NATURAL HISTORY OF ASD, VSD, PDA

NATURAL HISTORY OF
ASD/ VSD/ PDA
Dr. Murtaza Kamal
MBBS, MD, DNB
DNB SS RESIDENT (PEDIATRIC CARDIOLOGY)
murtaza.vmmc@gmail.com
DOP:031017
1

INCIDENCE
 Most common CHD
 Isolated VSD: 20 – 30% of all CHD
 Echo studies: 5-50/1,000 newborns [1]
 No sex preference
1. Ooshima A et al. Cardiology 1995;86:402-406.
3

CLASSIFICATION OF VSD
Soto et al classification of VSD
 Perimembranous (membranous/ infracristal ): 70-80%
 Muscular: 5-20%
 Central- mid muscular
 Apical
 Marginal- along RV septal junction
 Swiss cheese septum – multiple defects
 Inlet/ AV canal type: 5-8%
 Supracrital (Conal/ infundibular/subpulmonary/doubly
committed subarterial): 5-7%
Benigno soto et al. Br HeartJ 1980; 43: 332-343
4

HAEMODYNAMIC CLASSIFICATION
5
Perloff’s Clinical Recognition of CHD 6th Edition

FACTORS INFLUENCING OUTCOMES &
NATURAL HISTORY OF VSD
Position & Size
Number of defects
Anatomic structures in the vicinity of the
defect
Association of other malformation
Age at which the defect is recognized
Sex of the patient
6

NATURAL HISTORY: VSD
 Cardiac failure
 Spontaneous diminution in size or closure
 Right or Left ventricular outflow tract obstruction
 Aortic regurgitation
 Pulmonary vascular obstructive disease
 Infective endocarditis
7

CARDIAC FAILURE (CONT.)
Rare in small VSD as size limits the L-R shunt
 In large VSD the relative resistances of the systemic
and pulmonary circulations regulate flow
 Shunt occurs mainly in systole
 Shunt directly to PA
 Enlargement of LA, LV, PA 9

CARDIAC FAILURE (CONT.)
 Moderate sized VSD: Symptoms by 3- 6 months
 Large VSD: CHF in first few weeks
 Risk for recurrent pulmonary infection high, failure to
thrive
 If survives without therapy: Pulmonary vascular disease
develops in the first few years of life
 Rudolph AM, et al.Pediatrics 1965;36:763-772
10

2. SPONTANEOUS DIMINUTION IN SIZE
 Occurs in both: Perimembranous+ muscular
 Closure documented in fetus and adults
 VSD diagnosed in the fetus: 46% (in utero closed),
23.1% (closed in 1st YOL), 30.8% (remained patent)
 15.8% of defects <3mm, 71.4% of defects >3mm:
remained patent at 1YOL
 None of the malaligned defects closed
 69% of the perimembranous defects and 60% of
muscular trabecular defects closed spontaneously
11
*Nir A et al.Pediatr Cardiol 1990; 11: 208–10

SPONTANEOUS DIMINUTION IN SIZE
(CONT.)
12
More common in <10 years of age
75% of small VSDs and 83% of muscular defects close
spontaneously by 10 years of age[1]
1. Alpert BS et al., Spontaneous closure of small ventricular septal defects: ten-year follow-up. Pediatrics 1979; 63:
204–6

(CONT.)
.
 Spontaneous closure decreases substantially
after 1 year of age
 Female predominance
 1. Moe DG, Guntheroth WG. Spontaneous closure of uncomplicated ventricular septal defect.
Am J Cardiol 1987; 60: 674–8.
 2. Farina MA et al . J Pediatr 1978; 93: 1065–6.
13

(CONT.)
 During adolescence -- diminution in size of the defect in
> 20%[1]
 VSD can even close in the adults: [2]
Spontaneous closure
 In 10% of 188 adults
17 to 45 years
Followed for a mean of 13 years
 1. Onat T, Ahunbay G, Batmaz G, Celebi A.The natural course of isolated ventricular septal defect during
adolescence. Pediatr Cardiol 1998; 19: 230–4.
2. Neumayer U, Stone S, Somerville J. Small ventricular septal defects in adults. Eur Heart J 1998
14

