for a diagnostic and treatment algorithm for repaired TOF with residual PR; and Table 27 for routine
testing and follow-up intervals.
1. Cardiac magnetic resonance imaging is the gold standard imaging modality for quantification of right
ventricular size and function in patients with repaired TOF. It also allows for quantification of valve
regurgitation and pulmonary and systemic flows as well as delineating pulmonary artery anatomy and
detection of scar tissue in the ventricular myocardium. Serial cardiac magnetic resonance imaging
examinations allows for longitudinal follow-up of patients with repaired TOF and provides useful
information that aids in the timing of pulmonary valve replacement (S4.3.5-1, S4.3.5-34–S4.3.5-37).
2. Before any surgical or percutaneous intervention in patients with TOF, the origins and proximal
courses of the coronary arteries should be delineated. Patients with repaired TOF and abnormal
coronary artery anatomy have a substantial risk of coronary artery compression during percutaneous
pulmonary valve replacement or direct injury to the coronary during surgical pulmonary valve
replacement. During cardiac catheterization, the coronary pattern may be demonstrated by performing
simultaneous RVOT angiography and coronary angiography (S4.3.5-2). Coronary compression testing
generally involves simultaneous coronary angiography or aortography and balloon dilation of the RVOT
to ascertain whether a balloon expanded stent will compress the coronary artery.
3. Additional risk factors for SCD include (S4.3.5-24, S4.3.5-38, S4.3.5-39):
a. LV systolic or diastolic dysfunction
b. Nonsustained VT, QRS duration ≥180 ms
c. Extensive RV fibrosis by CMR
In adults with TOF, inducible sustained VT has been associated with an increased risk of clinical VT or
SCD, beyond standard ECG, hemodynamic, and clinical factors (S4.3.5-5). Programmed ventricular

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stimulation is most useful in risk stratifying patients at moderate risk of SCD rather than as a routine
surveillance tool in low-risk populations (S4.3.5-7).
4. Cardiac catheterization is the only method that can accurately and reliably determine PA pressure and
pulmonary vascular resistance.
5. Symptomatic patients (with dyspnea, chest pain, and/or exercise intolerance otherwise unexplained)
with repaired TOF and severe PR who undergo pulmonary valve replacement often report improved
functional class after intervention. Improvement in symptoms often correlates with a reduction in RV
size and relief of PR (S4.3.5-9–S4.3.5-11). Symptom improvement is more likely in patients with
underlying PS and PR than in patients with PR alone. For patients with significant LV or RV dysfunction,
pulmonary valve replacement may not be tolerated or sufficient; therefore, evaluation by ACHD
cardiologists and HF cardiologists is appropriate to decide appropriate course of action, particularly in
deciding if a patient may be appropriate for mechanical circulatory support or heart transplant.
6. Pulmonary valve replacement is reasonable in patients with at least 2 of the following (S4.3.5-1,
S4.3.5-9, S4.3.5-12–S4.3.5-14):
a. Mild or greater RV or LV dysfunction
b. Severe RV dilation (RV end-diastolic volume index ≥160 mL/m2
, RV end-systolic volume index
≥80 mL/m2
)
c. RV end-diastolic volume ≥2 times the LV end-diastolic volume
d. RV systolic pressure two thirds or higher systemic pressure
e. Progressive objective reduction in exercise capacity
The increasing use of CMR in the long-term follow-up for patients with repaired TOF has provided
quantification of ventricular size, function, and PR. However, there is lack of consensus regarding
optimal indications and timing of pulmonary valve replacement in this population. Pulmonary valve
replacement results in reduction of RV volume and relief of PR; however, these are only surrogates for
outcomes. Many patients with repaired TOF may deny symptoms yet demonstrate reduced exercise
tolerance. Pulmonary valve replacement in such patients has been associated with improved functional
status (S4.3.5-9, S4.3.5-10).
7. Risk factors for SCD include:
a. LV systolic or diastolic dysfunction
b. Nonsustained VT
c. QRS duration ≥180 ms
d. Extensive RV scarring
e. Inducible sustained VT at electrophysiological study
The largest study of patients with repaired TOF and ICDs included 121 patients from 11 North American
and European sites followed for a median of 3.7 years after ICD implantation. Overall, 30% of patients
received at least 1 appropriate ICD discharge, corresponding to annual appropriate shock rates of 7.7%
and 9.8% for primary and secondary prevention indications, respectively (S4.3.5-16). Unlike patients
with acquired HF, evidence suggests that patients with TOF who have inducible sustained polymorphic
VT (hazard ratio: 12.9) fare as poorly as or worse than those with inducible sustained monomorphic VT
(S4.3.5-5). Negative consequences associated with ICDs in adults with TOF must be carefully considered
in selecting appropriate candidates. These include high rates of inappropriate shocks (5% to 6% per
year), lead-related complications, and unfavorable patient-reported outcomes, including impaired QoL,
anxiety, depression, and psychosexual complications (S4.3.5-15, S4.3.5-17, S4.3.5-40).

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8. In patients with repaired TOF and moderate or greater PR who are undergoing cardiac surgery for a
separate lesion (e.g., RVOT aneurysm, TR, branch PA stenosis, residual VS D, arrhythmia ablation,
coronary artery revascularization, aortic root replacement), it may be reasonable to concurrently
perform pulmonary valve replacement (S4.3.5-41).
9. Although correction of the hemodynamic lesion (i.e., PR), may be clinically beneficial, pulmonary valve
replacement alone has not consistently been demonstrated to reduce risk of subsequent VT or SCD
(S4.3.5-42). Thus, in addition to pulmonary valve replacement, VT surgery and/or ICD implantation may
be considered (S4.3.5-43).
Table 27. TOF: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 24 12 12 12
TTE† 24 12–24 12 6–12
Holter monitor As needed As needed 12–24 12–24
CMR‡/CCT§ 36 24–36 12–24 12–24
Exercise test║ 36–60 24–60 12–24 12–24
‡CMR may be indicated for assessment of right ventricular size and funcƟon, pulmonary valve funcƟon, pulmonary artery
anatomy and left heart abnormalities. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat
imaging determined by anatomic and physiological findings.
§CCT may be used if CMR is not feasible and to evaluate origin and course of the coronary arteries, and cross-sectional imaging
status–post-stent therapy. If cardiac CCT is used instead of CMR imaging, the frequency should be weighed against radiation
exposure.
imaging; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; TOF, tetralogy of Fallot; and TTE, transthoracic
echocardiogram.

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Figure 4. Pulmonary Valve Replacement in Patients With TOF Repair and PR
*Symptoms may include dyspnea, chest pain, and/or exercise intolerance referable to PR or otherwise unexplained.
ACHD indicates adult congenital heart disease; HF, heart failure; LV, left ventricular; LVEDV, left ventricular end diastolic
volume; PR, pulmonary regurgitation; RV, right ventricular; RVEDV, right ventricular end diastolic volume; RVEDVI, right
ventricular end diastolic volume index; RVESVI, right ventricular end systolic volume index; RVOT, right ventricular outflow
tract; RVSP, right ventricular systolic pressure; and TOF, tetralogy of Fallot.
TOF repair with PR
Symptoms*
Any 2 of
the following:
• Mild or moderate RV or
LV systolic dysfunction
• Severe RV dilation
(RVEDVI ≥160 mL/m2
, or
RVESVI ≥80 mL/m2
, or
RVEDV ≥2x LVEDV)
• RVSP due to RVOT obstruction
≥2/3 systemic pressure
• Progressive reduction
in objective exercise
tolerance
Yes
Pulmonary valve
replacement
(Class I)
Yes
Moderate
or more PR
Follow-up with
ACHD cardiologist
(Class I)
PR severity
Pulmonary valve
replacement
(Class IIa)
No
Sustained
tachyarrhythmias
Yes
Pulmonary valve
replacement
(Class IIb)
Residual
lesions requiring surgical
interventions
Pulmonary valve
replacement
(Class IIb)
Follow-up with
ACHD cardiologist
(Class I)
Yes No
Mild PR
No
Severely
decreased LV or RV
systolic
function
No
Evaluation by an ACHD
cardiologist & advanced HF
team
(Class I)
Yes
No

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4.3.6. Right Ventricle–to-Pulmonary Artery Conduit
Recommendations for Right Ventricle–to-PA Conduit
Diagnostic
I B-NR
1. Coronary artery compression testing with simultaneous coronary
angiography and high-pressure balloon dilation in the conduit is
indicated before right ventricle–to-PA conduit stenting or transcatheter
valve placement (S4.3.6-1, S4.3.6-2).
I B-NR
2. In patients with stented right ventricle–to-PA conduits and worsening PS
or PR, evaluation for conduit complications should be performed,
including fluoroscopy to evaluate for stent fracture and blood cultures to
assess for IE (S4.3.6-3, S4.3.6-4).
IIa C-LD
3. In adults with right ventricle–to-PA conduit and arrhythmia, congestive
HF, unexplained ventricular dysfunction or cyanosis cardiac
catheterization is reasonable to assess the hemodynamics (S4.3.6-5,
S4.3.6-6).
Therapeutic
IIa B-NR
4. Right ventricle–to-PA conduit intervention is reasonable for adults with
right ventricle–to-PA conduit and moderate or greater PR or moderate or
greater stenosis (Table 22) with reduced functional capacity or
arrhythmia (S4.3.6-7–S4.3.6-11).
IIb B-NR
5. Right ventricle–to-PA conduit intervention may be reasonable for
asymptomatic adults with right ventricle–to-PA conduit and severe
stenosis or severe regurgitation with reduced RV ejection fraction or RV
dilation (S4.3.6-12–S4.3.6-14).
Synopsis
Right ventricle–to-PA conduits are widely used in the treatment of severe RVOT obstructive lesions
including pulmonary atresia. These conduits may be homografts or prosthetic conduits with
bioprosthetic valves used within the conduit. A minority of conduits may show early dysfunction
because of kinking or aneurysmal dilation. The remainder will become dysfunctional over time and
usually require replacement or intervention because of progressive stenosis within the conduit or at the
valve, and/or valvular regurgitation, at a mean interval of 10 to 15 years from placement, although some
conduits may last much longer than that (S4.3.6-15, S4.3.6-16).
1. Coronary compression testing generally involves simultaneous selective coronary angiography or
aortography and balloon dilation in the RVOT, to ascertain whether a balloon expanded stent will
compress the coronary artery. Coronary artery compression with conduit balloon angioplasty or stenting
occurs in approximately 5% to 6% of patients with right ventricle–to-PA conduits and usually involves
the left main/left anterior descending in those with conventional coronary anatomy. Patients with

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anomalous right or left coronary arteries are at risk of coronary compression as are those with
reimplanted coronary arteries.
2. Right ventricle–to-PA conduit stent fracture is common and occurred in approximately 26% of
patients in the Melody Valve Investigational Device Exemption trial (S4.3.6-7), especially in patients who
did not undergo conduit prestenting. Stent fracture typically presents with progressive stenosis and in
those with transcatheter valves may also present with worsening PR. Patients with an increase in PR or
PS should have fluoroscopic or x-ray assessment to rule out stent fracture.
Annualized rate of IE is up to 2.4% of patients treated with Melody valve implantation, but
infection in most cases involves valves other than the Melody valve, including left-sided valves (S4.3.6-3,
S4.3.6-17–S4.3.6-20). Patients typically present with fever and malaise as well as worsening PS or PR.
Cases may respond well to medical management with intravenous antibiotics if IE is identified and
treatment initiated early in the disease course, although sometimes surgical removal of the valve may
be necessary.
3. Although noninvasive imaging with echocardiography, CMR, or CTA provides a reasonably
comprehensive assessment of ventricular function, conduit function, and patency as well as pulmonary
arterial anatomy, cardiac catheterization is reasonable to directly assess hemodynamics in the setting of
clinical decompensation. Direct assessment of intracardiac and pulmonary arterial pressures and cardiac
output provides useful information regarding volume status, pulmonary arterial resistance, and degree
of conduit stenosis or regurgitation. Because of anatomic and technical factors, noninvasive imaging
may provide equivocal information and may underestimate the degree of conduit stenosis or
regurgitation; invasive assessment is especially important in such cases.
4. Right ventricle–to-PA conduit intervention includes surgical replacement or percutaneous stenting
and/or transcatheter valve placement. Patients with moderate or greater conduit stenosis (Table 22)
and/or regurgitation who have reduced exercise capacity or arrhythmias can benefit from surgical or
transcatheter conduit intervention to relieve stenosis and/or regurgitation. Transcatheter stenting and
pulmonary valve replacement may be performed with high procedural success and low mortality rates,
and result in improved hemodynamics and improved exercise capacity. Surgical conduit replacement
carries a higher risk of periprocedural complications with good long-term outcomes. Predictors of
conduit dysfunction and reoperation include placement of small diameter conduits; therefore, insertion
of conduits with the largest possible diameter should be attempted (S4.3.6-8), anticipating that
subsequent valve replacement may be via a transcatheter approach.
5. Right ventricle–to-PA conduit intervention, which includes surgical replacement or percutaneous
stenting and/or transcatheter valve placement, may be reasonable in asymptomatic patients with
severe right ventricle–to-PA conduit stenosis or regurgitation in the presence of reduced RV systolic
function or dilation in the expectation of improvement in hemodynamics, decreased RV size, improved
RV stroke volume, and improved RV ejection fraction. Moreover, peak oxygen consumption and
anaerobic threshold may also improve with conduit intervention.
Table 28. Right Ventricle–to-PA Conduit: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 12–24 12 12 12
TTE† 12–24 12 12 12
CMR‡/CCT§ 36–60 36–60 12–24 12–24

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Exercise test║ As needed As needed 12–24 12–24
‡CMR may be indicated for assessment of right ventricular size and function and valvular function, conduit anatomy and
pulmonary artery anatomy. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging
determined by anatomic and physiological findings.
§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status–post-stent therapy. If CCT is used
instead of CMR imaging, the frequency should be weighed against radiation exposure.
ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance;
CPET, cardiopulmonary exercise test; ECG, electrocardiogram; PA, pulmonary artery; and TTE, transthoracic echocardiogram.
4.4. Complex Lesions
4.4.1. Transposition of the Great Arteries
4.4.1.1. Transposition of the Great Arteries With Atrial Switch
Recommendations for d-TGA With Atrial Switch
Diagnostic
I C-EO
1. Ambulatory monitoring for bradycardia or sinus node dysfunction is
recommended for adults with d-TGA with atrial switch, especially if
treated with beta blockers or other rate-slowing agents.
I C-EO
2. Adults with d-TGA with atrial switch repair should undergo annual
imaging with either echocardiography or CMR to evaluate for common
long-term complications of the atrial switch.
IIa C-LD
3. Assessment for a communication through the interatrial baffle or venous
stenosis is reasonable for adults with d-TGA with atrial switch,
particularly if transvenous pacemaker/ICD implantation is considered or
leads are already present (S4.4.1.1-1).
Therapeutic
I B-NR
4. GDMT with appropriate attention to the need for anticoagulation is
recommended to promptly restore sinus rhythm for adults with d-TGA
with atrial switch repair presenting with atrial arrhythmia (S4.4.1.1-2).
Synopsis
Common problems for patients with d-TGA with atrial switch (Mustard or Senning procedure) include
leak across or obstruction of the venous pathways, arrhythmias, need for pacemakers/defibrillators, and
systolic dysfunction of the systemic ventricle. Although reports describing these sequelae abound, data
that inform management decisions are sparse, and many of the most common clinical issues cannot be
addressed by data-supported recommendations. Two such issues are medical therapy for RV
dysfunction and prevention of SCD.
The systematic review report, “Medical Therapy for Systemic Right Ventricles: A Systematic
Review (Part 1) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart
Disease,” has the complete systematic evidence review (S4.4.1.1-3) for additional data and analyses. The
results from the question “Are outcomes improved with angiotensin-converting enzyme inhibitors,
angiotensin-receptor blockers, beta blockers, or aldosterone antagonists alone or in combination in
patients with a systemic right ventricle?” and the writing committee’s review of the totality of the

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literature demonstrated that medical therapy for systolic ventricular dysfunction remains largely
uncertain. Consequently, no recommendations regarding specific medical therapy for systolic
dysfunction of the systemic right ventricle can be made.
In addition to the report provided by the ERC regarding angiotensin-converting enzyme
inhibitor, angiotensin-receptor blocker, and aldosterone antagonist use for patients with systemic right
ventricles, beta blockers and other commonly used HF medications lack data to support
recommendations in the treatment of atrial switch patients (S4.4.1.1-4–S4.4.1.1-7). Concerns regarding
routine use of beta blockers for asymptomatic RV dysfunction include potentially greater predisposition
to bradycardia and limited distensibility of the interatrial baffle, which creates a preload limited
physiology (S4.4.1.1-8). Although no clear benefit has been demonstrated for HF medical therapy
overall, there is speculation of benefit in more symptomatic patients or those with larger and/or more
dysfunctional right ventricles.
Patients with dysfunction of the systemic right ventricle are at risk of developing ventricular
arrhythmias. The role of ICD implantation for primary prevention of arrhythmia in patients with a low
systemic ventricular ejection fraction is uncertain. This practice is unsupported by any research and
cannot be universally recommended. Many such patients do not progress to receive therapies from
their device (S4.4.1.1-2). Decisions regarding primary prevention ICD implantation is based on the
patients' full clinical presentation and in consultation with cardiac electrophysiologists with ACHD
expertise.
other procedures in these patients; Section 3.4 for recommendations on diagnostic evaluation; Table 29
for routine testing and follow-up intervals; and Online Data Supplement 25 for referenced studies.
1. There is a progressive loss of sinus rhythm in patients who have undergone the Senning or Mustard
procedure for d-TGA, and the development of significant sinus bradycardia, while often asymptomatic,
is important to identify, because it will influence and limit treatment with antiarrhythmic medications.
2. Patients with d-TGA with atrial switch have abnormal cardiac anatomy, with common long-term
complications including systemic RV dysfunction, TR, subpulmonic obstruction, obstruction of systemic
or pulmonary venous return, and baffle leaks. Imaging should be goal-directed with an understanding of
potential long-term sequelae, and nuanced for the patient’s particular circumstances (S4.4.1.1-9). CMR
offers quantification of systemic RV function and should be used routinely unless there are
contraindications. Late gadolinium enhancement is an important tool that can identify areas of
myocardial scar that are associated with adverse clinical markers including atrial arrhythmia (S4.4.1.1-
10). The importance of change in late gadolinium enhancement over time in directing care is less clear,
so repetitive use of gadolinium contrast for this purpose is of less value.
3. Recognizing both the abnormal venous pathways after atrial switch palliation and the risk of
thromboembolic complications from transvenous pacing leads in those with intracardiac shunts,
thorough assessment of the venous pathways for either obstruction or baffle leak is a prudent step
before lead placement or revision. Baffle leaks should be sought because they are common and may
alter treatment considerations such as thromboembolic concerns or options for closure.
Echocardiography using agitated saline contrast is a sensitive method for this assessment. It is
unnecessary on every study, but interval assessment of baffle leak is appropriate, especially in
circumstances where therapy may be altered by the result. In some patients, injection in upper and
lower extremities may be necessary to evaluate superior and/or inferior systemic venous baffle leak,

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respectively, because a negative study from an injection in upper extremity may not exclude an inferior
systemic venous baffle leak. Assessment for baffle leak may involve use of TTE with agitated saline
contrast, TEE, intracardiac echocardiography, or angiography (S4.4.1.1-1).
4. Sustained intra-atrial reentrant tachycardia is a potential cause of SCD in adults who have undergone
atrial switch and puts patients at risk for thromboembolism. Treatment to maintain sinus rhythm may
involve antiarrhythmic medication or catheter ablation. Although there are not data demonstrating that
maintenance of sinus rhythm prevents SCD, there is evidence that atrial arrhythmias preceded or
coexisted with VT in 50% of cases, suggesting that atrial arrhythmias are a common trigger for
ventricular arrhythmias (S4.4.1.1-2, S4.4.1.1-11).
There is a biologically plausible explanation that may include longer atrial tachycardia cycle
lengths in the context of extensive atrial sutures/scar that could favor rapid (e.g., 1:1) ventricular
conduction, a reduction in stroke volume with faster heart rates attributable to poor atrial transport,
and myocardial ischemia despite the absence of CAD attributable to an inefficient coronary circulation
supplying the systemic ventricle (S4.4.1.1-12). Efforts to maintain sinus rhythm or atrial pacing (and not
simply rate control) should be the initial strategy of management, acknowledging that patients may
rarely tolerate permanent atrial tachycardia when attempts to maintain sinus rhythm have failed.
Atrial arrhythmias predominantly involve tissue of right atrial origin which, because of the
surgical anatomy, is found primarily in the pulmonary venous atrium, making access for catheter
ablation challenging.
Table 29. d-TGA With Atrial Switch: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 12 12 6–12 6–12
TTE† 12–24 12–24 12 12
Holter monitor 24 24 12 12
CMR‡/CCT§ 24–36 24 12–24 12–24
‡CMR may be indicated for assessment of ventricular size and function, systemic and venous baffle obstruction and leaks, and
valvular function. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined
anatomic and physiological findings.
§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status–post-stent therapy. If CCT is used
instead of CMR imaging, the frequency should be weighed against radiation exposure.
║6-minute exercise test or CPET, depending on clinical indication.
ACHD indicates adult congenital heart disease; CCT cardiac computed tomography; CMR, cardiovascular magnetic resonance
imaging; CPET, cardiopulmonary exercise test; d-TGA, dextro-transposition of the great arteries; ECG, electrocardiogram; and
TTE, transthoracic echocardiogram.

