ACHD guidelines part I
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This document provides guidelines for the diagnosis and treatment of atrial septal defects (ASD), ventricular septal defects (VSD), pulmonary arterial hypertension (PAH), and anomalous pulmonary venous connections (PVC). Key recommendations include: 1. Transcatheter or surgical closure of isolated ASD or VSD is recommended for those with impaired functional capacity, enlarged right heart chambers, and net left-to-right shunt. 2. Medical management of PAH with bosentan or PDE5 inhibitors is recommended. 3. Surgical repair is recommended for partial anomalous PVC or scimitar vein to reduce right heart volume overload.
ACHD guidelines part I
- 1. ACHD guidelines 2018 Part I Dr. Nagula Praveen
- 2. ACHD Generally for isolated defects • Class I - Symptomatic, significant overload, significant shunt, QP/QS>1.5:1, PASP <50% of SAP, PVR <2/3 of SVR • Class IIa - asymptomatic • Class IIb – defects QP/QS 1.5:1, PASP >50% of SAP, PVR > 1/3 SVR • Class III - Eisenmenger
- 3. Medical management of PAH • Bosentan – class I • PDE5 inhibitors – class IIa • Combination therapy – class IIa
- 5. Diagnostic Aspects • Class I recommendations • 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. • CMR, CCT, and/or TEE are useful to evaluate pulmonary venous connections in adults with ASD. • Echocardiographic imaging is recommended to guide percutaneous ASD closure.
- 6. Important Points • 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. • 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 the septum and pulmonary venous connections. • Anomalous right upper and middle lobe pulmonary venous connections often occur in combination with superior sinus venosus defect.
- 7. When to use CMR,CCT? • Ideal for delineating pulmonary venous connections, particularly those that are associated with veins that may be difficult or impossible to image by echocardiography (eg, innominate vein or vertical vein).
- 8. Therapuetic Recommendations Class I recommendation • Isolated ASD with impaired functional capacity • RA/RV enlargement, net left to right shunt • QP/QS > 1.5:1 without cyanosis at rest or during exercise • Systolic PA pressure < 50% of systolic systemic pressure or PVR < 1/3 of SVR. 1. Transcatheter or surgical closure to reduce RV volume and improve exercise tolerance is recommended. 2. Surgical closure of OP ASD, CS defect, sinus venosus defect .
- 9. Class IIA • Asymptomatic OSASD • RA/RV enlargement, net left to right shunt • QP/QS > 1.5:1 without cyanosis at rest or during exercise • Systolic PA pressure < 50% of systolic systemic pressure or PVR < 1/3 of SVR. • 1.Transcatheter or surgical closure • 2.Surgical closure of a secundum ASD when a concomitant surgical procedure is being done.
- 10. Class IIB Transcatheter or surgical closure of ASD is recommended when • QP/QS is 1.5:1 • SPAP ≥ 50% of systemic pressures • PVR is >1/3 SVR
- 11. Class III Closure is Not advised when • SPAP > 2/3 of systolic systemic pressure • PVR > 2/3 of SVR • Net Right to left shunt
- 12. What do you assess on TEE ? • Defect size, • Defect morphology, • Atrial rim adequacy, • Pulmonary venous anomalies, and • Left atrial appendage thrombus can be evaluated. • sizing by balloon diameter producing complete occlusion of the defect (“stop flow” diameter) • direct visualization and measurement using ICE
- 13. Does all need cath? • If noninvasive imaging is of sufficiently high quality to estimate PAP, shunt magnitude, not every patient with an ASD requires a diagnostic catheterization before surgical closure.
- 14. Catheterization • To determine detailed hemodynamics for decision-making. • To clarify discrepant or inconclusive noninvasive imaging data.
- 15. If not closed what would happen? What does cyanosis with exercise indicate? • Patients who do not undergo ASD closure have worse long-term outcomes, including atrial arrhythmias, reduced functional capacity, and eventually greater degrees of PAH. • 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.
- 16. Is it an absolute contraindication? NO • 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.
- 17. Other ASDs • 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.
- 18. Some patients may not improve … • 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.
- 19. Where to be precise in closure for benefits.. • Older adults - left atrial hypertension resulting from diastolic dysfunction (may cause symptoms simulating those from an ASD alone) -ASD closure could result in clinical worsening - 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.
- 20. What to do in patients with severe PAH .. • 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.
- 21. • 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.