(CONT.)
 Malaligned VSD rarely undergoes spontaneous
closure or diminution in size
Only in 4% of patients
All of the defects were initially < 4 mm in
diameter
Tomita H, Arakaki Y,Yagihara T, Echigo S. Incidence of spontaneous closure of outlet ventricular
septal defect. Jpn Circ J 2001; 65: 364–6.
15

MECHANISMS OF SPONTANEOUS
CLOSURE OF VSD
16
A. Closure of a perimembranous defect by adhesion of the tricuspid leaflets to the defect margin.
B. Closure of a small muscular defect by a fibrous tissue plug.
C. Closure of a muscular defect by hypertrophied muscle bundles in the right ventricle
D. Closure of a defect in subaortic location by adhesion of the prolapsed aortic valve cusp
E. Aneurysmal transformation of the membranous septum

3. RVOT OBSTRUCTION
 Incidence: 3-7%
 Mechanism:
 Hypertrophy of malaligned infundibular septum
 Hypertrophy of right ventricular muscle bundles
 Prolapsing aortic valve leaflet
High incidence in:
 Rt sided aortic arch
 Horizontal RVOT
Varghese PJ et al . Br Heart J 1970; 32: 537–46
Tyrrell MJ et al. Circulation 1970; 41 & 42(Suppl. III): 113
17

AORTIC VALVE PROLAPSE
 VSD with direct contact with aortic valve are most
prone to develop AVP:
All perimembranous defects
All doubly committed juxta arterial defects
Most of muscular outlet defects
Prevalence of AOP (2-7%)
Rare before age of 2 years
Nadas AS et al . Circulation 1977; 56(No.2, Suppl. I): 1–87.
18

AORTIC VALVE PROLAPSE (CONT.)
 RCC (60-70%), NCC (10-15%), Both (10-20%),
LCC (extremely rare)
 NCC prolapses in perimembranous type VSD
 AR may be due to incompetent bicuspid valve
 Prolapsed valve may perforate rarely
19

 Aortic regurgitation: 10%
 Increases with age
 Infundibular VSD vs membranous VSD – RR 2.5
 AR is progressive , so early surgical intervention
needed
 Momma K, Toyama K, Takao A, et al. Natural history of subarterial infundibular ventricular septal defect.
Am Heart J 1984; 108:1312
20

21

22
Parasternal long-axis echocardiogram view showing supracristal ventricular septal
defect (arrow) with buckling and prolapse of the right coronary cusp of the aortic
valve.

VSD WITH AI- INDIAN SCENARIO
 Sample size: 362, 37(10.2%) had AI
 Mean age: 13.4 y (2-45yrs), M:F::5:1
 31(84%): Infracristal; 6(16%): Supracristal VSDs
 In infracristal VSD: RCC prolapse:14(48%);
NCC: 12(41%); Both: 3(11%)
 In supracristal VSD: RCC prolapse: 5(83%);
NCC prolapse: 1(17%)
 2 patients, AR due to bicuspid aortic valve
 No relationship- severity of AR & location of VSD
 Somanath HS et al. Indian Heart J. 1990 Mar-Apr;42(2):113-6.
23

INTERVENTION IN VSD WITH AVP
 Indicated for both perimembranous and subarterial
VSDs when more than trivial AI
 Subarterial VSDs >5mm: Closed regardless of AVP
 Restrictive perimembranous VSD with AVP but
without AI, surgery indications less clear:
Regular follow up
Surgery indicated only if AI develops
 1. Elgamal MA et al . Ann Thorac Surg 68:1350-1355, 1999
2. Lun K et al. Am J Cardiol 87:1266-1270, 2001
3. Gabriel HM et al. J Am Coll Cardiol 39:1066-1071, 2002
24

LVOT OBSTRUCTION
 Usually above the defect and occasionally below
 May be evident from immediate post natal period
 Progression of the pre-existing lesion or can be
acquired
 Obstruction is either muscular of fibromuscular
Starnes SL et al. Thorac Cardiovasc Surg 2001; 122: 518–23., Liu SP et al. Endothelion 1994; 2: 11–33
25

4. PULMONARY VASCULAR OBSTRUCTION
 Incidence: 5-22%[1]
 Rare in small and moderate sized VSDs
 Mechanism: Excessive pulmonary blood flow Vessel
injury Thick adventitia, medial hypertrophy, intimal injury[2]
 Down syndrome babies develop PAH early
 No sex predilection
 Infants with VSD and increased pulmonary arterial pressure-
repair between 3-12 months
 1. Keith JD et al. Heart Disease in Infancy and Childhood. 1978: 320–79
 2. Wilkinson JL.. Ped Cardiovascular Med. 2000;289–09
 3. DuShane JW, et al.The Child with CHD after Surgery. Futura Publ.1976
26