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4.4.1.2. Transposition of the Great Arteries With Arterial Switch
Recommendations for d-TGA With Arterial Switch
Diagnostic
I C-LD
1) Baseline and serial imaging with either echocardiography or CMR should
be performed in adults with d-TGA with arterial switch who have
neoaortic dilation, valve dysfunction or PA or branch PA stenosis or
ventricular dysfunction (S4.4.1.2-1–S4.4.1.2-3).
I C-EO
2) Coronary revascularization for adults with d-TGA with arterial switch
should be planned by surgeons or interventional cardiologists with
expertise in revascularization in collaboration with ACHD providers to
ensure coronary and pulmonary artery anatomy are understood
IIa B-NR
3) It is reasonable to perform anatomic evaluation of coronary artery
patency (catheter angiography, or CT or MR angiography) in
asymptomatic adults with d-TGA with arterial switch (S4.4.1.2-4,
S4.4.1.2-5).
IIa C-EO
4) Physiological tests of myocardial perfusion for adults with d-TGA after
arterial switch can be beneficial for assessing symptoms suggestive of
myocardial ischemia.
IIa C-EO
5) GDMT is reasonable to determine the need for coronary
revascularization for adults with d-TGA after arterial switch (S4.4.1.2-6–
S4.4.1.2-8).
Therapeutic
IIa C-EO
6) GDMT is reasonable to determine indications for aortic valve
replacement in adults with d-TGA after arterial switch with severe
neoaortic valve regurgitation (S4.4.1.2-6).
IIa C-EO
7) Catheter or surgical intervention for PS is reasonable in adults with d-
TGA after arterial switch with symptoms of HF or decreased exercise
capacity attributable to PS.
Synopsis
Complications after the arterial switch include: 1) stenosis at the arterial anastomotic sites, most
commonly supravalvular PS; 2) neoaortic root dilation; 3) neoaortic valve regurgitation (native
pulmonary valve); and 4) coronary obstruction. Evaluation for the first 3 complications listed is
accomplished by usual imaging, including echocardiography, CCT, and/or CMR. Coronary complications
are inadequately evaluated by resting echocardiography, and stress imaging in asymptomatic patients is
not sensitive. It is unclear that coronary abnormalities will present de novo or that those present in
childhood will progress. However, because patients did not receive an arterial switch before the late
1980s, the long-term natural history of the coronary arteries after arterial switch is still unknown. This is
particularly true regarding the impact of risks for concomitant acquired coronary artery disease in
patients whose coronary substrate is not normal. At this time, investigation and management of
suspected coronary abnormalities in adults with the arterial switch for TGA should largely be symptom-
driven and in accordance with existing guidelines for acquired coronary artery diseases.
Several residua and sequelae in adults after arterial switch merit consideration of reoperation.
Severe RVOT obstruction (Table 22) not amenable or responsive to percutaneous treatment is an

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indication for reoperation; lesser degrees of obstruction can be considered an indication for
intervention if greater degrees of exercise are desired. Pulmonary valve replacement or repair is often
considered when severe PR is present and there is significant RV dilation or RV dysfunction. Coronary
ostial stenosis late after arterial switch may be repaired by coronary artery bypass graft surgery or ostial
arterioplasty techniques. The threshold aortic diameter at which dissection/rupture risk exceeds the risk
of operation is not known, and consequently the threshold for prophylactic operation for neoaortic root
dilation is undefined.
1. Imaging in patients after arterial switch should be performed with specific sequelae in mind, including
PS (recognizing the Lecompte maneuver has been used during surgery in most), and neoaortic root and
valve problems. Some patients with early arterial switch repairs had right ventricle–to-PA conduits
placed and may have related complications.
2. Because of nuances of the arterial switch, decisions regarding coronary intervention should be
considered jointly by ACHD providers and those with expertise in coronary revascularization techniques.
Abnormalities commonly occur proximally and close to the anteriorly positioned coronary buttons.
Coronary buttons are usually located posterior to the main PA after the Lecompte maneuver.
Revascularization techniques may include revision of the coronary buttons, ostioplasty, interposition
grafts, or coronary bypass grafting.
3. There is evidence that coronary abnormalities are common after arterial switch (6% to 10%),
especially in the setting of coronary anomalies at birth, or extensive manipulation of the coronaries at
the time of the operation. However, most coronary problems and events described so far tend to occur
in childhood in the first few years after surgery, with limited experience in adults (S4.4.1.2-1, S4.4.1.2-2,
S4.4.1.2-4, S4.4.1.2-5), although the prevalence of coronary issues may increase as the population ages.
Physiological testing lacks sensitivity. Therefore, a benchmark assessment of the anatomic course and
patency of the coronary arteries (i.e., catheter angiography or CT angiography) is prudent in adults in
whom this information has not already been obtained. MR coronary angiography may also be an option
for evaluating coronary patency (S4.4.1.2-9). Thereafter, coronary investigations will be prompted
largely by symptoms.
4. Once the coronary anatomy in an arterial switch recipient is documented, there is little justification
for serial anatomic imaging in an asymptomatic individual. Symptomatic patients should be offered
stress physiological imaging and repeat anatomic imaging considered if symptoms are suggestive of
coronary ischemia (S4.4.1.2-8).
5. Decisions about the indications and approach for coronary intervention after an arterial switch can be
guided according to management recommendations for care of atherosclerotic coronary disease,
emphasizing prudent medical therapy and a symptom-guided approach to intervention (S4.4.1.2-6,
S4.4.1.2-8). The unique aspects of the anatomic abnormalities and unusual course of the proximal
coronary arteries must be kept in mind, mandating collaboration between ACHD providers and those
with the necessary surgical or interventional expertise.
6. Although some degree of neoaortic valve regurgitation is common, surgery to replace the neoaortic
valve has only rarely been reported. Indications for valve replacement should be based on LV size and/or
symptoms according to the 2014 VHD guideline (S4.4.1.2-6). The more common concern is dilation of

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the neoaortic root with preserved aortic valve competence. Valve-sparing root replacement is often
considered in such cases, but surgical options should be individualized based upon anatomy and
changes over time. There are not data to support a specific aortic diameter beyond which the risk of
dissection or rupture increases sufficiently to warrant prophylactic aortic replacement.
7. PS affects 5% to 15% of patients after arterial switch (S4.4.1.2-1–S4.4.1.2-3, S4.4.1.2-10, S4.4.1.2-11)
and may occur anywhere in the pulmonary tree including the pulmonary valve, main PA, and branch
pulmonary arteries. Interventional decisions should be guided by a combination of symptoms and
severity of stenosis.
Table 30. d-TGA With Arterial Switch: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 12–24 12–24 12 6
TTE† 12–24 12–24 12 12
CMR‡/CCT§ 36–60 24–36 12–24 12–24
Exercise test║ 36–60 36–60 24–36 12–24
*See ACHD AP classification Table 4.
‡CMR may be indicated for assessment of neoaorƟc size, the origin and proximal course of the coronary arteries, branch
pulmonary arteries, ventricular function and valvular function. Baseline study is recommended with periodic follow-up CMR,
with frequency of repeat imaging determined by anatomic and physiological findings.
§CCT or catheterization once to establish knowledge of coronary artery anatomy and then as warranted by clinical condition.
CCT may be used if CMR is not feasible and to evaluate coronary artery anatomy and cross-sectional imaging status-post stent
therapy. If CCT is used instead of CMR imaging, the frequency should be weighed against radiation exposure.
║6-minute exercise test or CPET, depending on clinical indication.
imaging; CPET, cardiopulmonary exercise test; d-TGA, dextro-transposition of the great arteries; ECG, electrocardiogram; and
TTE, transthoracic echocardiogram.
4.4.1.3. Transposition of the Great Arteries With Rastelli Type Repair
other procedures in these patients; and Section 3.4 for recommendations on diagnostic evaluation.
The Rastelli operation is performed in patients with d-TGA with VSD and PS and for variations of double
outlet right ventricle with PS. The operation consists of 2 main components:
1. An intracardiac baffle that directs oxygenated blood from the left ventricle via a nonrestrictive
VSD to the aorta.
2. A right ventricle–to-PA conduit, which is usually valved.
The operation is designed to use the morphologic left ventricle as the systemic ventricle and the
morphologic right ventricle as the subpulmonic ventricle. Long-term considerations after the Rastelli
operation include:
1. Right ventricle–to-PA conduit dysfunction (Section 4.3.6)
2. VSD patch leaks/dehiscence (Section 4.1.3)
3. LV-to-aorta internal baffle stenosis (Section 4.2.3)

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4. Scar-based VT
Medical treatment, catheter interventions, and surgical interventions for each of these conditions,
which may occur in isolation or in combination, may be considered in accordance with the
recommended treatments for each of the individual conditions as outlined in this guideline document.
4.4.1.4. Congenitally Corrected Transposition of the Great Arteries
Recommendations for Congenitally Corrected Transposition of the Great Arteries
Diagnostic
IIa C-LD
1. CMR is reasonable in adults with CCTGA to determine systemic RV
dimensions and systolic function (S4.4.1.4-1, S4.4.1.4-2).
Therapeutic
I B-NR
2. Tricuspid valve replacement is recommended for symptomatic adults
with CCTGA and severe TR, and preserved or mildly depressed systemic
ventricular function (S4.4.1.4-3, S4.4.1.4-4).
IIa C-LD
3. Tricuspid valve replacement is reasonable for asymptomatic adults with
CCTGA and severe TR with dilation or mild dysfunction of the systemic
ventricle (S4.4.1.4-3).
IIb B-NR
4. Conduit intervention/replacement may be considered for adults with
CCTGA and symptomatic subpulmonary left ventricle–to-PA conduit
dysfunction, recognizing that unloading the subpulmonary ventricle may
have a detrimental impact on systemic atrioventricular valve function
(S4.4.1.4-5).
Synopsis
The clinical course of adults with CCTGA often depends on the presence and severity of associated
cardiac anomalies (S4.4.1.4-6), which will often have required pediatric intervention. Rarely, CCTGA may
be first diagnosed in adulthood, particularly if patients do not have associated cardiac lesions.
Conduction abnormalities are common, and the prevalence of spontaneous complete heart block
increases with age (S4.4.1.4-7, S4.4.1.4-8). PS, ASD, and VSD are common. Seventy percent to 90% of
patients with CCTGA have a dysplastic or Ebstein-like malformation of the tricuspid valve. This
anatomically abnormal systemic atrioventricular valve is at risk of progressive TR, which is an
independent predictor of death in CCTGA (S4.4.1.4-4, S4.4.1.4-9).
The systematic review report, “Medical Therapy for Systemic Right Ventricles: A Systematic
Review (Part 1) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart
Disease.” (S4.4.1.4-10) addressed the role of medical therapies for management of functional
deterioration in systemic RVs (S4.4.1.4-11–S4.4.1.4-13) (see additional details in Section 3.17).

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1. CMR is useful for quantification of systemic RV size and function (S4.4.1.4-1, S4.4.1.4-2).
Administration of gadolinium contrast is useful in identifying fibrotic myocardium demonstrated by late
gadolinium enhancement (S4.4.1.4-14).
2. Symptomatic adults with CCTGA and severe TR with no more than mildly depressed systemic
ventricular function should be evaluated for tricuspid valve replacement. In general, tricuspid valve
replacement is preferred to tricuspid repair in the adult CCTGA population. TR is often because of a
dysplastic tricuspid valve and has been shown to be an independent predictor of death in CCTGA
patients (S4.4.1.4-4). Systemic RV dysfunction is often attributable to longstanding TR, and efforts
should be made to relieve the TR before worsening dysfunction (S4.4.1.4-3, S4.4.1.4-9). Tricuspid valve
repair has been attempted; however, recurrent clinically significant TR is observed frequently after
tricuspid valve repair in patients with CCTGA; hence, valve replacement is preferred (S4.4.1.4-15).
3. Many adult CCTGA patients are referred for tricuspid valve replacement late, when symptomatic and
already suffering from moderate-to-severe TR and ventricular dysfunction (S4.4.1.4-16). In CCTGA
patients referred for TVR, 10-year postoperative survival is <20% when the preoperative systemic
ventricular ejection fraction is <40% (S4.4.1.4-9) or 44% (S4.4.1.4-3). In a retrospective review of 46
CCTGA patients referred for TR surgery, preoperative systemic ventricular ejection fraction was the only
independent predictor of postoperative systemic ventricular ejection fraction at 1 year (S4.4.1.4-3).
4. Adults with CCTGA and pulmonary atresia or stenosis were often managed in childhood by placing a
conduit from the morphologic LV to the PA, and progressive conduit dysfunction is common. Conduit
intervention or replacement will diminish the pressure in the subpulmonic ventricle and may result in
ventricular septal shift toward the subpulmonic left ventricle, including the septal leaflet of the systemic
tricuspid valve and thus can result in worsening of TR and a detrimental impact on systemic RV function
(S4.4.1.4-5).
Table 31. CCTGA: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
ECG 12 12 12 12
TTE† 12–24 12 12 12
Holter monitor 12–60 12–60 12–36 12
CMR‡/CCT§ 36–60 36–60 12–24 12
Exercise test║ 36–60 36–60 12–24 12
‡CMR may be indicated for assessment of ventricular size and funcƟon and valvular funcƟon. Baseline study is recommended
with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.
§CCT may be used if CMR is not feasible. If CCT is used instead of CMR imaging, the frequency should be weighed against
radiation exposure.
ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CCTGA, congenitally corrected
transposition of the great arteries; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test;

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4.4.2. Fontan Palliation of Single Ventricle Physiology (Including Tricuspid
Atresia and Double Inlet Left Ventricle)
Recommendations for Fontan Palliation of Single Ventricle Physiology
Diagnostic
I C-LD
1. New presentation of an atrial tachyarrhythmia in adults with Fontan
palliation should be managed promptly and include prevention of
thromboembolic events and consultation with an electrophysiologist
with CHD expertise (S4.4.2-1, S4.4.2-2).
I C-EO
2. Adults after Fontan palliation should be evaluated annually with either
echocardiography or CMR.
I C-EO
3. Cardiac catheterization should be performed in adults before initial
Fontan surgery or revision of a prior Fontan connection to assess
suitability of preintervention hemodynamics for Fontan physiology or
revision of a prior Fontan connection.
I C-EO
4. New onset or worsening atrial tachyarrhythmias in adults with single
ventricle after Fontan palliation should prompt a search for potential
hemodynamic abnormalities, which may necessitate imaging and/or
cardiac catheterization.
IIa B-R
5. In adults with Fontan palliation, it is reasonable to encourage a regular
exercise program appropriate to their abilities (S4.4.2-3–S4.4.2-5).
IIa C-LD
6. Imaging of the liver (ultrasonography, CMR, CT) and laboratory
evaluation of liver function for fibrosis, cirrhosis, and/or hepatocellular
carcinoma are reasonable in adults after Fontan palliation (S4.4.2-6).
IIa C-EO
7. In adults after Fontan palliation, it is reasonable to perform biochemical
and hematological testing on an annual basis especially for liver and
renal function.
IIa C-LD
8. Cardiac catheterization can be useful to evaluate a symptomatic adult
after Fontan palliation when noninvasive testing is insufficient to guide
therapy (S4.4.2-7, S4.4.2-8).
IIa C-LD
9. Evaluation for cardiac transplantation is reasonable in adults with Fontan
palliation and signs and symptoms of protein-losing enteropathy (S4.4.2-
9–S4.4.2-12).
IIb C-EO
10. It may be reasonable to perform catheterization in asymptomatic adults
after Fontan palliation to evaluate hemodynamics, oxygenation and
cardiac function to guide optimal medical, interventional and/or surgical
therapy.
Therapeutic
I C-EO
11. Anticoagulation with a vitamin K antagonist is recommended for adults
with Fontan palliation with known or suspected thrombus,
thromboembolic events, or prior atrial arrhythmia, and no
contraindications to anticoagulation.
IIa C-LD
12. Catheter ablation can be useful in adults after Fontan palliation with
intra-atrial reentrant tachycardia or focal atrial tachycardia (S4.4.2-13–
S4.4.2-15).

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IIa C-LD
13. Fontan revision surgery, including arrhythmia surgery as indicated, is
reasonable for adults with atriopulmonary Fontan connections with
recurrent atrial tachyarrhythmias refractory to pharmacological therapy
and catheter ablation who have preserved systolic ventricular function
and severe atrial dilation (S4.4.2-16–S4.4.2-18).
IIa B-R
14. Pulmonary vasoactive medications can be beneficial to improve exercise
capacity in adults with Fontan repair (S4.4.2-19–S4.4.2-25).
IIb B-NR
15. Antiplatelet therapy or anticoagulation with a vitamin K antagonist may
be considered in adults after Fontan palliation without known or
suspected thrombus, thromboembolic events, or prior arrhythmia
(S4.4.2-26).
IIb C-LD
16. Reoperation or intervention for structural/anatomic abnormalities in a
Fontan palliated patient with symptoms or with failure of the Fontan
circulation may be considered (S4.4.2-27).
Synopsis
Fontan repairs are the most common palliation of single ventricle physiology seen in adults. The
physiology is complex, with long-term consequences related to the obligatory elevation in central
venous pressure and reduced cardiac output. Proposed medical therapy for the “failing Fontan,” which
may manifest as protein-losing enteropathy, hepatic dysfunction, lower extremity venous congestion,
and/or exercise limitation, has included many different modalities, although there is limited proven
benefit in published research. Options for medical therapy include aldosterone antagonists or
subcutaneous unfractionated heparin, which may stabilize the proteoglycan layer of the gut. PAH
therapies are of increasing interest. Endothelin antagonists have been studied in a single RCT, which
showed improved exercise capacity in 75 subjects randomized to bosentan compared with placebo
(S4.4.2-19). Two other small nonrandomized studies demonstrated minimal response to therapy (S4.4.2-
22, S4.4.2-23).
Corticosteroids, specifically budesonide, may be helpful for Fontan patients with
hypoalbuminemia in the setting of protein-losing enteropathy poorly responsive to other therapies.
Budesonide seems to have fewer systemic effects than other oral steroids; however, close monitoring
for signs of hypercortisolism remains necessary (S4.4.2-28, S4.4.2-29). Octreotide may be considered; it
is a therapy with favorable but very limited anecdotal experience reported, with further research
needed (S4.4.2-28, S4.4.2-30). A combination of these therapies may be applied in an affected patient,
as such strategies collectively appear to have produced improved outcome compared with historic
controls (S4.4.2-31).
Fontan surgery has been associated with prolongation of atrial refractory periods, extensive
atrial scarring, and intra-atrial conduction delay (S4.4.2-32–S4.4.2-38). Sinus node dysfunction occurs in
up to 45% of adults during long-term follow-up after Fontan surgery and has been associated with a
reduction in preload to the single ventricle, increased pulmonary venous pressure, reduced cardiac
output, plastic bronchitis, and protein-losing enteropathy (S4.4.2-39–S4.4.2-43). Transvenous atrial
pacing may be feasible in most adults with atriopulmonary Fontan connections and in some with
intracardiac lateral tunnels (S4.4.2-44), although the potential for thrombotic complications must be
addressed. Ventricular pacing may be performed via the coronary sinus in selected patients, but most
require an epicardial approach (S4.4.2-45, S4.4.2-46). Management of atrial arrhythmias is discussed in
the associated recommendations.