- 23. Follow up
- 24. Anomalous pulmonary venous connections • Diagnostic • CMR CCT required for diagnosis (I) • Cardiac catheterization for defining hemodynamics (IIa)
- 25. Therapeutic • Class I recommendations • Partial anomalous pulmonary venous connection with impaired functional capacity, RA,RV enlargement, QP/QS>1.5:1, left to right shunt,PA systolic pressure < 50% systemic pressure, PVR <1/3 SVR. • 1. surgical repair • 2. repair is recommended when closing sinus venosus ASD • 3. repair of a scimitar vein • Class II a – asymptomatic patients PVC, scimitar vein
- 26. • 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. • Islaoted 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. • Most common is right UPV to SVC. • RPV to IVC – scimitar vein,sequestration of right inferior lobe of lung. • Left UPV to left innominate vein • Right UPV to SVC.
- 27. prognosis • RV volume overload as in ASD • Increased surgical risk – low velocity veins - thrombosis
- 28. • Surgery usually involves intracaval baffling into the left atrium, Warden procedure or direct reimplantation of the anomalous pulmonary vein directly into the left atrium. • 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. • increased postoperative vein thrombosis
- 29. Cavoatrial anastomosis • To reduce the risk of sinus node dysfunction and SVC obstruction.
- 30. Ventricular septal defect Dr. Nagula Praveen
- 31. Therapeutic recommendations • Class I • Adults with a VSD, left ventricular volume overload and hemodynamically significant shunts should undergo closure • Class II a • Surgical closure of perimembranous or supracristal VSD is reasonable if there is worsening AR secondary to VSD. • Class II b • Surgical closure of VSD if IE is caused by VSD (unless contraindicated). • Closure of VSD if SPAP is ≥ 50% of systemic pressure • Class III • Eisenmenger syndrome
- 32. Imp points • Volume overload to the left heart. • Hemodynamic impact is directly proportional to the size of the shunt and afterload to the ventricles. • Isolated VSDs are most common form of CHD encountered in the pediatric population.
- 33. Spectrum of VSD in ACHD • 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.
- 34. Imp. points • 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. Small defects – 96% Moderate defects – 86% Large defects – 61%. Eisenmenger syndrome – 42% Corone P, Doyon F, Gaudeau S, et al. Natural history of ventricular septal defect. A study involving 790 cases. Circulation. 1977;55:908–15.
- 35. • Transcatheter device occlusion of muscular and perimembranous VSD is feasible, and trials have demonstrated a good safety and efficacy profile. • 6% of patients with small supracristal (subaortic) or perimembranous defects may develop aortic valve prolapse and resultant AR that may be progressive. • 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 time of VSD closure, aortic valve repair may be performed in an effort to stabilize or improve AR.
- 36. • The use of fenestrated devices and fenestrated surgical patches in these patients leaves a small residual shunt to allow decompression of the right heart. • In theory, treatment of these patients with PAH therapies before closure could improve outcomes.
- 38. Follow up
- 40. For balanced AVSD Diagnostic • II a – when pulmonary hypertension is suspected, cardiac catheterization. Therapeutic • I – severe left AV valve regurgitation • I – surgery for primary repair of AVSD, residual shunts if feasible • IIa – for left ventricular obstruction - if maximum gradient is > 50 mm Hg, if HF present (lesser gradient),severe MR,AR. • IIb - if PASP is ≥ 50% SAP • III – Eisenmenger
- 41. • 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.
- 42. Rastelli classification • The Rastelli classification describes anatomic variations of the superior bridging leaflet of the atrioventricular valve. • The relative sizes of the ventricles as balanced or unbalanced guide the type of repair (eg, biventricular or single ventricle repair). • AVSD also occurs in association with other congenital lesions including TOF, CoA, and heterotaxy. • Strong association with syndromes, most commonly trisomy 21 (Down syndrome).
- 43. • 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.
- 44. • The atrioventricular node is typically displaced inferiorly in AVSD and is associated with relative hypoplasia of the left anterior fascicle. • Late-onset complete heart block (as late as 15 years 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.
- 45. Intervention timing same as acquired VHD They differ mainly • 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. • Risk of arrhythmias, subaortic stenosis, history of one prior repair. • 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. • 1/3 require reoperation
- 46. • Valve replacement – mechanical > bioprosthetic • Valve repair > replacement • 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 be needed.
- 49. • Class I – oxygen saturation in both hands and feer • Class IIa – Cardiac catheteriation in patients with suspected PH. • Class I – significant PDA • Class IIb – PASP > 50% SAP • Class III – eisenmenger
- 50. Follow up
- 51. Imp points • 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. • Closure is typically performed percutaneously with good success and minimal complications. • 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. • Surgical closure can be performed but is potentially hazardous in adults because of calcification and tissue fragility.
- 52. Thank you