5. INFECTIVE ENDOCARDITIS
 18.7 per 10,000 person years in non operated
cases
 Operated VSDs: 7.3 per 10,000 person-years
 Higher in small defect and lower during childhood
 Age >20years and male sex are other risk factors
Gersony WM et al.Circulation 1993; 87(Suppl. I):I-121–I-126
27

INFECTIVE ENDOCARDITIS (CONT.)
 TEE: Diagnostic procedure of choice
 Membranous VSD: Vegetation on the tricuspid valve
 Infundibular defects: Aortic or pulmonary valve vegetation
 ACC/AHA : No antibiotic prophylaxis for the acyanotic
uncomplicated VSD with no prior history of endocarditis
 Patients with a proven episode of endocarditis: Increased risk of
recurrent infections, so surgical closure may be recommendedtion
so surgical closure may be recommended 28

ENDOCARDITIS PROPHYLAXIS
 The 2007 guidelines of the AHA recommend no antibiotic
prophylaxis in children with an isolated VSD except :
 During the first six months after the repair with prosthetic
material or device
 Repaired VSD with a residual defect at the site or adjacent to
the site of a prosthetic device
 Prophylactic antibiotics is recommended for dental and
respiratory tract procedures 29

6. ARRHYTHMIAS
 Presenting signs of VSD in adults
 Ventricular tachycardias : 5.7 %
 Sudden death: 4.0 %
 Age and pulmonary artery pressure: Best predictors for
ventricular arrhythmias
 Assess for candidacy for VSD repair in all adults with VSD and
cardiac arrhythmias
1. Wolfe RR et al. Arrhythmias in patients with valvar aortic stenosis, valvar pulmonary stenosis, and
ventricular septal defect. Results of 24-hour ECG monitoring. Circulation 1993; 87:I89.
30

ARRHYTHMIAS (CONT.)
 VSD with Eisenmenger syndrome:
 VT: 19%
 Hypertrophied RV : Ideal for ventricular arrhythmias
 Atrial fibrillation:
 Prevalent in adult VSD
 Risk factors:
Increasing age
LV dysfunction
Elevated PA pressure
 Kidd L et al. Second natural history study of congenital heart defects. Results of treatment of
patients with ventricular septal defects. Circulation 1993; 87:I38 31

CAUSES OF IMPROVEMENT IN SYMPTOMS
OF VSD
 Closing defect: Soft S2
 Increasing PVR: Increased RV pulsations, parasternal
heave, S2 loud
 Infundibular hypertrophy & resulting decreased L to R
shunt: S2 decreases in intensity ,crescendo-
decrescendo systolic murmur in the ULSB
 Cyanosis (shunt reversal )
32

PREGNANCY AND VSD
 Mother: Cardiac complications rare
 No events of arrhythmias, heart failure
 Cardiovascular events (MI, stroke, CV mortality): 1%
 Fetus
 Preterm delivery :11.7%
 Fetal mortality : 1.3%
 Recurrent congenital heart disease of any type : 2.7%
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
34

NATURAL HISTORY OF CHD STUDIES
35

VSD: NATURAL HISTORY IN A NUTSHELL
36
Perloff 5th edition

INTRODUCTION
 Functional closure in term infants within 10–15 h
 <30 weeks gestation : PDA persists D4OL in 65 %
 Anatomical closure is usually complete: 3months
 PDA-Continued patency in infants older than 3 months
 Incidence : 5.3–11.0% (median 7.1%) of all congenital cardiac
lesions
 Casseles DE. The ductus arteriosus. 1973:91
38

INTRODUCTION (CONT.)
 2.9 per 10,000 live births (population-based study of
400,000 term infants)[1]
Isolated PDA among term infants - 0.03 to 0.08 %[2]
 In very low birth weight infants (< 1500 g) - 30 %[3]
1. Reller MD et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr
2008; 153:807
2. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39:1890
3. Lemons JA et al. Very low birth weight outcomes of the NICHD Neona 39