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See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization and
1. Atrial tachyarrhythmias occur in up to 60% of adults with Fontan palliation and are associated with
substantial morbidity and mortality (S4.4.2-2). These arrhythmias may be difficult to manage, are usually
poorly tolerated, and cause serious hemodynamic compromise often with dire consequences (S4.4.2-1).
Therefore, they should be addressed promptly, including urgent consultation with ACHD providers who
can help guide immediate management strategies, even if remotely. Consideration for antithrombotic
therapy in Fontan patients should take into account the high prevalence of thrombus formation and
potentially catastrophic impact of pulmonary or systemic thromboembolus. Standard decision-making
strategies about rhythm versus rate control or thromboembolic prophylaxis derived from and
recommended for patients with acquired heart disease and AF do not apply to patients with Fontan
physiology, for whom rhythm control and anticoagulation are of greater importance than would be
concluded from application of the standard algorithms.
Sinus node dysfunction may predispose Fontan patients to atrial tachyarrhythmias, the most
common being macro-reentrant circuits or intra-atrial reentrant tachycardia (S4.4.2-13, S4.4.2-47).
Nearly 90% of Fontan patients who die from HF have coexisting atrial tachyarrhythmias (S4.4.2-48).
Fontan patients are at increased risk of complications from antiarrhythmic therapy, such as torsades de
pointes with dofetilide (S4.4.2-49) and amiodarone-induced thyrotoxicosis (S4.4.2-50). Such agents
should be used cautiously and in consultation with ACHD cardiologists and electrophysiology specialists
with expertise in ACHD.
2. Serial imaging can be valuable for assessing many of the long-term sequelae of Fontan palliation such
as thrombosis, right-to-left shunts (e.g., fenestration, intrapulmonary AV malformation), obstructive
lesions, systemic AV valve dysfunction, diastolic or systolic ventricular function, collateral burden, and
branch PA obstruction. Imaging can be challenging and requires informed understanding about the
patient's particular situation. Although CCT is possible in patients with Fontan physiology, it is
challenging to ensure contrast dispersal through the pulmonary vasculature because of streaming of
venous return to the PA from multiple separate sources (e.g., superior vena cava, inferior vena cava
right atrium collaterals) (S4.4.2-51, S4.4.2-52).
3. Hemodynamic assessment, particularly of the pulmonary circulation, is crucial to making informed
decisions about the type and timing of surgical intervention.
4. Hemodynamic problems may first manifest through arrhythmia. Thus, first presentation of arrhythmia
should warrant thorough review of the patient’s Fontan circulation and ventricular function.
5. Aerobic exercise may help maintain respiratory mechanics, which can improve transpulmonary flow in
the Fontan circulation. Stroke volume during exercise and exercise capacity are directly related to
skeletal muscle function. Consequently, strength training may improve exercise capacity in patients with
Fontan palliation.
6. There is increasing recognition of hepatic vulnerability after Fontan palliation, including cirrhosis
(S4.4.2-6, S4.4.2-53) but uncertainty about which patients are at highest risk, or how to address
problems when identified. Routine assessment of liver function and structure may help inform broader
decisions such as timing and risk of surgery or transplantation, as well as provide insights into the
emerging natural history of this unique condition. Consultation with a hepatologist may be of value in
interpreting and following the liver abnormalities encountered in patients with Fontan physiology.

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7. Recognizing the multiorgan vulnerability of the Fontan circulation, annual routine blood tests may
have a role in identifying and addressing problems early.
8. Because of both the anatomic and physiological complexities of these patients, and the potential for
concurrent intervention, hemodynamic and interventional cardiac catheterization of the adult with
single ventricle/Fontan palliation should be performed only by persons with expertise in CHD in
coordination with an ACHD cardiologist. Recognizing that it is difficult to accurately assess Fontan
hemodynamics by clinical examination or noninvasive imaging, cardiac catheterization may be needed in
these scenarios and others:
a. Interval hemodynamic assessment, as filling pressures, mean PA pressure, and pulmonary
vascular resistance may change over time (S4.4.2-8)
b. Creation or closure of a fenestration or veno-veno collaterals, although with uncertain benefit of
either intervention (S4.4.2-54–S4.4.2-56)
c. Treatment of baffle obstruction, even in the setting of low or no pressure gradient (S4.4.2-57)
d. Assessment of protein-losing enteropathy or ascites, because elevated Fontan pressure
correlates with such complications, and lowering pressures may offer the potential for clinical
improvement (S4.4.2-58)
e. Facilitation of transvenous liver biopsy for monitoring liver function including as part of a
pretransplantation assessment
f. Preoperative assessment before Fontan revision (S4.4.2-18, S4.4.2-59).
9. Protein-losing enteropathy and plastic bronchitis contribute substantially to perioperative mortality,
yet transplantation may be curative (S4.4.2-9, S4.4.2-11). Medical therapy options are often ineffective.
Therefore, consideration of transplantation early in the course of PLE may be warranted. Evaluation of
additional organs is necessary, particularly the liver, as these patients are susceptible to cirrhosis as a
consequence of the Fontan circulation. Although symptoms may improve, there are no published data
regarding impact on survival for transplanted Fontan patients with PLE compared with those who do not
undergo transplantation.
10. Although catheterization plays an important role in management of single ventricle/Fontan patients,
it is often driven by symptoms. The role of routine hemodynamic assessment is less certain.
11. Fontan circulation imparts risk of thrombosis, and anticoagulation with vitamin K antagonists should
be offered as preventive therapy in clinical situations including prior arrhythmia (S4.4.2-60). Patients
may also benefit from anticoagulation if they have significant residual intracardiac right- to -left shunt or
veno-veno collaterals.
12. Catheter ablation has been associated with improved clinical status despite the frequent coexistence
of multiple arrhythmia substrates (S4.4.2-61). Given the progressive nature of the atrial myopathy,
successful ablation is less frequent than in acquired heart disease or other congenital heart diseases,
and recurrence is common. The development of new arrhythmias over time remains problematic, but
multiple ablation procedures may be justified in selected patients (S4.4.2-13, S4.4.2-14).
13. Conversion to a total cavopulmonary connection Fontan combined with a modified right atrial Maze
procedure may be considered in patients with symptomatic refractory recurrent intra-atrial reentrant
tachycardia (S4.4.2-16, S4.4.2-17). In the presence of documented AF, a left atrial Cox Maze procedure
may also be indicated (S4.4.2-16, S4.4.2-62). Some patients may not be appropriate surgical candidates
for reasons of elevated PA or Fontan pressures, elevated ventricular end-diastolic pressures, or renal or
hepatic dysfunction, and the decision to perform Fontan revision surgery is rarely straightforward.

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14. Pulmonary vasoactive medications, specifically endothelin receptor antagonists and PDE-5 inhibitors,
are of increasing interest as a means of reducing pulmonary vascular resistance and improving cardiac
output. In limited studies, use of PDE-5 inhibitors appears favorable for Fontan patients with
improvement noted in pulmonary blood flow and exercise capacity (S4.4.2-21, S4.4.2-63). Use of
endothelin antagonists has been investigated in a randomized trial (S4.4.2-19). After 14 weeks of
randomization in 69 subjects successfully completing the study, there was a modest but significant
increase in peak oxygen consumption and exercise duration in those taking bosentan compared with
those on placebo.
15. Although anticoagulation is prudent in those with prior arrhythmia or known thromboembolic
events, routine use of anticoagulation with vitamin K antagonist cannot as yet be strongly
recommended. An RCT in Fontan children/adolescents did not show benefit (S4.4.2-64), although adults
later after Fontan may be more at risk. However, a secondary analysis of that RCT as an observational
study (S4.4.2-65) found the risk of thromboembolism was lower in those patients on warfarin who
consistently achieved minimum target international normalized ratio levels, as well as in those on
acetylsalicylic acid compared with patients who often failed to meet target international normalized
ratio level. Rates of thrombosis were considerably higher in patients on warfarin who did not
consistently achieve target international normalized ratio. A study of modes of death in atriopulmonary
Fontan patients demonstrated lower rates of death in patients on “some” antiplatelet agent or
anticoagulation compared with those on none (S4.4.2-1). Direct oral anticoagulants are unstudied and
thus cannot be recommended at the present time. There are concerns about liver function vulnerability
in Fontan patients, which theoretically may increase the risk of complications with some of those
agents.
16. There are occasions where surgery or catheter intervention may be alternatives to transplantation
for a “failing Fontan” after weighing risks and benefits of the intended procedure (i.e., alleviation of
atrioventricular valve regurgitation, systemic or pulmonary venous pathway obstruction). Reoperation
for atrioventricular valve regurgitation may be high-risk, particularly when systemic ventricular function
is impaired. Although valve repair is preferred and operative risk is usually lower, it is not always
possible. Risk of valve replacement in this setting is high.
Table 32. Fontan Palliation: Routine Follow-Up and Testing Intervals
and Testing
Physiological
Stage A* (mo)
Physiologic al
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
Outpatient ACHD cardiologist 12 12 6 3–6
ECG 12 12 6–12 6
TTE† 12 12 12 12
Holter monitor 12 12 12 12
CMR‡/CCT§ 36 24 24 24
‡CMR may be indicated for assessment of the long-term sequelae of Fontan palliation: thrombosis, right-to-left shunts (e.g.,
fenestration. intrapulmonary atrioventricular malformation), obstructive lesion, systemic atrioventricular valve dysfunction,
ventricular size and function, collateral burden, and branch pulmonary artery obstruction. Baseline study is recommended with
periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings.
§CCT may be used if CMR is not feasible and to evaluate cross-sectional imaging status-post stent therapy. CCT with contrast
injection in Fontan patients can be misleading; therefore, it should be done only when clinically indicated and when it can be

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appropriately protocoled and interpreted. If CCT is used instead of CMR imaging, the frequency should be weighed against
radiation exposure.
imaging; CPET, cardiopulmonary exercise test; ECG, electrocardiogram; and TTE, transthoracic echocardiogram.
4.4.3. Hypoplastic Left Heart Syndrome/Norwood Repair
The Norwood repair is the first of 3 steps in palliation for hypoplastic left heart syndrome and consists of
atrial septectomy, transection, and ligation of the distal main PA with construction of a systemic-to-PA
shunt, and anastomosis of the proximal stump of the main PA to the hypoplastic ascending aorta with
augmentation of the entire aortic arch from the sinotubular junction to beyond the ductus arteriosus.
Hypoplastic left heart syndrome is fatal unless surgical palliation is performed in the neonatal period.
Subsequent surgeries include a bidirectional cavopulmonary anastomosis (often performed around 6
months of age), followed finally by a Fontan procedure (often at approximately 2 to 4 years of age).
Sequelae of hypoplastic left heart syndrome are largely those of the Fontan palliation, but additional
concerns related to the underlying anatomy and the Norwood repair is important in patients with
hypoplastic left heart syndrome. These include aortic obstruction related to anastomosis of the PA and
aorta, and neoaortic dilation. Additionally, native anatomy wherein coronary arteries arise from a small
aortic root make coronary ischemia a greater concern than in other underlying disorders managed with
Fontan repair. The frequency and spectrum of long-term sequelae specific to the Norwood repair are
not yet known.
4.4.4. Truncus Arteriosus
Truncus arteriosus in the adult has almost invariably been repaired in childhood, and in the rare
circumstances when an adult has unrepaired truncus arteriosus, Eisenmenger physiology is typical.
Pulmonary hypertension may be present in repaired patients. The types of operative repairs may involve
VSD closure, right ventricle–to-PA conduit placement, reconstruction of the pulmonary arteries, and
replacement of the truncal (neoaortic) valve. Unifocalization of the pulmonary arteries may be
necessary in very complex cases. The aorta may be dilated. Recommendations regarding assessment
and management of truncus arteriosus can generally be inferred in the recommendations for the
specific components, including right ventricle–to-PA conduit, VSD, aortic valve disease, and
aortopathies.
4.4.5. Double Outlet Right Ventricle
Double outlet right ventricle is an anatomic descriptor that includes abnormalities similar to TOF in some
patients (when the aorta is closely related to the VSD) and similar to d-TGA with a VSD in others (when
the PA is more closely related to the VSD than the aorta). Repairs are predicated on the underlying
anatomy and may involve VSD closure with relief of PS, right ventricle–to-PA conduit, or Rastelli-type
repair. In severe cases, single-ventricle physiology may be present. Consequently, recommendations for
the management of a patient with double outlet right ventricle can generally be inferred in the
recommendations for the lesion with the most similar anatomy and physiology (e.g., TOF can reasonably
be based on the recommendations in Section 4.4.1, recognizing that a patient with double outlet right
ventricle is more likely to have residual LVOT obstruction).
4.4.6. Severe PAH and Eisenmenger Syndrome

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4.4.6.1. Severe PAH
Recommendations for Severe PAH
Diagnostic
I B-NR
1. Patients with ACHD with pulmonary vascular resistance 2.5 Wood units
or greater (≥4 Wood units/m2
) should be assessed collaboratively by an
ACHD cardiologist and an expert in pulmonary hypertension to develop a
management plan (S4.4.6.1-1–S4.4.6.1-17).
I B-NR
2. Adults with septal or great artery shunts should undergo periodic
screening for pulmonary hypertension with TTE (S4.4.6.1-1–S4.4.6.1-18).
I B-NR
3. Cardiac catheterization to assess pulmonary vascular hemodynamics is
recommended for adults with septal or great artery shunts and clinical
symptoms, signs, or echocardiographic findings suggestive of pulmonary
hypertension (S4.4.6.1-1, S4.4.6.1-2, S4.4.6.1-4, S4.4.6.1-6, S4.4.6.1-7,
S4.4.6.1-11, S4.4.6.1-12, S4.4.6.1-15–S4.4.6.1-18).
I B-NR
4. In adults with septal or great artery shunts, cardiac catheterization with
hemodynamics (performed before or at time of closure) is beneficial to
assess suitability for closure (S4.4.6.1-1–S4.4.6.1-17).
I C-EO
5. BNP, chest x-ray, 6-minute walk test, and cardiac catheterization are
useful for initial and follow-up evaluation of patients with ACHD with
PAH.
Synopsis
Pulmonary hypertension is defined as elevation of mean pulmonary arterial pressure to ≥25 mm Hg at
rest and does not imply a specific underlying pathophysiology. Pulmonary hypertension is further
classified on the basis of the presumed mechanism (including elevation of pulmonary venous pressure
[denoted as “postcapillary pulmonary hypertension”], parenchymal or restrictive lung disease,
rheumatologic disease, portal hypertension, toxin exposure, and thromboembolism). It is also classified
by developmental or acquired anatomic abnormalities of decreased pulmonary arterial capacitance,
impedance, or stenosis throughout the pulmonary arterial vascular bed. PAH as initially described
required pulmonary venous pressure ≤15 mm Hg with concomitant elevation of pulmonary vascular
resistance. Although left-to-right shunting was the initial research model of triggered PAH, pulmonary
hypertension in patients with ACHD can be caused by, or associated with, any of the factors described
above. Effective therapies may be specific to the primary mechanism of pulmonary hypertension in a
given patient, so patients with CHD should have thorough investigation for all potential contributing
etiologies to pulmonary hypertension that may require specific therapy if best clinical outcomes are to
be achieved. Adverse effects of pulmonary hypertension therapies in patients with ACHD with
pulmonary hypertension may differ from those noted in other patients, because of concomitant
multiorgan and vascular effects from longstanding congenital heart and vascular disease.
Shunt-related PAH in patients with ACHD can develop in the pre- or perioperative period but
also may develop years to decades after closure of defects. Mechanisms for development of PAH may
include genetic factors and environmental exposures. Severity of PAH may range from incidentally noted
mild pressure and resistance elevation to profound systemic or suprasystemic levels of PA pressure and
pulmonary vascular resistance. If an anatomic defect that allows shunting is present, shunt reversal and

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cyanosis may develop as pulmonary resistance rises above systemic resistance (i.e., Eisenmenger
syndrome).
1. Patients with ACHD with pulmonary hypertension, particularly PAH, have a poorer prognosis than do
patients with ACHD with similar histories and anatomic abnormalities who do not have pulmonary
hypertension. The fields of ACHD and pulmonary vascular disease care have increasingly disparate but
complementary bodies of knowledge, and both are necessary to achieve optimal outcomes for patients
with PAH. Clinicians cross-trained in both subspecialties or partnering experts from each subspecialty
appear necessary to fully counsel patients with ACHD with PAH regarding: diagnostic evaluation,
prognosis, lifestyle choices, suitability for operative or catheter-based repair of existing shunts or
vascular obstructions contributing to PAH, nature and effectiveness of additional medical therapies,
mechanical circulatory and pulmonary vascular support, and goals of care.
2. PAH may develop years after shunt closure in patients with ACHD. Predictors for the development or
presence of PAH include:
a. Anatomic defects: complete AVSD, sinus venosus defect, large nonrestrictive defect (ASD >2 cm,
VSD >1 cm, PDA >0.6 cm), and concomitant ACHD AP classification II or III abnormalities.
b. Preintervention Qp:Qs ≥3 and/or PASP >40 mm Hg.
c. Presence of associated syndrome (e.g., Down syndrome).
d. Older age at repair.
e. Female sex.
f. Otherwise unexplained symptoms potentially attributable to PAH (decreased exercise capacity,
syncope, chest pain, hemoptysis).
g. Findings on clinical examination: systemic arterial desaturation, elevated systemic venous
pressures, other evidence of fluid retention, loud P2, new TR or PR, new arrhythmia, decreased
exercise capacity, electrocardiographic findings consistent with subpulmonary ventricular
hypertrophy or dilation. Echocardiography may demonstrate subpulmonic ventricular
dysfunction and/or enlargement and estimate central venous and PA pressures. However,
echocardiography alone is insufficient to accurately determine PA pressure or pulmonary
vascular resistance, so echocardiography is best used in conjunction with data obtained at
cardiac catheterization when making decisions about instituting or changing therapy for PAH
(S4.4.6.1-19–S4.4.6.1-21).
3. Cardiac catheterization remains the standard for accurate diagnosis of pulmonary hypertension
syndromes and for selection of optimal therapies for patients with ACHD with pulmonary hypertension.
4. Mechanical interventions targeting relief of anatomic contributors to PAH (e.g., closure of septal or
great arterial defects to eliminate shunting) may be considered as part of short-term plans of care for
patients with ACHD with PAH. However, even modest residual levels of PAH substantially determine
intermediate and longer-term outcomes. Patients should be followed for pulmonary hypertension.
5. Although history, noninvasive testing, and laboratory analysis (biochemistry and hematology) are all
part of the workup of pulmonary hypertension associated with CHD, cardiac catheterization with careful
hemodynamic measurements, with or without provocative maneuvers and/or angiography, remains
fundamental to accurate diagnosis and design of therapeutic plans.