Incidence of silent ductus not known
(0.1–0.2% of the population may be affected)
 Incidence of re-patency of the arterial duct after initial
closure: 0.9%[1]
 Incidence of isolated PDA in term infants: 1 in 2,000[2]
 Female predominance - 3:1 (except in congenital
rubella)[3]
 1. Raaijmaakers B etal., Difficulties generated by the small, permanently patent, arterial duct. Cardiol
Young 1999; 9: 392–5.
 2. Carlgren LE et al. Br Heart J. 1959;21:40 –50.
 3. Zetterquist P et al. University of Uppsala, Sweden 1972 40

 High incidence in:
Maternal rubella
Preterms, LBW babies:
<1750g: 50%
<1200g: 80%[1]
 Teratogens (18-60 days of gestation): Alcohol/
amphitamines/ phenytoin
 Siblings have increased risk (2-4%)
 1. Gibson S et al. Am J Dis Child. 1952;83:117–119.
41

NATURAL HISTORY
 Depends on:
Size and magnitude of shunt
Status of pulmonary vasculature
42

SIZE OF PDA
SMALL MODERATE LARGE
Mostly asymptomatic LV volume overload LV volume overload
and cardiac failure at
3 months of age
Increased risk of
endarteritis
Rarely rhythm
disturbances like atrial
fibrillation
Increased pulmonary
resistance
Eisenmenger’s
syndrome occurs by
1st decade
43

NATURAL HISTORY
 Congestive heart failure
 Infective endarteritis
 Pulmonary vascular disease
 Aneurysmal formation
 Thromboembolism
 Calcification
44

CONGESTIVE HEART FAILURE
 Clinical course is similar as VSD
 Depends on size, magnitude of shunt & the status of the
pulmonary vasculature
 CHF develops in infancy or during adult life
 Heart failure in infancy usually occurs before 3 months
of age
 Commonest cause of death
 Initially lt heart failure, later right heart failure
45

INFECTIVE ENDARTERITIS
 Incidence: 1% per year
 Decreased dramatically since the early natural
history studies before the era of routine surgical
closure and antibiotic usage
 Vegetations usually occur on PA end, embolic
events usually of the lungs rather than systemic
circulation
 Perloff 5TH edition
46

PULMONARY VASCULAR DISEASE
 Large PDA if defect not repaired
 No definite data on incidence
 Differential cyanosis
 Non restrictive PDA, Eisenmenger’s develops by 1st
decade
 Eisenmenger patients do no tolerate PDA closure
47

PULMONARY VASCULAR DISEASE (CONT.)
 PDA: Relatively common cause of PAH of unknown cause
in adolescents and adults
 In one series of 24 patients, 16 had a left-to-right shunt
with PAH of unexplained cause:
ASD in 8
PDA in 6
VSD in 2
In this series these lesions were best detected by
transesophageal echocardiography
Chen WJ, Chen JJ, Lin SC, et al. Detection of cardiovascular shunts by transesophageal echocardiography in
patients with pulmonary hypertension of unexplained cause. Chest 1995; 107:8
48

ANEURYSM OF DUCTUS ARTERIOSUS
 Incidence: 8%
 MC presents in infancy
 In adults develop after:
Infective endarteritis
Surgical closure
Transcatheter coil occlusion
49

ANEURYSM OF DUCTUS ARTERIOSUS
(CONT.)
 Complications:
 Thromboembolism
 Dissection
 Rupture
 Inspiratory stridor
 Left recurrent laryngeal nerve palsy
 Pulmonary artery obstruction and death
 Regression: Due to thrombosis and organization
50

MORTALITY
 Causes of death:
CCF: MC
Rupture of:
Ductal aneurysm
Hypertensive aneurysmal pulmonary
trunk
51

MORTALITY FOR UNTREATED PDA
 Cambell M estimated natural history mortality rates
for untreated PDA:
0.42% per year from age 2-19 yrs
1-1.5% per year in 3rd decade
2-2.5% per year in 4th decade
4% per year after 4th decade
 Campbell M. Natural history of persistent ductus arteriosus. Br Heart J 1968; 30: 4–13.
52

PDA IN PRETERMS
 Heart failure in first 2-3 DOL
 Surfactant treatment: Earlier manifestation
 Reopening of the duct
 Pulmonary effects:
Pulmonary edema
Pulmonary haemorrhage
Bronchopulmonary dysplasia
 Systemic and cerebral blood flow effects
53