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Table 33. Pulmonary Hypertension and Eisenmenger Syndrome: Routine Follow-Up and Testing
Intervals
and Testing
Physiological Stage C*
(mo)
Physiological Stage D*
(mo)
Outpatient ACHD cardiologist 6–12 3–6
ECG 12 12
TTE† 12 12
Pulse oximetry Each visit Each visit
CMR‡ As needed As needed
Exercise test§ 6–12 6–12
Cardiac catheterization║ As needed As needed
‡CMR may be indicated for assessment of right ventricular funcƟon and CHD anatomy not clariﬁed with TTE. Baseline study is
recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological
findings.
§6-minute walk test or CPET, depending on clinical indication.
║Cardiac catheterization should be performed at baseline and as needed.
ACHD indicates adult congenital heart disease; CMR, cardiovascular magnetic resonance; CPET, cardiopulmonary exercise test;
4.4.6.2. Eisenmenger Syndrome
Recommendations for Eisenmenger Syndrome
Diagnostic
I C-EO
1. When evaluating adults with presumed Eisenmenger syndrome,
clinicians should confirm diagnostic imaging and cardiac catheterization
data accuracy and exclude other potential contributors to right-to-left
shunting or pulmonary hypertension.
Therapeutic
I A
2. Bosentan is beneficial in symptomatic adults with Eisenmenger syndrome
with ASD or VSD (S4.4.6.2-1–S4.4.6.2-3).
IIa B-R
3. In symptomatic adults with Eisenmenger syndrome, bosentan and PDE-5
inhibitors are reasonable in combination if symptomatic improvement
does not occur with either medication alone (S4.4.6.2-1, S4.4.6.2-4–
S4.4.6.2-6).
IIa
C-EO 4. Bosentan is a reasonable therapy to treat symptomatic adults with
Eisenmenger syndrome with 1 of the following: shunts other than
ASD/VSD (e.g., PDA, aortopulmonary window) (Level of Evidence C-EO),
or complex congenital heart lesions (S4.4.6.2-1, S4.4.6.2-7) or Down
syndrome (S4.4.6.2-4, S4.4.6.2-5, S4.4.6.2-8–S4.4.6.2-10) (Level of
Evidence B-NR).
B-NR
IIa B-NR
5. It is reasonable to use PDE-5 inhibitors (e.g., sildenafil, tadalafil) to treat
symptomatic adults with Eisenmenger syndrome with ASD, VSD, or great
artery shunt (S4.4.6.2-1, S4.4.6.2-11–S4.4.6.2-16).

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Synopsis
Historically Eisenmenger syndrome has been understood as the most advanced form of PAH associated
with congenital intracardiac and great arterial shunting. The natural course and outcomes of PAH in
patients with ACHD with Eisenmenger syndrome, as contrasted to other adults with PAH, remain
incompletely defined. However, it is believed that better survival and functional ability of untreated
adults with Eisenmenger syndrome might be explained by sharing of loading conditions between right-
and left-sided cardiac chambers, as well as multiorgan system adaptations that develop over time.
The fundamental cause of Eisenmenger syndrome is elevated pulmonary vascular resistance
driving right-to-left intracardiac or great arterial shunting leading to systemic arterial desaturation. The
risk of development of Eisenmenger syndrome is influenced by concomitant congenital syndromes,
anatomic location of congenital defects, size of anatomic defects, genetic factors, and environmental
exposures.
Pathophysiological mechanisms contributing to development of Eisenmenger syndrome are not fully
understood. Suggested triggers and pathways include blood flow-induced shear and circumferential
stress, vasoconstriction, and vascular cell proliferation associated with fibrosis and thrombosis.
Cyanosis, erythrocytosis, abnormalities of loading conditions, and abnormalities of systemic and
pulmonary perfusion all contribute to functional incapacity and potential for multiorgan system
dysfunction and other sequelae, including stroke, brain abscess, osteoarthropathy, iron deficiency,
reduced glomerular clearance and susceptibility to acute renal insufficiency, nephrosis, pulmonary
arterial thrombosis and dissection, hemoptysis, pulmonary parenchymal infections, diastolic and systolic
cardiac dysfunction, arrhythmia, HF, and SCD.
Palliative therapies that may be helpful include supplemental oxygen if systemic arterial oxygen
saturation is empirically noted to rise in response, systemic anticoagulation, and avoidance of
circumstances recognized to contribute to risk (e.g., high altitude, pregnancy, exposure to high heat or
humidity leading to vasodilation, nephrotoxin exposure, extreme exertion, large shifts in intravascular
volume). However, supportive data for these strategies are limited or nonexistent. Systemic
anticoagulation has the potential for adverse as well as possible helpful effects.
Mechanical circulatory and pulmonary support, lung transplantation with concomitant repair of
anatomic cardiovascular defects, and heart–lung transplantation have all been applied in patients with
ACHD with Eisenmenger syndrome with deteriorating functional ability. Indications for such therapies
for adults with Eisenmenger syndrome are not standardized; comparative outcomes have not been
tested, and to date successes have been limited. However, pharmacological treatment of PAH is helpful
in the management of certain patients with Eisenmenger syndrome.
1. Right-to-left shunting through septal defects or connections between the great arteries associated
with subpulmonary ventricular hypertension may be diagnosed as Eisenmenger syndrome. PAH
medications may be beneficial for patients with Eisenmenger syndrome; however, other conditions may
cause right-to-left shunting for reasons other than shunt-related PAH and thus may require different
treatment options. These other conditions include: a) severe pulmonary hypertension of other cause
(e.g., thromboembolic disease, rheumatic disease), b) subpulmonary chamber outflow obstruction, c)
abnormalities of subpulmonary chamber compliance, and d) vascular streaming. Accurate diagnosis is

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necessary to guide therapy. For example, PAH therapies will not be beneficial if the source of right-to-
left shunting is RVOT obstruction; rather, alleviation of the RVOT obstruction is the necessary treatment.
Accurate diagnosis of Eisenmenger syndrome and exclusion of other potential contributors to right-to-
left shunting or pulmonary hypertension by means of advanced imaging and cardiac catheterization are
crucial prerequisites to optimize therapy for adults with Eisenmenger syndrome.
2. In adults with Eisenmenger syndrome associated with ASD or VSD in World Health Organization
functional class III or IV, RCTs demonstrate improved 6-minute walk distance, hemodynamics, and
subjective functional ability after 4 months of oral bosentan (S4.4.6.2-17). Longer-term benefit has been
demonstrated through open-label extension of this initial RCT (S4.4.6.2-1) and in single-center registry
cohorts (S4.4.6.2-1, S4.4.6.2-17). There may be a class effect for endothelin receptor antagonists, but
others have not been studied in this population.
3. A randomized crossover trial of combination PAH therapy (PDE-5 inhibitor therapy and endothelin
receptor antagonist therapy) enrolled adults with Eisenmenger syndrome or with idiopathic PAH and
demonstrated improvement in systemic arterial saturation but not in functional ability or
hemodynamics (S4.4.6.2-6). Use of combination PAH therapy for adults with Eisenmenger syndrome
was further supported by a single-center cohort series suggesting improvement in 6-minute walk testing
and hemodynamics in adults with Eisenmenger syndrome using combined PDE-5 inhibitory therapy and
endothelin receptor antagonist therapy (S4.4.6.2-1, S4.4.6.2-4, S4.4.6.2-18).
4. Open-label single-center registries and cohort studies of adults with Eisenmenger syndrome,
attributable to shunts other than ASD/VSD or with complex congenital heart lesions, suggest benefit in
functional capacity or hemodynamics after months of endothelin receptor antagonist therapy (S4.4.6.2-
1, S4.4.6.2-7). Patients with ACHD and Down syndrome have greater likelihood to develop pulmonary
hypertension, and they have unique comorbidities that influence the nature of their pulmonary
hypertension, the metrics used in follow-up, and the potential for benefit from as well as adverse
response to therapy. Open-label single-center registries and cohorts of adults with Down syndrome and
Eisenmenger syndrome suggest benefit in subjective and/or objective functional capacity after months
of endothelin receptor antagonist therapy, generally as contrasted to performance before institution of
endothelin receptor antagonist therapy (S4.4.6.2-8–S4.4.6.2-10). Accurate diagnosis of PAH and
Eisenmenger syndrome remains essential before initiating such therapy.
5. RCTs (S4.4.6.2-16) regarding PDE-5 inhibitor therapy for adults with Eisenmenger syndrome have
limitations, but are supported by multiple open-label prospective studies and information from a large
single-center retrospective registry (S4.4.6.2-1, S4.4.6.2-11–S4.4.6.2-16). These studies suggest benefit
in functional capacity and hemodynamics after use of either sildenafil or tadalafil at varying doses and
for varying periods of follow-up. Benefit was either in comparison to subjects’ performance before
institution of therapy or to other adults with similar Eisenmenger syndrome anatomy and physiology
who were not prescribed PDE-5 inhibitors.
4.4.7. Coronary Anomalies
Coronary abnormalities are among the most common congenital cardiovascular anomalies, surpassing in
prevalence nearly all others combined. Coronary anomalies include anomalous aortic origin of a
coronary artery (AAOCA), coronary fistula, and myocardial bridge. Many congenital coronary
abnormalities have a benign outcome. In contrast, natural history studies of anomalous coronary artery
from the PA (particularly anomalous left coronary artery from the PA) suggest poor outcome in
untreated patients; similar natural history studies are lacking regarding untreated patients with AAOCA,

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but other evidence raises concern. See Table 34 and Figure 5 for a diagnostic and treatment algorithm
for AAOCA.
Assessment of the risk of SCD in patients with AAOCA and of the role of AAOCA in causing
ischemia or symptoms is difficult because available data do not adequately capture the clinical spectrum
of these anomalies. Autopsy series are available that help describe the anomalies found in patients who
suffered SCD contrasted to other causes of death (S4.4.7-1–S4.4.7-5). There are surgical case series that
describe findings before operation, operative anatomy and postoperative course (S4.4.7-2, S4.4.7-5–
S4.4.7-8). There are imaging studies describing the anatomy and potential pathophysiological
abnormalities associated with AAOCA (S4.4.7-6, S4.4.7-9–S4.4.7-11). There are surgical series describing
improvement in symptoms after operation (S4.4.7-6–S4.4.7-8). There are surveys and registries that
describe the heterogeneous management strategies applied to AAOCA (S4.4.7-12–S4.4.7-14). What is
lacking are data proving that any particular management strategy prevents SCD. As a consequence,
decisions regarding whether surgery is necessary or exercise restriction or medical therapy might be
beneficial are all based on synthesizing limited data and applying to an individual patient. Clinicians
commonly extrapolate to assist in medical decision-making, but the consequences of being “wrong” for
a young patient with AAOCA may be perceived to be greater than for many other conditions.
Consequently, there is often a clinical urge to seek a reason to do something like surgical repair, because
the available data do not identify clinical features that provide reassurance that a patient is at low risk of
cardiovascular events. Unfortunately, evidence demonstrating that surgical repair ameliorates SCD risk,
derived from large enough cohorts followed over a sufficient period of time, is not available.
Table 34. Factors That May Relate to the Clinical Importance of AAOCA and Risk of SCD
Age AAOCA is more commonly invoked as the cause of SCD in patients <35 y of age than
in patients >35 y of age, in whom atherosclerotic coronary disease becomes a more
prevalent cause. However, death has been attributed to AAOCA in patients of all
ages; there does not seem to be an age beyond which the AAOCA may not be
relevant, even in the setting of atherosclerotic coronary disease and other
concomitant conditions (S4.4.7-1, S4.4.7-2).
Anatomy of coronary
ostium and proximal
coronary course
Slit-like/fish-mouth-shaped orifice, acute angle takeoff, intramural course,
interarterial course and hypoplasia of the proximal coronary artery have all been
proposed as reasons for symptoms, ischemia and SCD in patients with AAOCA. The
slit-like orifice is more commonly seen in anomalous right coronary artery arising
from the left sinus. Each of these anatomic findings offers a pathophysiological
mechanism for intermittent ischemia, particularly at times of high cardiac output
and/or increased aortic wall tension, such as during exercise (S4.4.7-6, S4.4.7-9–
S4.4.7-11).
Anomalous origin Left coronary artery arising from the right cusp is less common than the right
coronary artery arising from the left cusp but is more often found in autopsy series of
SCD (S4.4.7-1, S4.4.7-3, S4.4.7-15). This suggests that anomalous origin of the left
coronary artery from the right cusp is more likely to cause SCD than anomalous origin
of the right coronary artery from the left cusp. This may be due either to anatomic
features that make anomalous aortic origin of the left coronary artery prone to
coronary compromise or because a larger proportion of myocardium is supplied by
the left coronary artery, or both.
Exercise Autopsy series suggest a most patients die during, or in close temporal association
with, exercise (S4.4.7-3–S4.4.7-5).
Ischemia Autopsy series demonstrate myocardial fibrosis in a significant number of patients
whose deaths were attributed to AAOCA, particularly in patients with anomalous left
coronary artery arising from the right cusp (S4.4.7-5). Surgical series describe patients

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with ischemia or MI before surgical repair in the absence of other CAD, suggesting a
relation of the coronary anomaly to the ischemia (S4.4.7-16). This suggests that had
perfusion imaging been obtained before SCD, ischemia would have been found in
such patients (S4.4.7-17, S4.4.7-18). However, other data indicate that a normal
stress test does not preclude a SCD event, with the proviso that most of those studies
used only stress ECG, rather than the more sensitive and specific modalities of
nuclear perfusion imaging or stress echocardiography. In addition, postoperative
studies have shown that ischemia may be found after surgical repair in the
distribution not supplied by the abnormal coronary artery and may not persist on
repeat testing (S4.4.7-19).
Symptoms In autopsy and surgical series, a significant number of patients reported
cardiovascular symptoms, including before SCD events (S4.4.7-4, S4.4.7-7, S4.4.7-8,
S4.4.7-20, S4.4.7-21). Symptoms are more commonly reported in patients in whom
the left coronary artery arises from the right sinus. Surgical series have described
improvement in symptoms after surgical repair (S4.4.7-3–S4.4.7-8).
AAOCA indicates anomalous aortic origin of the coronary artery; CAD, coronary artery disease; ECG, electrocardiogram; MI,
myocardial infarction; and SCD, sudden cardiac death.
4.4.7.1. Anomalous Coronary Artery Evaluation
Recommendations for Anomalous Coronary Artery Evaluation
Diagnostic
I C-LD
1. Coronary angiography, using catheterization, CT, or CMR, is
recommended for evaluation of anomalous coronary artery (S4.4.7.1-1–
S4.4.7.1-3).
I C-LD
2. Anatomic and physiological evaluation should be performed in patients
with anomalous aortic origin of the left coronary from the right sinus
and/or right coronary from the left sinus (S4.4.7.1-4–S4.4.7.1-9).
1. CTA, CMR, and catheterization can all delineate the proximal course of the coronary artery and
relationship to other structures. CTA is generally preferred because it has superior spatial and temporal
resolution, although CMR may also provide adequate delineation of the relationship of the coronary
artery to the aorta, PA and other structures, including whether the proximal course appears to be
intramural. Coronary angiography by catheterization can be helpful when there is concern about
stenosis in the coronary artery or when concomitant hemodynamic evaluation for shunt assessment or
intravascular ultrasonography/flow evaluation is needed.
2. Assessment of AAOCA is enhanced when the precise anatomy and physiological impact of the
coronary artery anomaly are understood. As described in Table 34, the specific anomalous origin,
anatomy of the orifice and proximal vessel and presence of ischemia may all influence the clinical course
and thus the management options. Understanding these issues as precisely as possible will better
inform clinical decisions.

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Figure 5. Anomalous Aortic Origin of the Coronary Artery
*Surgical intervention to involve unroofing or coronary revascularization for patients with concomitant fixed obstruction.
4.4.7.2. Anomalous Aortic Origin of Coronary Artery
Recommendations for Anomalous Aortic Origin of Coronary Artery
Therapeutic
I B-NR
1. Surgery is recommended for AAOCA from the left sinus or AAOCA from
the right sinus for symptoms or diagnostic evidence consistent with
coronary ischemia attributable to the anomalous coronary artery
(S4.4.7.2-1–S4.4.7.2-3).
IIa C-LD
2. Surgery is reasonable for anomalous aortic origin of the left coronary
artery from the right sinus in the absence of symptoms or ischemia
(S4.4.7.2-4–S4.4.7.2-6).
IIa C-EO
3. Surgery for AAOCA is reasonable in the setting of ventricular
arrhythmias.
IIb B-NR 4. Surgery or continued observation may be reasonable for asymptomatic

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patients with an anomalous left coronary artery arising from the right
sinus or right coronary artery arising from the left sinus without ischemia
or anatomic or physiological evaluation suggesting potential for
compromise of coronary perfusion (e.g., intramural course, fish-mouth-
shaped orifice, acute angle) (S4.4.7.2-4–S4.4.7.2-6).
1. In patients with symptoms related to AAOCA, repair of the anomaly should alleviate symptoms. In
autopsy and surgical series, cardiac symptoms are more common in patients with a left coronary artery
arising from the right coronary cusp. In autopsy studies of patients who died because of an anomalous
coronary artery, fibrosis is a common finding, suggesting that ischemia preceded the terminal event.
However, there are patients in whom a SCD event occurred despite normal stress ECG, and
consequently absence of ischemia is not reassuring. Autopsy series show that many patients whose
death is attributed to anomalous coronary arteries are young, thus management of patients should take
age into account, with heightened concern about the risk of sudden death in younger patients (S4.4.7.2-
7–S4.4.7.2-9).
2. Anomalous left coronary from the right sinus is less common than anomalous right coronary from the
left sinus (S4.4.7.2-10), but anomalous left coronary artery from the right is more commonly found in
autopsy series of athletes and military recruits who had nontraumatic death than right coronary from
the left sinus (S4.4.7.2-1, S4.4.7.2-11–S4.4.7.2-13). The overrepresentation of the anomalous left
coronary from the right sinus suggests a higher risk of SCD, particular at extremes of exertion and in
patients <35 years of age.
There are some anatomic features that are thought to be associated with increased risk of
compromise of coronary flow and/or SCD, including a fish-mouth-shaped or slit-like orifice, or intramural
course (S4.4.7.2-14), although the slit-like orifice is more commonly encountered in a right coronary
arising from the left cusp. It is difficult to quantitate the absolute risk of SCD associated with anomalous
aortic origin of the left coronary from the right sinus, and data demonstrating that surgery ameliorates
the SCD risk have not been published. Until studies suggest otherwise, limited data and expert
consensus suggest that it is reasonable that adults with this malformation should undergo surgical
unroofing unless there are extenuating circumstances that would make surgery high risk.
3. In patients with ventricular arrhythmias presumed related to ischemia caused by anomalous origin of
a coronary artery, repair is an option to alleviate the ischemia and presumably mitigate the recurrence
of ventricular arrhythmias. However, care should be individualized, as there may be other factors (e.g.,
CAD, cardiomyopathy, residual ischemia) contributing to ventricular arrhythmias that warrant continued
vigilance and additional therapy.
4. Anomalous aortic origin of the right coronary from the left sinus is more common than anomalous
aortic origin of the left coronary from the right sinus. The risk of SCD with the former malformation is
difficult to quantitate. There is some physiological rationale to believe that asymptomatic patients
without evidence of compromised blood flow would benefit from unroofing, but there are not data to
demonstrate that surgical interventions alter the risk of SCD. Thus, watchful waiting may be an
appropriate course as well, particularly for a patient with an anomalous right coronary arising from the
left sinus.

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4.4.7.3. Anomalous Coronary Artery Arising From the PA
Recommendations for Anomalous Coronary Artery Arising From the PA
Therapeutic
I B-NR
1. Surgery is recommended for anomalous left coronary artery from the PA
(S4.4.7.3-1–S4.4.7.3-7).
I C-EO
2. In a symptomatic adult with anomalous right coronary artery from the
PA with symptoms attributed to the anomalous coronary, surgery is
recommended.
IIa C-EO
3. Surgery for anomalous right coronary artery from the PA is reasonable in
an asymptomatic adult with ventricular dysfunction or with myocardial
ischemia attributed to anomalous right coronary artery from the PA.
1. Surgery can include reimplantation of the left coronary artery directly into the aorta with or without
an interposition graft. Ligation or closure of the left coronary artery at the level of the PA with coronary
artery bypass grafting can also be performed, usually using the left internal mammary artery
anastomosed to the left anterior descending.
2. Surgery can include reimplantation of the right coronary artery directly into the aorta with or without
an interposition graft. Ligation or closure of the right coronary artery at the level of the PA with coronary
artery bypass grafting can also be performed, usually using the right internal mammary artery
anastomosed to the right coronary or posterior descending coronary artery.
3. Surgery to alleviate ischemia or ventricular dysfunction is reasonable if the anomalous coronary artery
is thought to be the cause. Surgery can include reimplantation of the right coronary artery directly into
the aorta with or without an interposition graft. Ligation or closure of the right coronary artery at the
level of the PA with coronary artery bypass grafting can also be performed, usually using the right
internal mammary artery anastomosed to the right coronary or posterior descending coronary artery.
4.4.8. Coronary Artery Fistula
Coronary artery fistula is an abnormal communication between a coronary artery and another
cardiovascular structure, which may include a cardiac chamber, coronary sinus, superior vena cava, or
PA. The incidence of coronary artery fistula is 0.1% to 0.2% in all patients undergoing coronary
angiography (S4.4.8-1, S4.4.8-2). Fistulous communications may be congenital or acquired. Specific
management strategies, which can include surgical repair or catheter embolization, have been
controversial. In a series of 46 patients treated with surgery, predominant preoperative symptoms
included angina and HF (S4.4.8-3). Importantly, postoperative myocardial infarction occurred in 11%
because of low flow in the dilated coronary artery proximal to fistula closure. Late survival was also
significantly reduced compared with an age-matched population. The presence of coronary artery
fistula(s) requires review by a knowledgeable team that may include congenital or noncongenital
cardiologists and surgeons to determine the role of medical therapy and/or percutaneous or surgical
closure (S4.4.8-3).

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5. Evidence Gaps and Future Directions
There are multiple challenges to developing evidenced-based care for patients with ACHD. The
heterogeneity of conditions leads to small numbers of specific ACHD populations from which to derive
guidelines. Additionally, lack of infrastructure to track prevalence, fragmented care systems, loss to
follow-up, and changes in treatment strategies over time all contribute to the challenges of developing
GDMT care (S5-1). Comprehensive multicenter and population registries and databases are needed to
have adequate numbers of patients to address clinical questions. Novel study methodologies are
needed to ascertain effectiveness of diagnostic and therapeutic options when each disease is sufficiently
rare and events occur over sufficiently long periods that RCTs are impractical. Although there are data
that patients with complex CHD have improved survival when cared for at an ACHD center, how can
networks of care be developed that ensure patients get the expert care needed when there are
inadequate number of ACHD cardiologists and ACHD centers? How do we ensure that patients are not
lost to care as they transition from pediatric to adult cardiology? How do we ensure that patients with
ACHD who would benefit from heart transplantation receive accurate listing priority? See Table 35 for a
collection of high-impact research questions in ACHD.
Table 35. High-Impact Research Questions in ACHD
General
Pathophysiology • What are the mechanisms of heart failure that can be prevented, reversed, or treated?
• Why does the systemic right ventricle fail?
• Will all patients with Fontan physiology develop clinically important cirrhosis, and how
can we prevent this?
• Who is at risk of aortic rupture and dilation?
• Are patients with manipulated coronary arteries (e.g., ASO, Ross repair) at risk of
premature coronary artery disease?
• What is the impact of radiation exposure on long-term health?
• Can we predict who will develop pulmonary hypertension/pulmonary vascular disease?
Medical and
surgical
treatment
• How can we modify current CHD surgical procedures to prevent or reduce later
development of heart failure and/or arrhythmias?
• Which patients with ACHD can use direct oral anticoagulants instead of warfarin?
• What is the best algorithm for contraception choices?
• Beyond those with severe PAH, which patients will benefit from PAH therapies?
• Do patients with ACHD with systemic right ventricles and HF benefit from standard
therapies (beta blockers, ACE inhibitors/ARBs, aldosterone antagonist)? Which one(s)?
• What medical therapies benefit patients with failing Fontan physiology?
• Do asymptomatic patients with ACHD with PAH benefit from PAH-specific therapy?
• Who will benefit from ventricular assist devices?
• What should be the threshold(s) for aortic aneurysm surgery?
• What pacing and resynchronization strategies are of most benefit, and when should
they be used?
Outcomes/risk
assessment
• What criteria should determine transplantation eligibility?
• Which patients benefit from primary prevention ICDs?
• How can we risk stratify for SCD in patients with systemic right ventricles?
• What operative risk score predicts outcomes in ACHD reoperations?
• What HF risk score predicts outcomes in patients with ACHD?
• Is there a level of exercise where risk exceeds benefit?
• What is the rate and/or risk of endocarditis?

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Assessment • Who is at high risk of neurodevelopmental abnormalities and would benefit from
neuropsychiatric evaluation and treatment?
• Who should be screened for anxiety and depression, what treatment is most effective,
and are there differences compared with non-patients with ACHD?
• What is the standard protocol for assessing right ventricular size and function by CMR
imaging?
• Which biomarkers are predictive of mortality and morbidity?
Disease-specific
Coarctation of
the aorta
• Which measure of hypertension—resting, exercise, or ambulatory—best predicts
outcomes?
• Is there an optimal antihypertensive regimen?
• What should blood pressure goals be?
• How often should patients be screened for thoracic aneurysm?
• Should exercise-induced hypertension be treated?
• What criteria warrant reintervention in recoarctation?
• Is long-term outcome better with medical therapy or catheter intervention for less than
severe recoarctation?
• Should patients be screened for intracranial aneurysm, and if so, how often?
Ebstein anomaly • What is the indication for surgery in the asymptomatic patient?
• Who should have a Glenn shunt at the time of tricuspid valve surgery?
• Should surgeons attempt tricuspid valve repair or routinely perform replacement in all
patients?
TOF • What is the optimal timing for pulmonary valve replacement in asymptomatic patients
with TOF?
• Do pulmonary valve replacement and ventricular tachycardia ablation decrease the risk
of SCD?
• Who needs a primary prevention ICD, and does this strategy reduce mortality?
• Is there a role for PAH therapies in TOF?
• Why does left ventricular dysfunction develop?
TGA/systemic
right ventricle
• Who benefits from ACE inhibitors/ARBs/beta blockers/spironolactone?
• Who needs a primary prevention ICD, and does this strategy prevent mortality?
• What imaging findings predict mortality/morbidity?
• In CCTGA with VSD/PS, does the double switch have better long-term outcomes than
VSD closure and left ventricle–to-PA conduit?
• When should tricuspid valve replacement be performed?
• What is the role of cardiac resynchronization therapy in patients with systemic right
ventricle?
ASO • What are the long-term outcomes after ASO?
• How should the possibility of asymptomatic coronary disease (ostial, compression) and
ischemia be assessed?
Single
ventricle/Fontan
• Is warfarin or aspirin beneficial in patients with a Fontan?
• Are PAH therapies beneficial?
• Is exercise capacity predictive of mortality?
• What liver screening is appropriate and at what intervals?
• How is protein-losing enteropathy best medically treated?
• Why do some patients fail with preserved ejection fraction, whereas other have
decreased ejection fraction?
• What are the long-term outcomes of hypoplastic left heart syndrome?
• What is ideal timing for heart transplantation in single ventricle Fontan patients, and
should liver issues prompt earlier transplantation than might be felt necessary from a
cardiac perspective?

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• Which has better long-term outcomes, the Fontan operation or bidirectional Glenn
alone?
Coronary
anomalies
• Does surgical intervention in anomalous aortic origin of coronary arteries improve
survival?
ACE indicates angiotensin-converting enzyme; ACHD, adult congenital heart disease; ARB, angiotensin-receptor blocker; ASO,
arterial switch operation; CCTGA, congenitally corrected transposition of the great arteries; CHD, congenital heart disease;
CMR, cardiac magnetic resonance; ICD, implantable cardioverter-defibrillator; HF, heart failure; PA, pulmonary artery; PAH,
pulmonary artery hypertension; PS, pulmonary stenosis; SCD, sudden cardiac death; TGA, transposition of the great arteries;
TOF, tetralogy of Fallot; and VSD, ventricular septal defect.
Presidents and Staff
American College of Cardiology
C. Michael Valentine, MD, FACC, President
Cathleen C. Gates, Interim Chief Executive Officer and Chief Operating Officer
William J. Oetgen, MD, MBA, FACC, Executive Vice President, Science, Education, Quality, and
Publications
MaryAnne Elma, MPH, Senior Director, Science, Education, Quality, and Publishing
Amelia Scholtz, PhD, Publications Manager, Science, Education, Quality, and Publishing
American College of Cardiology/American Heart Association
Katherine A. Sheehan, PhD, Director, Guideline Strategy and Operations
Abdul R. Abdullah, MD, Senior Manager, Guideline Science
American Heart Association
Ivor Benjamin, MD, FAHA, President
Nancy Brown, Chief Executive Officer
Rose Marie Robertson, MD, FAHA, Chief Science and Medical Officer
Gayle R. Whitman, PhD, RN, FAHA, FAAN, Senior Vice President, Office of Science Operations
Prashant Nedungadi, PhD, Science and Medicine Advisor, Office of Science Operations
Jody Hundley, Production and Operations Manager, Scientific Publications, Office of Science Operations
Key Words: ACC/AHA Clinical Practice Guidelines ■ arrhythmias ■ cardiac catheterization ■ cardiac
defects ■ congenital heart disease ■ congenital heart surgery ■ unoperated/repaired heart defect

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Appendix 1. Author Relationships With Industry and Other Entities (Relevant)—2018 AHA/ACC
Guideline for the Management of Adults With Congenital Heart Disease* (February 2018)
Committee
Member Employment Consultant
Speakers
Bureau
Ownership/
Partnership/
Principal
Personal
Research
Institutional,
Organizational, or
Other Financial
Benefit
Expert
Witness
Voting
Recusals by
Section†
Karen K. Stout
(Chair)
University of Washington—
Director, Adult Congenital Heart
Disease Program, Professor,
Internal Medicine and Pediatrics
None None None None None None None
Curt J. Daniels
(Vice Chair)
The Ohio State University Heart
Center and Nationwide Children’s
Hospital—Director, Adult
Congenital Heart Disease and
Pulmonary Hypertension
Program, Professor, Internal
Medicine and Pediatrics
None None None None • Actelion‡ None 4.1.1, 4.1.2,
4.1.3, 4.1.4,
4.1.5,
4.4.6.1,
4.4.6.2
Jamil A.
Aboulhosn
UCLA Adult Congenital Heart
Disease Center—Director
• Actelion
• GE Medical
• Edward
Lifescience
s§
• Medtronic
None None • Gore • United
Therapeutics
• Actelion
• Medtronic
• St. Jude
• Edward
Lifesciences
None 4.1.1, 4.1.2,
4.1.3, 4.1.4,
4.1.5, 4.2.6,
4.3.1.1,
4.3.1.2,
4.3.5,
4.4.6.1,
4.4.6.2
Biykem Bozkurt Baylor College of Medicine—
Professor of Medicine
None None None None • Novartis None None

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Craig S. Broberg Oregon Health and Science
University—Associate Professor
of Medicine
None None None None • Actelion None 4.1.1, 4.1.2,
4.1.3, 4.1.4,
4.1.5,
4.4.6.1,
4.4.6.2
Jack M. Colman University of Toronto—Professor
of Medicine and Obstetrics &
Gynecology, Toronto Congenital
Cardiac Centre for Adults and
Pregnancy and Heart Disease
Program; University Health
Network and Mount Sinai
Hospital—Senior Attending
Cardiologist
Stephen R.
Crumb
Boston Children’s Hospital—
Nurse Practitioner and
Coordinator, COACH and
Pulmonary Hypertension
Programs
Joseph A.
Dearani
Mayo Clinic—Professor of
Surgery and Chair, Division of
Cardiovascular Surgery
None None None None • Sorin
(LivaNova)§║
• Cormatrix║
None None
Stephanie
Fuller
University of Pennsylvania
Perelman School of Medicine—
Associate Professor of Clinical
Surgery
Michelle
Gurvitz
Harvard Medical School; Brigham
and Women’s Hospital—
Instructor of Pediatrics, Assistant
Professor

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Paul Khairy Montreal Heart Institute Adult
Congenital Center—Director;
None None None None • Boehringer
Ingelheim§
• St. Jude
Medical§
• Medtronic§
• Actelion§
None 4.1.1, 4.1.2,
4.1.3, 4.1.4,
4.1.5, 4.2.6,
4.3.1.1,
4.3.1.2,
4.3.5,
4.4.6.1,
4.4.6.2
Michael J.
Landzberg
Boston Children’s Hospital—
Director, Adult Congenital Heart
Service; Harvard Medical
School—Associate Professor of
Medicine
None None None None • Gilead
• Actelion
None 4.1.1, 4.1.2,
4.1.3, 4.1.4,
4.1.5,
4.4.6.1,
4.4.6.2
Arwa Saidi University of Florida College of
Medicine—Professor, Congenital
Heart Center
None None None None • Actelion§ None 4.1.1, 4.1.2,
4.1.3, 4.1.4,
4.1.5,
4.4.6.1,
4.4.6.2
Anne Marie
Valente
Boston Children’s Hospital,
Brigham and Women’s
Hospital—Outpatient Director,
Boston Adult Congenital Heart
Disease and Pulmonary
Hypertension Service; Harvard
Medical School—Associate
Professor of Medicine and
Pediatrics
George F. Van
Hare
Washington University School of
Medicine—Director, Pediatric
Cardiology; St. Louis Children’s,
Washington University Heart
Center—Co-Director

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This table represents the relationships of committee members with industry and other entities that were determined to be relevant to this document. These relationships were
reviewed and updated in conjunction with all meetings and/or conference calls of the writing committee during the document development process. The table does not
necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest represents ownership of
≥5% of the voting stock or share of the business entity, or ownership of ≥$5,000 of the fair market value of the business entity; or if funds received by the person from the
business entity exceed 5% of the person’s gross income for the previous year. Relationships that exist with no financial benefit are also included for the purpose of transparency.
Relationships in this table are modest unless otherwise noted.
According to the ACC/AHA, a person has a relevant relationship IF: a) the relationship or interest relates to the same or similar subject matter, intellectual property or asset,
topic, or issue addressed in the document; or b) the company/entity (with whom the relationship exists) makes a drug, drug class, or device addressed in the document or makes
a competing drug or device addressed in the document; or c) the person or a member of the person’s household, has a reasonable potential for financial, professional or other
personal gain or loss as a result of the issues/content addressed in the document.
*The ACHD Guideline began in March 2014. Over the initial years of the CMS Open Payment System, understandably, there have been issues related to accurate reporting of
food and beverage payments. For this reason, the ACC and AHA have not considered these minor charges relevant relationships with industry.
†Writing committee members are required to recuse themselves from voting on sections to which their specific relationships with industry and other entities may apply.
‡No financial benefit.
§Significant relationship.
║CMS reported payments to Dr. Dearani in 2016 related to research for the Sorin Group and Cormatrix; however, he disagrees with this report. The sections authored by Dr.
Dearani have been reviewed, and it was affirmed that there was no implication of any influence of industry.
ACC indicates American College of Cardiology; AHA, American Heart Association; CMS, Centers for Medicare & Medicaid Services; COACH, Columbus Ohio Adult Congenital
Heart; and UCLA, University of California, Los Angeles.

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Appendix 2. Reviewer Relationships With Industry and Other Entities (Comprehensive)—2018
AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease (February
2018)
Reviewer Representation Employment Consultant
Speakers
Bureau
Ownership/
Partnership/
Principal
Personal
Research
Institutional,
Organizational, or
Other Financial
Benefit
Expert
Witness
Samuel J.
Asirvatham
Official
Reviewer—AHA
Medicine and Pediatrics
• Abiomed
• AtriCure
• Biosense
Webster
• Biotronik
• Boston
Scientific*
• Medtronic
• Sanofi-aventis
• St. Jude
Medical
None None None None None
Wendy M.
Book
Official
Reviewer—AHA
Emory University—
Director of Emory Adult
Congenital Heart Center,
Department of Medicine
None None None None • Actelion • Defendant,
congenital
heart
disease,
2015
Samuel S.
Gidding
Official
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
Nemours Cardiac Center
DuPont Hospital for
Children—Chief, Division
of Pediatric Cardiology
None None None None None None

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Yuli Y. Kim Official
Reviewer—AHA
University of
Pennsylvania—Assistant
Professor of Medicine;
Children’s Hospital of
Philadelphia—Medical
Director, Philadelphia
Adult Congenital Heart
Center
Geetha
Raghuveer
Official
Reviewer—ACC
Board of
Governors
Children’s Mercy
Hospital— Pediatric
Cardiologist; University of
Missouri, Kansas City
School of Medicine—
Professor of Pediatrics
Carole A.
Warnes
Official
Reviewer—ACC
Board of Trustees
Medicine
Peter J. Bartz Organizational
Reviewer—ASE
Children’s Hospital of
Wisconsin—Associate
Professor, Medical College
of Wisconsin
Mitchell I.
Cohen
Organizational
Reviewer—HRS
Inova Fairfax Children’s
Hospital—Co-Director of
the Heart Center and
Chief, Pediatric Cardiology
Marshall L.
Jacobs
Organizational
Reviewer—AATS
Johns Hopkins School of
Medicine—Professor of
Surgery and Director,
Pediatric Heart Surgery
Outcomes Research
Larry A.
Latson
Organizational
Reviewer—SCAI
Joe DiMaggio Children’s
Hospital Heart Institute—
Medical Director, Pediatric
and Congenital
Interventional Cardiology
• Gore Medical None None None • St. Jude Medical None

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Constantine
Mavroudis
Organizational
Reviewer—STS
Florida Hospital for
Children—Medical
Director, Pediatric and
Congenital Heart Center
Doff B.
McElhinney
Organizational
Reviewer—SCAI
Stanford University—
Professor, Cardiothoracic
Surgery and of Pediatrics
• Medtronic* None None None None None
Erwin N.
Oechslin
Organizational
Reviewer—
ISACHD
University of Toronto—
Professor of Medicine;
Peter Munk Cardiac
Centre—Director, Adult
Program
• Actelion None None None None None
John K.
Triedman
Organizational
Reviewer—HRS
Boston Children’s
Hospital—Senior Associate
in Cardiology; Harvard
Medical School—Professor
of Pediatrics
• Biosense
Webster
Naser
Ammash
Content Reviewer Mayo Clinic—Professor of
Medicine
Helmut
Baumgartner
Content Reviewer University of Muenster—
Professor of Cardiology
and Adult Congenital Heart
Disease; University
Hospital Muenster—
Director, Division of Adult
Congenital and Valvular
Heart Disease, Department
of Cardiovascular Medicine
James C.
Blankenship
Content
Reviewer—ACC
Interventional
Section
Leadership
Council
Geisinger Medical
Center—Staff Physician
and Director, Cardiac
Catheterization Laboratory
None None None None • AbboƩ Vascular†
• Boston
ScienƟﬁc†
• GlaxoSmithKline†
• Takeda
PharmaceuƟcal†
None

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Ralph G.
Brindis
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
Northern California Kaiser
Permanente—Senior
Advisor, Cardiovascular
Disease; University of
California, San Francisco—
Clinical Professor of
Medicine
Robert M.
Campbell
Content Reviewer Emory University School of
Medicine, Sibley Heart
Center Cardiology—
Professor of Pediatrics
Lesley H.
Curtis
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
Duke University School of
Medicine
None None None None • Boston
Scientific*
• GE Healthcare*
• GlaxoSmithKline*
• Medtronic*
• Novartis*
None
Kristi K.
Fitzgerald
Content Reviewer Nemours Cardiac Center
DuPont Hospital for
Children—Genetic
Counselor, Division of
Pediatric Cardiology
Lee A.
Fleisher
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
University of Pennsylvania
Health System Department
of Anesthesiology and
Critical Care—Robert
Dunning Dripps Professor
of Anesthesiology
Federico
Gentile
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
Centro Cardiologico
Gentile—Director,
Cardiovascular Disease
Louise Harris Content Reviewer Toronto General
Hospital—Professor of
Medicine
• St. Jude
Medical

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Mark A.
Hlatky
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
Stanford University School
of Medicine—Professor of
Health Research and
Policy, and of
Cardiovascular Medicine
Craig T.
January
Content Reviewer University of Wisconsin
School of Medicine and
Public Health—Professor,
Division of Cardiovascular
Medicine
José A. Joglar Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
UT Southwestern Medical
Center—Professor of
Internal Medicine
Thomas K.
Jones
Content Reviewer Seattle Children’s
Hospital—Professor of
Pediatrics and Director,
Cardiac Catheterization
Laboratories
• Gore
Medical*
• Medtronic*
None None None • Gore Medical*
• Medtronic*
• St. Jude Medical*
None
Sana M. Al-
Khatib
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
Duke University Medical
Medicine
Brian E.
Kogon
Medicine—Associate
Professor, Surgery and
Surgical Director of Emory
Adult Congenital Heart
Center and Chief of
Pediatric Cardiac Surgery