PDA IN PRETERMS (CONT.)
 Reopening of the duct:
 Spontaneously or following indomethacin treatment
 Study on 77 preterms with complete clinical closure of PDA
after indomethacin treatment:
Clinically significant PDA recurred in 23%
Rate of reopening was related to decreasing gestational
age:
<27 weeks: 37%
27-33 weeks; 11%
 Weiss H. Factors determining reopening of DA after successful clinical closure with indomethacin. J Pediatr
995;127:466
54

 Pulmonary haemorrhage:
Increased pulmonary blood flow
126 babies born <30 weeks gestation
12 had pulmonary haemorrhage
Greater median PDA diameter ( 2 vs 0.5mm)
Greater PBF (326vs 237 ml/kg/min)
 Kluckow M et al. Ductal stenting, Hign PBF and pulmonary haemorrhage. J Pediatr 2000;137:68

55

 Bronchopulmonary dysplasia:
In clinically significant PDA: Pulmonary edema
and subsequently BPD
865 VLBW neonates: PDA associated with 4.5
fold increase in BPD
 Marshall DD et al. Risk factors for CLD in the surfactant era. North Carolina Neonatologists Association.
Pediatrics. 1999;104:1345 56

 Long term pulmonary effects: Related to the
duration of ductal patency even when PDA is
asymptomatic
 Increased risk of intraventricular haemorrhage in
preterms with PDA
57

PDA: NATURAL HISTORY IN A
NUTSHELL
58
Perloff 5th edition

INCIDENCE
 941 per million live births[1]
 13% of CHD
 Birth prevalence of 1.64 per 1,000 live births[2]
1. Hoffman JIE, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39: 1890–900.
2. van der Linde D et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-
analysis. J Am Coll Cardiol 2011; 58:2241
60

INCIDENCE (CONT.)
 Female predominance : 2:1 in OS ASD[1]
 OS ASD: Sporadic with multifactorial inheritance
 Incidence of recurrence of ASD with an affected parent: 10%
(3 – 16%)[2]
 Particular association with the Holt–Oram syndrome (AD)
1. Beerman LB et al., Atrial septal defect. In: Anderson RH et al., eds. Paediatric Cardiology, 2nd edn. London:
Churchill Livingstone, 2002
2. Gold RJ, Rose V, Yau Y. Severity and recurrence risk of congenital heart defects exemplified by atrial septal
defect secundum. Clin Genet 1987; 32: 148–55
61

ASD: NATURAL HISTORY
 The natural history depends on:
Size of the defect
Rt. and Lt. ventricular diastolic compliance
Pulmonary to systemic vascular resistance
62

NATURAL HISTORY (CONT.)
 Hemodynamic / anatomic abnormalities resulting from
a OS ASD includes:
Right ventricular and atrial volume overload
Pulmonary vascular obstructive disease
Tricuspid and / or pulmonary valve regurgitation
Supraventricular tachyarrythmias 63

 Shunt direction and magnitude are variable and age dependent
 Fetal life - RV noncompliance - unidirectional right- to–left flow
at the atrial level
 Immediately after birth - RV compliance comparable to that of
LV - little net shunting through ASD
 Physiological fall in pulmonary vascular resistance  the RV
thins  compliance increases  left-to-right shunt develops
 With similarly sized ASDs, adults have larger shunts
64

 Most infants with ASD: Asymptomatic
 CHF: Rare in 1st decade
 Heart failure/ FTT in infancy: Rule out associated
cardiac defects
 In one series of 6 infants who had OS ASD repair before 1
year of age for failure to thrive 5 had other
pulmonary and/or cardiac disorders
Andrews R et al. Atrial septal defect with failure to thrive in infancy: hidden pulmonary vascular disease?
Pediatr Cardiol 2002; 23:528
65

 Most children and adolescents with an isolated OS
ASD are asymptomatic even in the presence of
large shunts
 Review of 481 patients with OS ASD seen b/w
1957-76, who underwent surgical correction before
age of 40:
 Defect discovered in routine exam: 202(42%)
 More than one half had symptoms of dyspnea and
fatigue
Richard H et al. ASD of secundum type in patients under 40 years of age. A review of 481
operated cases. Symptoms, signs, treatment and early results. Scand J Thorac Cardiovasc Surg
1979;13:123
66