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Gautam
Kumar
Content
Reviewer—ACC
Interventional
Section
Leadership
Council
Emory University School of
Medicine, Division of
Cardiology—Associate
• Abiomed None None None • OrbusNeich
Medical
None
Eric V.
Krieger
Content Reviewer University of
Washington—Associate
Associate Director, Adult
Congenital Heart Service
• Actelion None None None None None
Glenn N.
Levine
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
Baylor College of
Medicine; Michael E.
DeBakey VA Medical
Center—Director, Cardiac
Care Unit
C. Huie Lin Content
Reviewer—SCAI
Houston Methodist
DeBakey Heart & Vascular
Center—Cardiologist
• Gore Medical
• ACI Clinical
(DSMB)
• Abiomed None • St. Jude
Medical
None None
Massimo
Mancone
Content
Reviewer—ACC
AIG
Sapienza University of
Rome—Cardiology
Consultant
Ariane
Marelli
Content Reviewer McGill University Health
Medicine and Director,
MAUDE Unit
Koichiro
Niwa
Content Reviewer St. Luke’s International
Hospital—Director,
Department of Cardiology

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Matthew
Oster
Medicine—Associate
Professor; Children’s
Healthcare of Atlanta—
Director, Children’s Cardiac
Outcomes Research
Program at Sibley Heart
Center
Catherine M.
Otto
Content Reviewer University of Washington
School of Medicine—
Professor of Medicine,
Division of Cardiology and
Director, Heart Valve Clinic
Richard L.
Page
Content Reviewer University of Wisconsin
School of Medicine and
Public Health—Chair,
Department of Medicine
James Perry Content Reviewer Rady Children’s Hospital;
University of California,
San Diego—Professor of
Pediatrics, Affiliate
Professor of
Bioengineering, and
Director, Electrophysiology
and Adult Congenital Heart
Programs
Susan J.
Pressler
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
Indiana University School
of Nursing—Professor and
Director, Center for
Enhancing Quality of Life in
Chronic Illness
None None None None • Pﬁzer† None
Candice K.
Silversides
Content Reviewer University of Toronto—
Associate Professor

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Duminda N.
Wijeysundera
Content
Reviewer—
ACC/AHA Task
Force on Clinical
Practice
Guidelines
University of Toronto—
Assistant Professor,
Department of Anesthesia
and Institute of Health
Policy Management and
Evaluation
Ali N. Zaidi Content Reviewer Montefiore Einstein Center
for Heart and Vascular
Care—Director,
Montefiore Adult
Program; Albert Einstein
College of Medicine—
Associate Professor,
Internal Medicine and
Pediatrics
Elisa
Zaragoza-
Macias
Content Reviewer PeaceHealth North
Cascade Cardiology—
Cardiologist
This table represents the relationships of reviewers with industry and other entities that were disclosed at the time of peer review and determined to be relevant to this
document. It does not necessarily reflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest
represents ownership of ≥5% of the voting stock or share of the business entity, or ownership of ≥$5,000 of the fair market value of the business entity; or if funds received by
the person from the business entity exceed 5% of the person’s gross income for the previous year. A relationship is considered to be modest if it is less than significant under the
preceding definition. Relationships that exist with no financial benefit are also included for the purpose of transparency. Relationships in this table are modest unless otherwise
noted. Names are listed in alphabetical order within each category of review. Please refer to http://www.acc.org/guidelines/about-guidelines-and-clinical-
documents/relationships-with-industry-policy for definitions of disclosure categories or additional information about the ACC/AHA Disclosure Policy for Writing Committees.
*Significant relationship.
†No ﬁnancial beneﬁt.
AATS indicates American Association for Thoracic Surgery; ACC, American College of Cardiology; AHA, American Heart Association; AIG, Assembly of International Governors;
ASE, American Society of Echocardiography; DSMB, Data Safety Monitoring Board; HRS, Heart Rhythm Society; ISACHD, International Society for Adult Congenital Heart Disease;
MAUDE, McGill Adult Unit for Congenital Heart Disease Excellence; SCAI, Society for Cardiovascular Angiography and Interventions; and STS, Society of Thoracic Surgeons; and
UT, University of Texas.

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References
Preamble
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1.1. Methodology and Evidence Review
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1.4. Scope of the Guideline
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S1.4-5. van der Bom T, Bouma BJ, Meijboom FJ, et al. The prevalence of adult congenital heart disease,
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S1.4-7. Lien WP, Chen JJ, Chen JH, et al. Frequency of various congenital heart diseases in Chinese adults:
analysis of 926 consecutive patients over 13 years of age. Am J Cardiol. 1986;57:840-4.
S1.4-8. Samánek M, Slavík Z, Zborilová B, et al. Prevalence, treatment, and outcome of heart disease in live-
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S1.4-9. Mylotte D, Pilote L, Ionescu-Ittu R, et al. Specialized adult congenital heart disease care: the impact
of policy on mortality. Circulation. 2014;129:1804-12.
S1.4-10. Shen W-K, Sheldon RS, Benditt DG, et al. 2017 ACC/AHA/HRS guideline for the evaluation and
management of patients with syncope: a report of the American College of Cardiology/American
Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society.
Circulation. 2017;136:e60-122.
S1.4-11. Page RL, Joglar JA, Caldwell MA, et al. 2015 ACC/AHA/HRS guideline for the management of adult
patients with supraventricular tachycardia: a report of the American College of Cardiology/American
S1.4-12. Callaway CW, Donnino MW, Fink EL, et al. Part 8: post-cardiac arrest care: 2015 American Heart
Association guidelines update for cardiopulmonary resuscitation and emergency cardiovascular care.
Circulation. 2015;132:S465-82.
S1.4-13. Amsterdam EA, Wenger NK, Brindis RG, et al. 2014 AHA/ACC guideline for the management of
patients with non-ST-elevation acute coronary syndromes: a report of the American College of
Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation.
2014;130:e344-426.
S1.4-14. Fleisher LA, Fleischmann KE, Auerbach AD, et al. 2014 ACC/AHA guideline on perioperative
cardiovascular evaluation and management of patients undergoing noncardiac surgery: a report of
the American College of Cardiology/American Heart Association Task Force on Practice Guidelines.
Circulation. 2014;130:e278-333.
S1.4-15. January CT, Wann LS, Alpert JS, et al. 2014 AHA/ACC/HRS guideline for the management of patients
with atrial fibrillation: a report of the American College of Cardiology/American Heart Association
Task Force on Practice Guidelines and the Heart Rhythm Society. Circulation. 2014;130:e199-267.
S1.4-16. Fihn SD, Blankenship JC, Alexander KP, et al. 2014 ACC/AHA/AATS/PCNA/SCAI/STS focused update of
the guideline for the diagnosis and management of patients with stable ischemic heart disease: a
report of the American College of Cardiology/American Heart Association Task Force on Practice
Guidelines, and the American Association for Thoracic Surgery, Preventive Cardiovascular Nurses
Association, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic
Surgeons. Circulation. 2014;130:1749-67.

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S1.4-17. Fihn SD, Gardin JM, Abrams J, et al. 2012 ACCF/AHA/ACP/AATS/PCNA/SCAI/STS guideline for the
diagnosis and management of patients with stable ischemic heart disease: a report of the American
College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, and
the American College of Physicians, American Association for Thoracic Surgery, Preventive
Cardiovascular Nurses Association, Society for Cardiovascular Angiography and Interventions, and
Society of Thoracic Surgeons. Circulation. 2012;126:e354-471.
S1.4-18. Goff DC Jr, Lloyd-Jones DM, Bennett G, et al. 2013 ACC/AHA guideline on the assessment of
cardiovascular risk: a report of the American College of Cardiology/American Heart Association Task
Force on Practice Guidelines. Circulation. 2014;129:S49-73.
S1.4-19. Stone NJ, Robinson JG, Lichtenstein AH, et al. 2013 ACC/AHA guideline on the treatment of blood
cholesterol to reduce atherosclerotic cardiovascular risk in adults: a report of the American College
of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation.
2014;129:S1-45.
S1.4-20. Jensen MD, Ryan DH, Apovian CM, et al. 2013 AHA/ACC/TOS guideline for the management of
overweight and obesity in adults: a report of the American College of Cardiology/American Heart
Association Task Force on Practice Guidelines and The Obesity Society. Circulation. 2014;129:S102-
38.
S1.4-21. Eckel RH, Jakicic JM, Ard JD, et al. 2013 AHA/ACC guideline on lifestyle management to reduce
cardiovascular risk: a report of the American College of Cardiology/American Heart Association Task
Force on Practice Guidelines. Circulation. 2014;129:S76-99.
S1.4-22. Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC focused update of the 2014 AHA/ACC
guideline for the management of patients with valvular heart disease: a report of the American
College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.
Circulation. 2017;135:e1159-95.
S1.4-23. Whelton PK, Carey RM, Aronow WS, et al. 2017
ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/PCNA guideline for the prevention,
detection, evaluation, and management of high blood pressure in adults: a report of the American
Hypertension. 2018;71:e13-115.
S1.4-24. Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and ascending aorta guidelines for
management and quality measures. Ann Thorac Surg. 2013;95:S1-66.
S1.4-25. O’Gara PT, Kushner FG, Ascheim DD, et al. 2013 ACCF/AHA guideline for the management of ST-
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Foundation/American Heart Association Task Force on Practice Guidelines. Circulation.
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guideline for the management of heart failure: a report of the American College of
Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart
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for device-based therapy of cardiac rhythm abnormalities: a report of the American College of
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surgery: a report of the American College of Cardiology Foundation/American Heart Association Task
Force on Practice Guidelines. Circulation. 2011;124:e652-735.
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coronary intervention: a report of the American College of Cardiology Foundation/American Heart
Association Task Force on Practice Guidelines and the Society for Cardiovascular Angiography and
Interventions. Circulation. 2011;124:e574-651.
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congenital heart disease (new version 2010). Eur Heart J. 2010;31:2915-57.
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American College of Cardiology Foundation/American Heart Association Task Force on Practice
Guidelines, American Association for Thoracic Surgery, American College of Radiology, American
Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular
Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons,
and Society for Vascular Medicine. Circulation. 2010;121:e266-369.
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S1.4-36. Cohen MS, Eidem BW, Cetta F, et al. Multimodality imaging guidelines of patients with transposition
of the great arteries: a report from the American Society of Echocardiography. Developed in
collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of
Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 2016;29:571-621.
S1.4-37. Lang RM, Badano LP, Mor-Avi V, et al. Recommendations for cardiac chamber quantification by
echocardiography in adults: an update from the American Society of Echocardiography and the
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Heart Rhythm. 2014;11:e102-65.
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tetralogy of Fallot: a report from the American Society of Echocardiography. Developed in
collaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric
Radiology. J Am Soc Echocardiogr. 2014;27:111-41.
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the management of thoracic aortic disease. Can J Cardiol. 2014;30:577-89.
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with congenital heart disease: a scientific statement from the American Heart Association.
Circulation. 2013;127:2147-59.
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Heart Association. Circulation. 2012;126:1143-72.
S1.4-43. European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC),
German Society for Gender Medicine (DGesGM), et al. ESC guidelines on the management of
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97.
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with congenital heart disease: the transition process and medical and psychosocial issues: a scientific
statement from the American Heart Association. Circulation. 2011;123:1454-85.
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pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on

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Expert Consensus Documents and the American Heart Association. Developed in collaboration with
the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary
Hypertension Association. Circulation. 2009;119:2250-94.
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American Heart Association: a guideline from the American Heart Association Rheumatic Fever,
Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and
the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality
of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116:1736-54.
2. Background and Pathophysiology
2.1. Anatomic and Physiological Terms
S2.1-1. Giroud JM, Jacobs JP, Spicer D, et al. Report from the International Society for Nomenclature of
Paediatric and Congenital Heart Disease: creation of a visual encyclopedia illustrating the terms and
definitions of the international pediatric and congenital cardiac code. World J Pediatr Congenit Heart
Surg. 2010;1:300-13.
S2.1-2. Bergersen L, Giroud JM, Jacobs JP, et al. Report from The International Society for Nomenclature of
Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric
cardiac disease (Part 2 - Nomenclature of complications associated with interventional cardiology).
Cardiol Young. 2011;21:260-5.
S2.1-3. Bergersen L, Everett AD, Giroud JM, et al. Report from the International Society for Nomenclature of
Paediatric and Congenital Heart Disease: cardiovascular catheterisation for congenital and paediatric
cardiac disease (Part 1 - Procedural nomenclature). Cardiol Young. 2011;21:252-9.
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disease: historical perspectives and The International Pediatric and Congenital Cardiac Code. Cardiol
Young. 2008;18(suppl 2):70-80.
S2.1-5. Franklin RCG, Béland MJ, Krogmann ON. Mapping and coding of nomenclatures for paediatric and
congenital heart disease. Cardiol Young. 2006;16:105-6.
S2.1-6. International Society for Nomenclature of Paediatric and Congenital Heart Disease. International
Paediatric and Congenital Cardiac Code. Available at: http://ipccc.net/. Accessed: May 29, 2018.
2.2. Severity of ACHD
S2.2-1. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults
with congenital heart disease: a report of the American College of Cardiology/American Heart
Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the
Management of Adults With Congenital Heart Disease). Circulation. 2008;118:e714-833.
S2.2-3. Boodhwani M, Andelfinger G, Leipsic J, et al. Canadian Cardiovascular Society position statement on
the management of thoracic aortic disease. Can J Cardiol. 2014;30:577-89.
S2.2-4. Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and ascending aorta guidelines for
S2.2-5. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Mortality in adult congenital heart disease.
Eur Heart J. 2010;31:1220-9.
S2.2-6. Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia burden in adults with surgically repaired
tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122:868-75.
S2.2-7. Khairy P, Fernandes SM, Mayer JE Jr, et al. Long-term survival, modes of death, and predictors of
mortality in patients with Fontan surgery. Circulation. 2008;117:85-92.

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S2.2-8. Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot.
Circulation. 2008;117:363-70.
S2.2-9. Dimopoulos K, Diller G-P, Koltsida E, et al. Prevalence, predictors, and prognostic value of renal
dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320-8.
S2.2-10. Dimopoulos K, Diller G-P, Giannakoulas G, et al. Anemia in adults with congenital heart disease
relates to adverse outcome. J Am Coll Cardiol. 2009;54:2093-100.
S2.2-11. Dimopoulos K, Diller G-P, Petraco R, et al. Hyponatraemia: a strong predictor of mortality in adults
with congenital heart disease. Eur Heart J. 2010;31:595-601.
S2.2-12. Diller G-P, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease:
comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828-35.
S2.2-13. Buys R, Cornelissen V, Van De Bruaene A, et al. Measures of exercise capacity in adults with
congenital heart disease. Int J Cardiol. 2011;153:26-30.
S2.2-14. Gratz A, Hess J, Hager A. Self-estimated physical functioning poorly predicts actual exercise capacity
in adolescents and adults with congenital heart disease. Eur Heart J. 2009;30:497-504.
S2.2-15. Dimopoulos K, Okonko DO, Diller G-P, et al. Abnormal ventilatory response to exercise in adults with
congenital heart disease relates to cyanosis and predicts survival. Circulation. 2006;113:2796-802.
S2.2-16. Inuzuka R, Diller G-P, Borgia F, et al. Comprehensive use of cardiopulmonary exercise testing
identifies adults with congenital heart disease at increased mortality risk in the medium term.
Circulation. 2012;125:250-9.
S2.2-17. Müller J, Hager A, Diller G-P, et al. Peak oxygen uptake, ventilatory efficiency and QRS-duration
predict event free survival in patients late after surgical repair of tetralogy of Fallot. Int J Cardiol.
2015;196:158-64.
S2.2-18. Kempny A, Dimopoulos K, Uebing A, et al. Reference values for exercise limitations among adults
with congenital heart disease. Relation to activities of daily life—single centre experience and review
of published data. Eur Heart J. 2012;33:1386-96.
S2.2-19. Dolgin M, New York Heart Association, eds. Nomenclature and Criteria for Diagnosis of Diseases of
the Heart and Great Vessels. 9th ed. Boston, MA: Little, Brown and Co; 1994.
S2.2-20. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J
Am Coll Cardiol. 2013;62:D42-50.
2.3. The ACHD AP Classification
Eur Heart J. 2010;31:1220-9.
S2.3-4. Dimopoulos K, Okonko DO, Diller G-P, et al. Abnormal ventilatory response to exercise in adults with
congenital heart disease relates to cyanosis and predicts survival. Circulation. 2006;113:2796-802.
S2.3-5. Hebson CL, McCabe NM, Elder RW, et al. Hemodynamic phenotype of the failing Fontan in an adult
population. Am J Cardiol. 2013;112:1943-7.
S2.3-6. Khairy P, Aboulhosn J, Gurvitz MZ, et al. Arrhythmia burden in adults with surgically repaired
tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122:868-75.
S2.3-7. Khairy P, Harris L, Landzberg MJ, et al. Sudden death and defibrillators in transposition of the great
arteries with intra-atrial baffles: a multicenter study. Circ Arrhythm Electrophysiol. 2008;1:250-7.
S2.3-8. Inuzuka R, Diller G-P, Borgia F, et al. Comprehensive use of cardiopulmonary exercise testing
identifies adults with congenital heart disease at increased mortality risk in the medium term.
Circulation. 2012;125:250-9.
S2.3-9. Müller J, Hager A, Diller G-P, et al. Peak oxygen uptake, ventilatory efficiency and QRS-duration
predict event free survival in patients late after surgical repair of tetralogy of Fallot. Int J Cardiol.
2015;196:158-64.

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S2.3-10. Stefanescu A, Macklin EA, Lin E, et al. Usefulness of the Seattle Heart Failure Model to identify adults
with congenital heart disease at high risk of poor outcome. Am J Cardiol. 2014;113:865-70.
S2.3-11. Giannakoulas G, Dimopoulos K, Engel R, et al. Burden of coronary artery disease in adults with
congenital heart disease and its relation to congenital and traditional heart risk factors. Am J Cardiol.
2009;103:1445-50.
S2.3-12. Giannakoulas G, Dimopoulos K, Bolger AP, et al. Usefulness of natriuretic peptide levels to predict
mortality in adults with congenital heart disease. Am J Cardiol. 2010;105:869-73.
S2.3-13. Khairy P, Fernandes SM, Mayer JE Jr, et al. Long-term survival, modes of death, and predictors of
S2.3-14. Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot.
Circulation. 2008;117:363-70.
S2.3-15. Dimopoulos K, Diller G-P, Giannakoulas G, et al. Anemia in adults with congenital heart disease
relates to adverse outcome. J Am Coll Cardiol. 2009;54:2093-100.
S2.3-16. Dimopoulos K, Diller G-P, Petraco R, et al. Hyponatraemia: a strong predictor of mortality in adults
with congenital heart disease. Eur Heart J. 2010;31:595-601.
S2.3-17. Yancy CW, Jessup M, Bozkurt B, et al. 2013 ACCF/AHA guideline for the management of heart
failure: a report of the American College of Cardiology Foundation/American Heart Association Task
Force on Practice Guidelines. Circulation. 2013;128:e240-327.
3. General Principles
3.1. ACHD Program
3.2. Access to Care
S3.2-1. Gurvitz M, Valente AM, Broberg C, et al. Prevalence and predictors of gaps in care among adult
congenital heart disease patients: HEART-ACHD (The Health, Education, and Access Research Trial). J
Am Coll Cardiol. 2013;61:2180-4.
S3.2-2. Mackie AS, Islam S, Magill-Evans J, et al. Healthcare transition for youth with heart disease: a clinical
trial. Heart. 2014;100:1113-8.
S3.2-3. Yeung E, Kay J, Roosevelt GE, et al. Lapse of care as a predictor for morbidity in adults with
S3.2-4. Wacker A, Kaemmerer H, Hollweck R, et al. Outcome of operated and unoperated adults with
congenital cardiac disease lost to follow-up for more than five years. Am J Cardiol. 2005;95:776-9.
3.3. Delivery of Care
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heart disease: operation by pediatric heart surgeons decreases in-hospital death. Circulation.
2008;118:2345-52.
S3.3-3. Hijazi ZM, Ruiz CE, Zahn E, et al. SCAI/AATS/ACC/STS operator and institutional requirements for
transcatheter valve repair and replacement, part III: pulmonic valve. J Am Coll Cardiol.
2015;65:2556-63.

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S3.3-4. Ruiz CE, Feldman TE, Hijazi ZM, et al. Interventional fellowship in structural and congenital heart
disease for adults. JACC Cardiovasc Interv. 2010;3:e1-15.
S3.3-5. Armsby L, Beekman RHr, Benson L, et al. SCAI expert consensus statement for advanced training
programs in pediatric and congenital interventional cardiac catheterization. Catheter Cardiovasc
Interv. 2014;84:779-84.
S3.3-6. Mascio CE, Pasquali SK, Jacobs JP, et al. Outcomes in adult congenital heart surgery: analysis of the
Society of Thoracic Surgeons database. J Thorac Cardiovasc Surg. 2011;142:1090-7.
S3.3-7. Hörer J, Vogt M, Wottke M, et al. Evaluation of the Aristotle complexity models in adult patients
with congenital heart disease. Eur J Cardiothorac Surg. 2013;43:128-34.
S3.3-8. van Gameren M, Putman LM, Takkenberg JJM, et al. Risk stratification for adult congenital heart
surgery. Eur J Cardiothorac Surg. 2011;39:490-4.
S3.3-9. Kogon B, Oster M. Assessing surgical risk for adults with congenital heart disease: are pediatric
scoring systems appropriate? J Thorac Cardiovasc Surg. 2014;147:666-71.
S3.3-10. Gajjar TP, Hiremath CS, Desai NB. Surgical closure of sinus venosus atrial septal defect using a single
patch—transcaval repair technique. J Card Surg. 2011;26:429-34.
S3.3-11. Videbæk J, Laursen HB, Olsen M, et al. Long-term nationwide follow-up study of simple congenital
heart disease diagnosed in otherwise healthy children. Circulation. 2016;133:474-83.
S3.3-12. Karonis T, Scognamiglio G, Babu-Narayan SV, et al. Clinical course and potential complications of
small ventricular septal defects in adulthood: late development of left ventricular dysfunction
justifies lifelong care. Int J Cardiol. 2016;208:102-6.
S3.3-13. Bonello B, Kilner PJ. Review of the role of cardiovascular magnetic resonance in congenital heart
disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis. 2012;105:605-13.
S3.3-14. Kilner PJ. Imaging congenital heart disease in adults. Br J Radiol. 2011;84 Spec No 3:S258-68.
S3.3-15. Cohen MS, Eidem BW, Cetta F, et al. Multimodality imaging guidelines of patients with transposition
of the great arteries: a report from the American Society of Echocardiography. Developed in
collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of
Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 2016;29:571-621.
S3.3-16. Valente AM, Cook S, Festa P, et al. Multimodality imaging guidelines for patients with repaired
tetralogy of Fallot: a report from the American Society of Echocardiography. Developed in
collaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric
Radiology. J Am Soc Echocardiogr. 2014;27:111-41.
S3.3-17. Ginde S, Bartz PJ, Hill GD, et al. Restrictive lung disease is an independent predictor of exercise
intolerance in the adult with congenital heart disease. Congenit Heart Dis. 2013;8:246-54.
S3.3-18. Alonso-Gonzalez R, Borgia F, Diller G-P, et al. Abnormal lung function in adults with congenital heart
disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation.
2013;127:882-90.
S3.3-19. Zach KJ, Ramakrishna H, Chandrasekaran K, et al. Laparoscopic colectomy in an adult with single
ventricle physiology: anesthetic implications and management. Ann Card Anaesth. 2015;18:252-6.
S3.3-20. Duffels MGJ, Engelfriet PM, Berger RMF, et al. Pulmonary arterial hypertension in congenital heart
disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007;120:198-204.
S3.3-21. Lowe BS, Therrien J, Ionescu-Ittu R, et al. Diagnosis of pulmonary hypertension in the congenital
heart disease adult population impact on outcomes. J Am Coll Cardiol. 2011;58:538-46.
S3.3-22. Van De Bruaene A, Delcroix M, Pasquet A, et al. The importance of pulmonary artery pressures on
late atrial arrhythmia in transcatheter and surgically closed ASD type secundum. Int J Cardiol.
2011;152:192-5.
S3.3-23. Barst RJ, Ivy DD, Foreman AJ, et al. Four- and seven-year outcomes of patients with congenital heart
disease-associated pulmonary arterial hypertension (from the REVEAL Registry). Am J Cardiol.
2014;113:147-55.
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heart disease and Down’s syndrome. Int J Cardiol. 2013;164:323-6.
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heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007;93:682-7.

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S3.3-26. Manes A, Palazzini M, Leci E, et al. Current era survival of patients with pulmonary arterial
hypertension associated with congenital heart disease: a comparison between clinical subgroups.
Eur Heart J. 2014;35:716-24.
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S3.3-28. Van De Bruaene A, Moons P, Belmans A, et al. Predictive model for late atrial arrhythmia after
closure of an atrial septal defect. Int J Cardiol. 2013;164:318-22.
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hypertension after shunt closure. Int J Cardiol. 2013;168:3797-801.
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3.4. Evaluation of Suspected and Known CHD
3.4.1. Electrocardiogram
S3.4.1-1. Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected
transposition of the great arteries: a multi-institutional study. J Am Coll Cardiol. 2000;36:255-61.
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the adult: functional status and complications. J Am Coll Cardiol. 1996;27:1238-43.
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S3.4.1-4. Borkon AM, Pieroni DR, Varghese PJ, et al. The superior QRS axis in ostium primum ASD: a proposed
mechanism. Am Heart J. 1975;90:215-21.
S3.4.1-5. Wei W, Zhan X, Xue Y, et al. Features of accessory pathways in adult Ebstein’s anomaly. Europace.
2014;16:1619-25.
S3.4.1-6. Iturralde P, Nava S, Sálica G, et al. Electrocardiographic characteristics of patients with Ebstein’s
anomaly before and after ablation of an accessory atrioventricular pathway. J Cardiovasc
Electrophysiol. 2006;17:1332-6.
S3.4.1-7. Reich JD, Auld D, Hulse E, et al. The Pediatric Radiofrequency Ablation Registry’s experience with
Ebstein’s anomaly. Pediatric Electrophysiology Society. J Cardiovasc Electrophysiol. 1998;9:1370-7.
S3.4.1-8. Liberman L, Pass RH, Hordof AJ, et al. Late onset of heart block after open heart surgery for
congenital heart disease. Pediatr Cardiol. 2008;29:56-9.
3.4.2. Ionizing Radiation Principles
S3.4.2-1. Glatz AC, Purrington KS, Klinger A, et al. Cumulative exposure to medical radiation for children
requiring surgery for congenital heart disease. J Pediatr. 2014;164:789-94.e10.
S3.4.2-2. Johnson JN, Hornik CP, Li JS, et al. Cumulative radiation exposure and cancer risk estimation in
children with heart disease. Circulation. 2014;130:161-7.
S3.4.2-3. Yakoumakis E, Kostopoulou H, Makri T, et al. Estimation of radiation dose and risk to children
undergoing cardiac catheterization for the treatment of a congenital heart disease using Monte
Carlo simulations. Pediatr Radiol. 2013;43:339-46.
S3.4.2-4. Andreassi MG, Ait-Ali L, Botto N, et al. Cardiac catheterization and long-term chromosomal damage
in children with congenital heart disease. Eur Heart J. 2006;27:2703-8.
S3.4.2-5. Brenner DJ, Doll R, Goodhead DT, et al. Cancer risks attributable to low doses of ionizing radiation:
assessing what we really know. Proc Natl Acad Sci USA. 2003;100:13761-6.
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3.4.3. Echocardiography
S3.4.3-1. Randolph GR, Hagler DJ, Connolly HM, et al. Intraoperative transesophageal echocardiography
during surgery for congenital heart defects. J Thorac Cardiovasc Surg. 2002;124:1176-82.
3.4.4. CMR Imaging
S3.4.4-1. Puchalski MD, Williams RV, Askovich B, et al. Assessment of right ventricular size and function: echo
versus magnetic resonance imaging. Congenit Heart Dis. 2007;2:27-31.
S3.4.4-2. Blalock SE, Banka P, Geva T, et al. Interstudy variability in cardiac magnetic resonance imaging
measurements of ventricular volume, mass, and ejection fraction in repaired tetralogy of Fallot: a
prospective observational study. J Magn Reson Imaging. 2013;38:829-35.
S3.4.4-3. Crean AM, Maredia N, Ballard G, et al. 3D Echo systematically underestimates right ventricular
volumes compared to cardiovascular magnetic resonance in adult congenital heart disease patients
with moderate or severe RV dilatation. J Cardiovasc Magn Reson. 2011;13:78.
S3.4.4-4. Bonello B, Kilner PJ. Review of the role of cardiovascular magnetic resonance in congenital heart
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3.4.6. Cardiac Catheterization
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3.4.7. Exercise Testing
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S3.13.1-18. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital
heart disease: a literature review. J Am Coll Cardiol. 2007;49:2303-11.
S3.13.1-19. Pedersen LM, Pedersen TAL, Ravn HB, et al. Outcomes of pregnancy in women with tetralogy of
Fallot. Cardiol Young. 2008;18:423-9.
S3.13.1-20. Vriend JWJ, Drenthen W, Pieper PG, et al. Outcome of pregnancy in patients after repair of aortic
coarctation. Eur Heart J. 2005;26:2173-8.
S3.13.1-21. Kovacs AH, Harrison JL, Colman JM, et al. Pregnancy and contraception in congenital heart disease:
what women are not told. J Am Coll Cardiol. 2008;52:577-8.
S3.13.1-22. Beauchesne LM, Connolly HM, Ammash NM, et al. Coarctation of the aorta: outcome of pregnancy. J
Am Coll Cardiol. 2001;38:1728-33.
S3.13.1-23. Krieger EV, Landzberg MJ, Economy KE, et al. Comparison of risk of hypertensive complications of
pregnancy among women with versus without coarctation of the aorta. Am J Cardiol.
2011;107:1529-34.
S3.13.1-24. Balci A, Sollie-Szarynska KM, van der Bijl AGL, et al. Prospective validation and assessment of
cardiovascular and offspring risk models for pregnant women with congenital heart disease. Heart.
2014;100:1373-81.
S3.13.1-25. Nishimura RA, Otto CM, Bonow RO, et al. 2014 AHA/ACC guideline for the management of patients
S3.13.1-26. Cardiovascular disease and use of oral and injectable progestogen-only contraceptives and
combined injectable contraceptives. Results of an international, multicenter, case-control study.
World Health Organization Collaborative Study of Cardiovascular Disease and Steroid Hormone
Contraception. Contraception. 1998;57:315-24.
S3.13.1-27. Thorne S, MacGregor A, Nelson-Piercy C. Risks of contraception and pregnancy in heart disease.
Heart. 2006;92:1520-5.
S3.13.1-28. Lu C-W, Shih J-C, Chen S-Y, et al. Comparison of 3 risk estimation methods for predicting cardiac
outcomes in pregnant women with congenital heart disease. Circ J. 2015;79:1609-17.
S3.13.1-29. Kumar RK, Newburger JW, Gauvreau K, et al. Comparison of outcome when hypoplastic left heart
syndrome and transposition of the great arteries are diagnosed prenatally versus when diagnosis of
these two conditions is made only postnatally. Am J Cardiol. 1999;83:1649-53.
S3.13.1-30. Bonnet D, Coltri A, Butera G, et al. Detection of transposition of the great arteries in fetuses reduces
neonatal morbidity and mortality. Circulation. 1999;99:916-8.
3.13.2. Contraception
S3.13.2-1. Kovacs AH, Harrison JL, Colman JM, et al. Pregnancy and contraception in congenital heart disease:
what women are not told. J Am Coll Cardiol. 2008;52:577-8.
S3.13.2-2. Vigl M, Kaemmerer M, Seifert-Klauss V, et al. Contraception in women with congenital heart disease.
Am J Cardiol. 2010;106:1317-21.
S3.13.2-3. Kaemmerer M, Vigl M, Seifert-Klauss V, et al. Counseling reproductive health issues in women with
congenital heart disease. Clin Res Cardiol. 2012;101:901-7.
S3.13.2-4. Lidegaard Ø, Løkkegaard E, Svendsen AL, et al. Hormonal contraception and risk of venous
thromboembolism: national follow-up study. BMJ. 2009;339:b2890.
S3.13.2-5. Pijuan-Domènech A, Baró-Mariné F, Rojas-Torrijos M, et al. Usefulness of progesterone-only
components for contraception in patients with congenital heart disease. Am J Cardiol.
2013;112:590-3.
S3.13.2-6. Silversides CK, Sermer M, Siu SC. Choosing the best contraceptive method for the adult with
congenital heart disease. Curr Cardiol Rep. 2009;11:298-305.
3.13.3. Infertility Treatment
S3.13.3-1. Drenthen W, Pieper PG, van der Tuuk K, et al. Fertility, pregnancy and delivery in women after
biventricular repair for double outlet right ventricle. Cardiology. 2008;109:105-9.

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S3.13.3-2. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Pregnancy and delivery in women after Fontan
palliation. Heart. 2006;92:1290-4.
S3.13.3-3. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital
heart disease: a literature review. J Am Coll Cardiol. 2007;49:2303-11.
S3.13.3-4. Pedersen LM, Pedersen TAL, Ravn HB, et al. Outcomes of pregnancy in women with tetralogy of
Fallot. Cardiol Young. 2008;18:423-9.
S3.13.3-5. Vriend JWJ, Drenthen W, Pieper PG, et al. Outcome of pregnancy in patients after repair of aortic
coarctation. Eur Heart J. 2005;26:2173-8.
3.13.4. Sexual Function
S3.13.4-1. Winter MM, Reisma C, Kedde H, et al. Sexuality in adult patients with congenital heart disease and
their partners. Am J Cardiol. 2010;106:1163-8, e1-8.
S3.13.4-2. Moons P, Van Deyk K, Marquet K, et al. Sexual functioning and congenital heart disease: something
to worry about? Int J Cardiol. 2007;121:30-5.
S3.13.4-3. Cook SC, Arnott LM, Nicholson LM, et al. Erectile dysfunction in men with congenital heart disease.
Am J Cardiol. 2008;102:1728-30.
S3.13.4-4. Vigl M, Hager A, Bauer U, et al. Sexuality and subjective wellbeing in male patients with congenital
heart disease. Heart. 2009;95:1179-83.
S3.13.4-5. Opić P, Roos-Hesselink JW, Cuypers JAAC, et al. Sexual functioning is impaired in adults with
3.14. Heart Failure and Transplant
3.14.1. Heart Failure
S3.14.1-1. Verheugt CL, Uiterwaal CSPM, van der Velde ET, et al. Mortality in adult congenital heart disease.
Eur Heart J. 2010;31:1220-9.
S3.14.1-2. Zomer AC, Vaartjes I, van der Velde ET, et al. Heart failure admissions in adults with congenital heart
disease; risk factors and prognosis. Int J Cardiol. 2013;168:2487-93.
S3.14.1-3. Oechslin EN, Harrison DA, Connelly MS, et al. Mode of death in adults with congenital heart disease.
Am J Cardiol. 2000;86:1111-6.
S3.14.1-4. Zomer AC, Vaartjes I, Uiterwaal CSPM, et al. Circumstances of death in adult congenital heart
disease. Int J Cardiol. 2012;154:168-72.
S3.14.1-5. Stout K, Valente AM, Bartz PJ, et al. Task Force 6: pediatric cardiology fellowship training in adult
congenital heart disease. Circulation. 2015;132:e91-8.
S3.14.1-6. Norozi K, Bahlmann J, Raab B, et al. A prospective, randomized, double-blind, placebo controlled
trial of beta-blockade in patients who have undergone surgical correction of tetralogy of Fallot.
S3.14.1-7. Babu-Narayan SV, Uebing A, Davlouros PA, et al. Randomised trial of ramipril in repaired tetralogy of
Fallot and pulmonary regurgitation: the APPROPRIATE study (Ace inhibitors for Potential PRevention
Of the deleterious effects of Pulmonary Regurgitation In Adults with repaired TEtralogy of Fallot). Int
J Cardiol. 2012;154:299-305.
S3.14.1-8. Lester SJ, McElhinney DB, Viloria E, et al. Effects of losartan in patients with a systemically
functioning morphologic right ventricle after atrial repair of transposition of the great arteries. Am J
Cardiol. 2001;88:1314-6.
S3.14.1-9. Hechter SJ, Fredriksen PM, Liu P, et al. Angiotensin-converting enzyme inhibitors in adults after the
Mustard procedure. Am J Cardiol. 2001;87:660-3, A11.
S3.14.1-10. Dore A, Houde C, Chan K-L, et al. Angiotensin receptor blockade and exercise capacity in adults with
systemic right ventricles: a multicenter, randomized, placebo-controlled clinical trial. Circulation.
2005;112:2411-6.

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S3.14.1-11. Giardini A, Lovato L, Donti A, et al. A pilot study on the effects of carvedilol on right ventricular
remodelling and exercise tolerance in patients with systemic right ventricle. Int J Cardiol.
2007;114:241-6.
S3.14.1-12. Doughan ARK, McConnell ME, Book WM. Effect of beta blockers (carvedilol or metoprolol XL) in
patients with transposition of great arteries and dysfunction of the systemic right ventricle. Am J
Cardiol. 2007;99:704-6.
S3.14.1-13. Therrien J, Provost Y, Harrison J, et al. Effect of angiotensin receptor blockade on systemic right
ventricular function and size: a small, randomized, placebo-controlled study. Int J Cardiol.
2008;129:187-92.
S3.14.1-14. Bouallal R, Godart F, Francart C, et al. Interest of β-blockers in patients with right ventricular
systemic dysfunction. Cardiol Young. 2010;20:615-9.
S3.14.1-15. Tutarel O, Meyer GP, Bertram H, et al. Safety and efficiency of chronic ACE inhibition in symptomatic
heart failure patients with a systemic right ventricle. Int J Cardiol. 2012;154:14-6.
S3.14.1-16. van der Bom T, Winter MM, Bouma BJ, et al. Effect of valsartan on systemic right ventricular
function: a double-blind, randomized, placebo-controlled pilot trial. Circulation. 2013;127:322-30.
S3.14.1-17. Dos L, Pujadas S, Estruch M, et al. Eplerenone in systemic right ventricle: double blind randomized
clinical trial. The EVEDES Study. Int J Cardiol. 2013;168:5167-73.
S3.14.1-18. Kouatli AA, García JA, Zellers TM, et al. Enalapril does not enhance exercise capacity in patients after
Fontan procedure. Circulation. 1997;96:1507-12.
S3.14.1-19. Giardini A, Balducci A, Specchia S, et al. Effect of sildenafil on haemodynamic response to exercise
and exercise capacity in Fontan patients. Eur Heart J. 2008;29:1681-7.
S3.14.1-20. Goldberg DJ, French B, McBride MG, et al. Impact of oral sildenafil on exercise performance in
children and young adults after the Fontan operation: a randomized, double-blind, placebo-
controlled, crossover trial. Circulation. 2011;123:1185-93.
S3.14.1-21. Rhodes J, Ubeda-Tikkanen A, Clair M, et al. Effect of inhaled iloprost on the exercise function of
Fontan patients: a demonstration of concept. Int J Cardiol. 2013;168:2435-40.
S3.14.1-22. Galiè N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome:
a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006;114:48-54.
3.14.2. Heart Transplant
S3.14.2-1. Everitt MD, Donaldson AE, Stehlik J, et al. Would access to device therapies improve transplant
outcomes for adults with congenital heart disease? Analysis of the United Network for Organ
Sharing (UNOS). J Heart Lung Transplant. 2011;30:395-401.
S3.14.2-2. Davies RR, Russo MJ, Yang J, et al. Listing and transplanting adults with congenital heart disease.
Circulation. 2011;123:759-67.
S3.14.2-3. Karamlou T, Hirsch J, Welke K, et al. A United Network for Organ Sharing analysis of heart
transplantation in adults with congenital heart disease: outcomes and factors associated with
mortality and retransplantation. J Thorac Cardiovasc Surg. 2010;140:161-8.
S3.14.2-4. Stout KK, Broberg CS, Book WM, et al. Chronic heart failure in congenital heart disease: a scientific
S3.14.2-5. Gelow JM, Song HK, Weiss JB, et al. Organ allocation in adults with congenital heart disease listed for
heart transplant: impact of ventricular assist devices. J Heart Lung Transplant. 2013;32:1059-64.
S3.14.2-6. Goerler H, Simon A, Gohrbandt B, et al. Heart-lung and lung transplantation in grown-up congenital
heart disease: long-term single centre experience. Eur J Cardiothorac Surg. 2007;32:926-31.
S3.14.2-7. Burchill LJ, Edwards LB, Dipchand AI, et al. Impact of adult congenital heart disease on survival and
mortality after heart transplantation. J Heart Lung Transplant. 2014;33:1157-63.
S3.14.2-8. Greutmann M, Prêtre R, Furrer L, et al. Heart transplantation in adolescent and adult patients with
congenital heart disease: a case-control study. Transplant Proc. 2009;41:3821-6.
S3.14.2-9. Seddio F, Gorislavets N, Iacovoni A, et al. Is heart transplantation for complex congenital heart
disease a good option? A 25-year single centre experience. Eur J Cardiothorac Surg. 2013;43:605-11.
S3.14.2-10. Alshawabkeh LI, Hu N, Carter KD, et al. Wait-list outcomes for adults with congenital heart disease
listed for heart transplantation in the U.S. J Am Coll Cardiol. 2016;68:908-17.