 Symptoms, heart size, RVH, PA pressures, systemic
desaturation, and atrial arrhythmias increased
progressively with age[1]
 Only 4% of those over 40 years of age denied
symptoms[2]
1. Hamilton WT et al. Atrial septal defect secundum: clinical profile and physiologic correlates. In: Roberts WC, ed.
Congenital Heart Disease in Adults. Philadelphia: FA Davis, 1979: 267–81.
2. Borow KM, Karp R.Atrial septal defect. Lessons from the past, directions for the future. N Engl J Med 1990;
323: 1698–700.
67

SPONTANEOUS CLOSURE
 Spontaneous closure most likely in:
 ASDs < 7-8mm
 Younger age at diagnosis
 A review of 101 infants : mean age at diagnosis 26 days, average
follow up of 9 months:
 Spontaneous closure in all 32 ASDs < 3mm
 87% of 3 – 5 mm ASDs
 80 % of 5 - 8 mm ASDs
 None of 4 infants with defects >8mm
 Radzik D et al., J Am Coll Cardiol, 1993 68

SPONTANEOUS CLOSURE (CONT.)
Cross sectional echocardiograms on 102 consecutive neonates.
Atrial openings were evident in:
24 infants (24%): < 1 week
13 (13%): >1 week
7 (7%): > 1 month
5 (5%): > 6 months
2 (2%): > 1 year
Fukazawa M et al., Atrial septal defects in neonates with reference to spontaneous closure. Am
Heart J 1988; 116: 123–7.
69

SPONTANEOUS CLOSURE (CONT.)
 A follow up of 84 children for 4 years showed a spontaneous
closure or decreased size in:
 89% with a 4 mm ASD
 79% with 5-6mm defect
 7% with defect >6mm
 Even infants with CHF can have spontaneous reduction in the size
of ASD years after diagnosis
 Occasionally spontaneous closure can occur as late as 16 years
 Helgason H et al., pediatr cardiol, 1999 70

MECHANISMS OF SPONTANEOUS
CLOSURE OF ASD
Fusion of valve-like openings in the oval fossa:
predominant mechanism[1]
Atrial septal aneurysm: Spontaneous closure[2]
 1. Fukazawa M et al., Atrial septal defects in neonates with reference to spontaneous closure. Am Heart J
1988; 116: 123–7.
2. Brand A et al., Natural course of atrial septal aneurysm in children and the potential for spontaneous closure of
associated septal defect. Am J Cardiol 1989; 64: 996–1001. 71

ENLARGEMENT IN SIZE OF ASD
 Series of 104 patients (average age 4.5 years at diagnosis)
with isolated ASD >3 mm in size ; follow up – 3 yrs
Spontaneous closure -- in only 4 patients
ASD diameter increased in 65 %
30 % of patients had a >50 % increase in diameter
12 % had an increase to >20 mm
The only independent factor associated with growth was
the initial size of the ASD
McMahon CJ, Feltes TF, Fraley JK, et al. Natural history of growth of secundum atrial septal defects and
implications for transcatheter closure. Heart 2002; 87:256
72

SMALL ASD
Outcomes of 30 infants with an ASD considered too small for surgical
closure
 Mean age at diagnosis: 1.3 years
 Mean follow-up duration: 11.5 years
 17/30: Spontaneous closure (Mean age – 8.4 yrs)
 7/30: Asymptomatic (Average – 14 yrs) – Defect 1–6 mm
 6/30:
 Increase in size of the defect
 Secondary clinical and hemodynamic consequences
 Required intervention
Brassard M, Fouron JC, van Doesburg NH, Mercier LA, De Guise P. Outcome of children with atrial septal
defect considered too small for surgical closure. Am J Cardiol 1999; 83: 1552–5.
73

NATURAL HISTORY IN ADULTS
 Four common clinical presentation of ASD in adults:
Progressive shortness of breath with exertion
Pulmonary vascular obstructive disease
Atrial arrhythmia
Stroke or other systemic ischemic event 74

PROGRESSIVE SHORTNESS OF
BREATH
 Initial symptoms associated with an ASD may be mild and
ignored by the patient
 In a series of 32 patients diagnosed by incidental findings
on physical examination, chest x-ray, or echocardiography
who were thought to be asymptomatic, exercise
tolerance improved after closure of the ASD
Giardini A et al. Determinants of cardiopulmonary functional improvement after transcatheter atrial septal
defect closure in asymptomatic adults. J Am Coll Cardiol 2004; 43:1886 75

ATRIAL ARRHYTHMIAS
 Part of the late natural history of ASD
 May be the first presenting sign:
13% in > 40 years
 52% in >60 years of age[1]
 Associated with morbidity and mortality especially in the older
adult patient
 Associated with the onset of CHF and systemic embolization
(stroke)
 1. St John Sutton MG et al., circulation 1981
76

ATRIAL ARRHYTHMIAS (CONT.)
 Higher pulmonary arterial pressures and a worse NYHA functional
class[1]
 Prevention of atrial arrhythmia is one of the reasons for repairing
ASD in young asymptomatic patients
 Development of AF post intervention may depend on the patient’s
age at intervention and may occur despite surgery in pts >25 years
of age[2]
 1. Gatzoulis MA, Freeman MA, Siu SC, Webb GD, Harris L. Atrial arrhythmia after surgical closure of atrial septal
defects in adults. N Engl J Med 1999; 340: 839–46.
2. Murphy JG et al., N Engl J Med 1990
77

ATRIAL FIBRILLATION
 In three series with a total of over 600 patients, atrial
fibrillation or atrial flutter was present in 20 %
 In a report of 211 adults, atrial fibrillation or atrial flutter:
18 - 40 years: Uncommon (1%)
40 – 60 years: 15%
>60 years : 61%
Berger F, Vogel M, Kramer A, et al. Incidence of atrial flutter/fibrillation in adults with atrial septal defect
before and after surgery. Ann Thorac Surg 1999; 68:75 78

ATRIAL FIBRILLATION (CONT.)
 Multifactorial nature of atrial fibrillation in ASD:
 Longstanding volume loading
 Pulmonary hypertension
 Ventricular dysfunction
 Atrioventricular valve regurgitation
 Increase atrial pre- and afterload
 Increase the degree of atrial myocardial stretch
 Prolongs atrial refractoriness in a heterogeneous manner
 Vulnerable to the induction of fibrillation
79

ATRIAL FIBRILLATION (CONT.)
 Predisposing conditions
Age (with a RR of 1.9 per decade of age)
LA dimension (RR 2.8 for each 10 mm increase)
MR (RR 3.0 for each degree of MR)
TR (RR 1.9 for each degree of TR)
Oliver JM, Gallego P, Gonzalez A et al. Predisposing conditions for atrial fibrillation in atrial septal
defect with and without operative closure. Am J Cardiol 2002; 89: 39–43.
80

PULMONARY VASCULAR OBSTRUCTION
 Uncommon in ASD
 Incidence: 5-10% of untreated ASDs
 Predominantly in females
 Sinus venosus ASDs have higher PA pressures and
resistances than patients with OS ASDs
Vogel M et al. Heart. 1999
81

(CONT.)
 169 patients with ASD:
 Pulmonary hypertension:
Sinus venosus defect: 26 %
Isolated secundum ASD: 9 %
 Elevated pulmonary vascular resistance in:
Sinus venosus defect: 16 %
Isolated secundum ASD: 4 %
Vogel M, Berger F, Kramer A, et al. Incidence of secondary pulmonary hypertension in adults with atrial
septal or sinus venosus defects. Heart 1999; 82:30
82

PULMONARY VASCULAR
OBSTRUCTION (CONT.)
83
Cherian et al. 709 patients
isolated ASD:
Used a PVR> 5Woods U as
criteria:
 12% of those <10y
 10% of those b/w 11-20y
 17% of those b/w 21-30 y
 19% of those b/w 31-40y
 11% of those >40y
Eisenmenger in:
 7% in 1st decade
 8% in 2nd decade
 10% in 3rd decade
 11% in 4th decade and
beyond
Development of pulmonary
vascular disease was
independent of age
Cherian G et al. Am Heart J.1983.