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S3.14.2-11. Krishnamurthy Y, Cooper LB, Lu D, et al. Trends and outcomes of patients with adult congenital heart
disease and pulmonary hypertension listed for orthotopic heart transplantation in the United States.
J Heart Lung Transplant. 2016;35:619-24.
S3.14.2-12. Harper AR, Crossland DS, Perri G, et al. Is alternative cardiac surgery an option in adults with
congenital heart disease referred for thoracic organ transplantation? Eur J Cardiothorac Surg.
2013;43:344-51.
3.14.3. Multiorgan Transplant
S3.14.3-1. Toyoda Y, Toyoda Y. Heart-lung transplantation: adult indications and outcomes. J Thorac Dis.
2014;6:1138-42.
S3.14.3-2. Yusen RD, Edwards LB, Dipchand AI, et al. The Registry of the International Society for Heart and
Lung Transplantation: thirty-third adult lung and heart-lung transplant report—2016; focus theme:
primary diagnostic indications for transplant. J Heart Lung Transplant. 2016;35:1170-84.
S3.14.3-3. Yusen RD, Edwards LB, Kucheryavaya AY, et al. The registry of the International Society for Heart and
Lung Transplantation: thirty-first adult lung and heart-lung transplant report—2014; focus theme:
retransplantation. J Heart Lung Transplant. 2014;33:1009-24.
S3.14.3-4. Bolman RMr, Shumway SJ, Estrin JA, et al. Lung and heart-lung transplantation. Evolution and new
applications. Ann Surg. 1991;214:456-68.
S3.14.3-5. Atluri P, Gaffey A, Howard J, et al. Combined heart and liver transplantation can be safely performed
with excellent short- and long-term results. Ann Thorac Surg. 2014;98:858-62.
S3.14.3-6. Cannon RM, Hughes MG, Jones CM, et al. A review of the United States experience with combined
heart-liver transplantation. Transpl Int. 2012;25:1223-8.
S3.14.3-7. Barbara DW, Rehfeldt KH, Heimbach JK, et al. The perioperative management of patients undergoing
combined heart-liver transplantation. Transplantation. 2015;99:139-44.
S3.14.3-8. Simpson KE, Esmaeeli A, Khanna G, et al. Liver cirrhosis in Fontan patients does not affect 1-year
post-heart transplant mortality or markers of liver function. J Heart Lung Transplant. 2014;33:170-7.
3.15. Palliative Care
S3.15-1. Greutmann M, Tobler D, Colman JM, et al. Facilitators of and barriers to advance care planning in
adult congenital heart disease. Congenit Heart Dis. 2013;8:281-8.
S3.15-2. Tobler D, Greutmann M, Colman JM, et al. Knowledge of and preference for advance care planning
by adults with congenital heart disease. Am J Cardiol. 2012;109:1797-800.
S3.15-3. Tobler D, Greutmann M, Colman JM, et al. End-of-life in adults with congenital heart disease: a call
for early communication. Int J Cardiol. 2012;155:383-7.
S3.15-4. Tobler D, Greutmann M, Colman JM, et al. End-of-life care in hospitalized adults with complex
congenital heart disease: care delayed, care denied. Palliat Med. 2012;26:72-9.
3.16. Cyanosis
S3.16-1. Martin L, Khalil H. How much reduced hemoglobin is necessary to generate central cyanosis? Chest.
1990;97:182-5.
S3.16-2. Broberg CS, Jayaweera AR, Diller GP, et al. Seeking optimal relation between oxygen saturation and
hemoglobin concentration in adults with cyanosis from congenital heart disease. Am J Cardiol.
2011;107:595-9.
S3.16-3. Broberg CS, Bax BE, Okonko DO, et al. Blood viscosity and its relationship to iron deficiency,
symptoms, and exercise capacity in adults with cyanotic congenital heart disease. J Am Coll Cardiol.
2006;48:356-65.
S3.16-4. Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart
disease. J Am Coll Cardiol. 1996;28:768-72.
S3.16-5. Chang Y-L, Hung S-H, Ling W, et al. Correction: association between ischemic stroke and iron-
deficiency anemia: a population-based study. PLoS One. 2017;12:e0170872.

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S3.16-6. Perloff JK, Marelli AJ, Miner PD. Risk of stroke in adults with cyanotic congenital heart disease.
Circulation. 1993;87:1954-9.
S3.16-7. Kaemmerer H, Fratz S, Braun SL, et al. Erythrocyte indexes, iron metabolism, and
hyperhomocysteinemia in adults with cyanotic congenital cardiac disease. Am J Cardiol.
2004;94:825-8.
S3.16-8. Tay ELW, Peset A, Papaphylactou M, et al. Replacement therapy for iron deficiency improves
exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the
Eisenmenger syndrome. Int J Cardiol. 2011;151:307-12.
S3.16-9. Broberg CS. Challenges and management issues in adults with cyanotic congenital heart disease.
Heart. 2016;102:720-5.
S3.16-10. Oechslin E. Management of adults with cyanotic congenital heart disease. Heart. 2015;101:485-94.
S3.16-11. Kajimoto H, Nakazawa M, Murasaki K, et al. Increased thrombogenesity in patients with cyanotic
congenital heart disease. Circ J. 2007;71:948-53.
S3.16-12. Jensen AS, Johansson PI, Idorn L, et al. The haematocrit—an important factor causing impaired
haemostasis in patients with cyanotic congenital heart disease. Int J Cardiol. 2013;167:1317-21.
S3.16-13. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. I. Br
Med J. 1958;2:701-9.
S3.16-14. Broberg C, Ujita M, Babu-Narayan S, et al. Massive pulmonary artery thrombosis with haemoptysis
in adults with Eisenmenger’s syndrome: a clinical dilemma. Heart. 2004;90:e63.
S3.16-15. Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration
and death. Eur Heart J. 1998;19:1845-55.
S3.16-16. Sandoval J, Santos LE, Córdova J, et al. Does anticoagulation in Eisenmenger syndrome impact long-
term survival? Congenit Heart Dis. 2012;7:268-76.
S3.16-17. Dedkov EI, Perloff JK, Tomanek RJ, et al. The coronary microcirculation in cyanotic congenital heart
S3.16-18. Duffels MGJ, Mulder KM, Trip MD, et al. Atherosclerosis in patients with cyanotic congenital heart
disease. Circ J. 2010;74:1436-41.
S3.16-19. Horigome H, Hiramatsu Y, Shigeta O, et al. Overproduction of platelet microparticles in cyanotic
congenital heart disease with polycythemia. J Am Coll Cardiol. 2002;39:1072-7.
S3.16-20. Tsui I, Shamsa K, Perloff JK, et al. Retinal vascular patterns in adults with cyanotic congenital heart
disease. Semin Ophthalmol. 2009;24:262-5.
S3.16-22. Broberg CS, Van Woerkom RC, Swallow E, et al. Lung function and gas exchange in Eisenmenger
syndrome and their impact on exercise capacity and survival. Int J Cardiol. 2014;171:73-7.
S3.16-23. Broberg CS, Uebing A, Cuomo L, et al. Adult patients with Eisenmenger syndrome report flying safely
on commercial airlines. Heart. 2007;93:1599-603.
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Saunders/Elsevier; 2009.
3.17. Pharmacological Therapy for ACHD
S3.17-1. Yancy CW, Jessup M, Bozkurt B, et al. 2017 ACC/AHA/HFSA focused update of the 2013 ACCF/AHA
guideline for the management of heart failure: a report of the American College of
Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart
Failure Society of America. Circulation. 2017;136:e137-61.
S3.17-2. Doughan ARK, McConnell ME, Book WM. Effect of beta blockers (carvedilol or metoprolol XL) in
Cardiol. 2007;99:704-6.
S3.17-3. Dos L, Pujadas S, Estruch M, et al. Eplerenone in systemic right ventricle: double blind randomized
S3.17-4. van der Bom T, Winter MM, Bouma BJ, et al. Effect of valsartan on systemic right ventricular

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S3.17-5. Dore A, Houde C, Chan K-L, et al. Angiotensin receptor blockade and exercise capacity in adults with
2005;112:2411-6.
S3.17-6. Babu-Narayan SV, Uebing A, Davlouros PA, et al. Randomised trial of ramipril in repaired tetralogy of
Fallot and pulmonary regurgitation: the APPROPRIATE study (Ace inhibitors for Potential PRevention
Of the deleterious effects of Pulmonary Regurgitation In Adults with repaired TEtralogy of Fallot). Int
J Cardiol. 2012;154:299-305.
S3.17-7. Lester SJ, McElhinney DB, Viloria E, et al. Effects of losartan in patients with a systemically
functioning morphologic right ventricle after atrial repair of transposition of the great arteries. Am J
Cardiol. 2001;88:1314-6.
S3.17-8. Hechter SJ, Fredriksen PM, Liu P, et al. Angiotensin-converting enzyme inhibitors in adults after the
Mustard procedure. Am J Cardiol. 2001;87:660-3, A11.
S3.17-9. Therrien J, Provost Y, Harrison J, et al. Effect of angiotensin receptor blockade on systemic right
2008;129:187-92.
S3.17-10. Galiè N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome:
S3.17-11. Giardini A, Balducci A, Specchia S, et al. Effect of sildenafil on haemodynamic response to exercise
S3.17-12. Hebert A, Mikkelsen UR, Thilen U, et al. Bosentan improves exercise capacity in adolescents and
adults after Fontan operation: the TEMPO (Treatment With Endothelin Receptor Antagonist in
Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen
Consumption) study. Circulation. 2014;130:2021-30.
S3.17-13. Goldberg DJ, French B, McBride MG, et al. Impact of oral sildenafil on exercise performance in
4. Specific Lesions
4.1. Shunt Lesions
4.1.1. Atrial Septal Defect
S4.1.1-1. Oster M, Bhatt A, Zaragoza-Macias E, et al. Interventional therapy versus medical therapy for
secundum atrial septal defect: a systematic review (part 2) for the 2018 AHA/ACC guideline for the
management of adults with congenital heart disease: a report of the American College of
Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2018;
In press.
S4.1.1-2. Ammash NM, Seward JB, Warnes CA, et al. Partial anomalous pulmonary venous connection:
diagnosis by transesophageal echocardiography. J Am Coll Cardiol. 1997;29:1351-8.
S4.1.1-3. Haramati LB, Moche IE, Rivera VT, et al. Computed tomography of partial anomalous pulmonary
venous connection in adults. J Comput Assist Tomogr. 2003;27:743-9.
S4.1.1-4. Nordmeyer S, Berger F, Kuehne T, et al. Flow-sensitive four-dimensional magnetic resonance
imaging facilitates and improves the accurate diagnosis of partial anomalous pulmonary venous
drainage. Cardiol Young. 2011;21:528-35.
S4.1.1-5. Kardon RE, Sokoloski MC, Levi DS, et al. Transthoracic echocardiographic guidance of transcatheter
atrial septal defect closure. Am J Cardiol. 2004;94:256-60.
S4.1.1-6. Zanchetta M, Rigatelli G, Pedon L, et al. Transcatheter atrial septal defect closure assisted by
intracardiac echocardiography: 3-year follow-up. J Interv Cardiol. 2004;17:95-8.
S4.1.1-7. Attie F, Rosas M, Granados N, et al. Surgical treatment for secundum atrial septal defects in patients
>40 years old. A randomized clinical trial. J Am Coll Cardiol. 2001;38:2035-42.

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S4.1.1-8. Brochu M-C, Baril J-F, Dore A, et al. Improvement in exercise capacity in asymptomatic and mildly
symptomatic adults after atrial septal defect percutaneous closure. Circulation. 2002;106:1821-6.
S4.1.1-9. Shah D, Azhar M, Oakley CM, et al. Natural history of secundum atrial septal defect in adults after
medical or surgical treatment: a historical prospective study. Br Heart J. 1994;71:224-7.
S4.1.1-10. Roos-Hesselink JW, Meijboom FJ, Spitaels SEC, et al. Excellent survival and low incidence of
arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age. A
prospective follow-up study of 21-33 years. Eur Heart J. 2003;24:190-7.
S4.1.1-11. Altındag T, Roos-Hesselink JW, Cuypers JA, et al. Transcatheter device closure of atrial septal defects
in patients aged 40 years and older. Neth Heart J. 2010;18:537-42.
S4.1.1-12. Varma C, Benson LN, Silversides C, et al. Outcomes and alternative techniques for device closure of
the large secundum atrial septal defect. Catheter Cardiovasc Interv. 2004;61:131-9.
S4.1.1-13. Du Z-D, Cao Q-L, Rhodes J, et al. Choice of device size and results of transcatheter closure of atrial
septal defect using the amplatzer septal occluder. J Interv Cardiol. 2002;15:287-92.
S4.1.1-14. Gajjar TP, Hiremath CS, Desai NB. Surgical closure of sinus venosus atrial septal defect using a single
patch—transcaval repair technique. J Card Surg. 2011;26:429-34.
S4.1.1-15. Du Z-D, Hijazi ZM, Kleinman CS, et al. Comparison between transcatheter and surgical closure of
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4.4. Complex Lesions
4.4.1. Transposition of the Great Arteries
4.4.1.1. Transposition of the Great Arteries With Atrial Switch
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4.4.1.2. Transposition of the Great Arteries With Arterial Switch
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4.4.7. Coronary Anomalies
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4.4.7.1. Anomalous Coronary Artery Evaluation
S4.4.7.1-1. Basso C, Maron BJ, Corrado D, et al. Clinical profile of congenital coronary artery anomalies with
Cardiol. 2000;35:1493-501.
S4.4.7.1-2. Davies JE, Burkhart HM, Dearani JA, et al. Surgical management of anomalous aortic origin of a
S4.4.7.1-3. Sharma V, Burkhart HM, Dearani JA, et al. Surgical unroofing of anomalous aortic origin of a
S4.4.7.1-4. Eckart RE, Shry EA, Burke AP, et al. Sudden death in young adults: an autopsy-based series of a
S4.4.7.1-5. Angelini P, Uribe C, Monge J, et al. Origin of the right coronary artery from the opposite sinus of
Valsalva in adults: characterization by intravascular ultrasonography at baseline and after stent
angioplasty. Catheter Cardiovasc Interv. 2015;86:199-208.
S4.4.7.1-6. Opolski MP, Pregowski J, Kruk M, et al. Prevalence and characteristics of coronary anomalies
S4.4.7.1-7. Lee H-J, Hong YJ, Kim HY, et al. Anomalous origin of the right coronary artery from the left coronary
sinus with an interarterial course: subtypes and clinical importance. Radiology. 2012;262:101-8.
S4.4.7.1-8. Kaushal S, Backer CL, Popescu AR, et al. Intramural coronary length correlates with symptoms in
patients with anomalous aortic origin of the coronary artery. Ann Thorac Surg. 2011;92:986-91.
S4.4.7.1-9. Frescura C, Basso C, Thiene G, et al. Anomalous origin of coronary arteries and risk of sudden death:
a study based on an autopsy population of congenital heart disease. Hum Pathol. 1998;29:689-95.
4.4.7.2. Anomalous Aortic Origin of Coronary Artery
S4.4.7.2-1. Basso C, Maron BJ, Corrado D, et al. Clinical profile of congenital coronary artery anomalies with
Cardiol. 2000;35:1493-501.
S4.4.7.2-2. Davies JE, Burkhart HM, Dearani JA, et al. Surgical management of anomalous aortic origin of a
S4.4.7.2-3. Sharma V, Burkhart HM, Dearani JA, et al. Surgical unroofing of anomalous aortic origin of a
S4.4.7.2-4. Frommelt PC, Sheridan DC, Berger S, et al. Ten-year experience with surgical unroofing of anomalous
aortic origin of a coronary artery from the opposite sinus with an interarterial course. J Thorac
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wrong coronary sinus, using standard and novel approaches. J Thorac Cardiovasc Surg.
2007;134:1171-8.
S4.4.7.2-6. Mainwaring RD, Reddy VM, Reinhartz O, et al. Anomalous aortic origin of a coronary artery:
medium-term results after surgical repair in 50 patients. Ann Thorac Surg. 2011;92:691-7.
S4.4.7.2-7. Brothers JA, McBride MG, Seliem MA, et al. Evaluation of myocardial ischemia after surgical repair of
anomalous aortic origin of a coronary artery in a series of pediatric patients. J Am Coll Cardiol.
2007;50:2078-82.
S4.4.7.2-8. Donaldson RM, Raphael M, Radley-Smith R, et al. Angiographic identification of primary coronary
anomalies causing impaired myocardial perfusion. Cathet Cardiovasc Diagn. 1983;9:237-49.
S4.4.7.2-9. Kragel AH, Roberts WC. Anomalous origin of either the right or left main coronary artery from the
aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases.
Am J Cardiol. 1988;62:771-7.
S4.4.7.2-10. Davis JA, Cecchin F, Jones TK, et al. Major coronary artery anomalies in a pediatric population:
incidence and clinical importance. J Am Coll Cardiol. 2001;37:593-7.
S4.4.7.2-11. Eckart RE, Shry EA, Burke AP, et al. Sudden death in young adults: an autopsy-based series of a

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S4.4.7.2-12. Maron BJ, Doerer JJ, Haas TS, et al. Sudden deaths in young competitive athletes: analysis of 1866
deaths in the United States, 1980-2006. Circulation. 2009;119:1085-92.
S4.4.7.2-13. Eckart RE, Scoville SL, Campbell CL, et al. Sudden death in young adults: a 25-year review of
autopsies in military recruits. Ann Intern Med. 2004;141:829-34.
S4.4.7.2-14. Opolski MP, Pregowski J, Kruk M, et al. Prevalence and characteristics of coronary anomalies
4.4.7.3. Anomalous Coronary Artery Arising From the PA
S4.4.7.3-1. Cochrane AD, Coleman DM, Davis AM, et al. Excellent long-term functional outcome after an
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origin of left coronary artery from pulmonary artery. Circulation. 1973;48:III2-6.
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4.4.8. Coronary Artery Fistula
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characteristics, natural history. Cathet Cardiovasc Diagn. 1995;35:116-20.
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fistulas—a word of caution. J Thorac Cardiovasc Surg. 2013;145:455-60.
5. Evidence Gaps and Future Directions
S5-1. Gurvitz M, Burns KM, Brindis R, et al. Emerging research directions in adult congenital heart disease:
a report from an NHLBI/ACHA Working Group. J Am Coll Cardiol. 2016;67:1956-64.

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