(CONT.)
 Eisenmenger syndrome in ASD occurs predominantly in
females
 Brickner Me at. Al. Congenital Heart Disease in Adults. N Engl J Med.2000.
84

PARADOXICAL EMBOLISATION
 Series of 103 patients (mean age 52 years) with presumed
paradoxical embolism:
PFO was present in 81
ASD in 12
Both a PFO and ASD in 10
Khositseth A et al. Transcatheter Amplatzer device closure of atrial septal defect and patent foramen ovale
in patients with presumed paradoxical embolism. Mayo Clin Proc 2004; 79:35
85

MVP/MR IN ASD
 MR may complicate the late course of an unrepaired OS ASD
 The morphology responsible for MR:
Prolapsing MV
Isolated cleft of the anterior mitral leaflet
 Myxomatous abnormality of the MV: 25% OS ASDs
 Mitral valve prolapse is present in up to 70 % of patients with OS
ASD
 Related to a change in the left ventricular geometry associated
with right ventricular volume overload
 Balakrishnan KG et al. Structural basis of MV dysfunction associate with OS ASDs. Cardiology. 1993;82:409-
14
86

LV DYSFUNCTION
 Uncommon
 Can occur after many years in patients with an
uncomplicated ASD
 More subtle evidence of LV dysfunction is a frequent
finding
 In a study of 12 adults with OS ASD, mean CI was
significantly reduced (3.6 vs 4.5L/min/m2)
 Papio Kaet al. Abnormalities of LV function and geometry in adults with ASD. Ventriculographic,
haemodynamic and echocardiographic studies. Am J Cardiol;1975;36:302.
87

LV DYSFUNCTION (CONT.)
 Cause: Unclear
 ?? Associated intrinsic LV abnormality
 Currently thought that reversible mechanical factors operating
primarily on diastolic function are of primary importance
 Ferlinz J. LV function in ASD: are IV interactions still too complex to permit definitive analysis? J Am Coll
Catdiol 1988;12:1237
88

LV DYSFUNCTION (CONT.)
 In an echocardiographic study of 34 children (mean age 9
years) undergoing percutaneous device closure of an
ASD:
LVEDV was diminished prior to the procedure as a
result of leftward IVS shift
 After ASD closure:
Septal shift resolved  LVEDV increased significantly
Increase in EF (from 54.9 to 62.1 %)
 Walker RE et al. Evidence of adverse ventricular interdependence in patients with atrial septal defects. Am
J Cardiol 2004; 93:1374
89

PREGNANCY & ASD
 MC Congenital cardiac lesion in pregnant women[1]
 Uncomplicated ASD; Tolerated well[2]
 Haemorrhage during delivery Inc SVR, dec
venous return Inc in Lt to Rt shunt
 ASD with pulmonary vascular disease:
 High risk of maternal/ fetal mortality
 Pregnancy to be avoided
 1. Zuber M et al. Outcome of pregnancy in women with congenital shunt lesion
 2. McFaul PB et al. Pregnancy complicated by maternal heart disease. A review of 519 women. Br J
Obstet Gynaecol 1988;95:861 90

MORTALITY
 25% OS ASD died just before their 27th year
 50% by their 36th year
 75% by 50th year
 90% by 60th year
 Mean age at death; 37.5 yrs
 Cambell M. Natural history of ASD. Br Heart J. 1970;32:820-6
91

SURVIVAL RATE
 The survival rate of children with ASD is excellent
 United Kingdom Northern Congenital Abnormality survey
of children born between 1985 and 2003 showed:
20-year estimated survival rate of 96.3 for children
diagnosed with ASD

Tennant PW, Pearce MS, Bythell M, Rankin J. 20-year survival of children born with congenital
anomalies: a population-based study. Lancet 2010; 375:649
92

POST INTERVENTION COMPLICATIONS AND
THEIR FOLLOWUP
 Postpericardiotomy syndrome with tamponade
 Evaluation with ECHO
 Annual clinical follow-up if ASD was repaired as an adult
and the following conditions persist or develop:
 PAH
 Atrial arrhythmias
 RV or LV dysfunction
 Coexisting valvular or other cardiac lesions 93

POST INTERVENTION COMPLICATIONS
AND THEIR FOLLOWUP
 Evaluation for possible device migration, erosion, or
other complications - 3 months to 1 year after device
closure and periodically thereafter
 Device erosion (chest pain or syncope) should warrant
urgent evaluation
94

ASD: NATURAL HISTORY IN A
NUTSHELL
95
Perloff 5th edition

NATURAL HISTORY OF ASD, VSD, PDA

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