Most infants with ASDs are asymptomatic They may present at 6 to 8 weeks of age with a soft systolic ejection murmur and possibly a fixed and widely split S2 CHF rare in the first decades of life but it can become common once the patient is older than 40 yrs

1. DR. JEETHENDER JAIN
Nizam’s Institute of Medical Sciences,
Hyderabad.
jeetu_jain24@yahoo.com

3.  ASD
 VSD
 PDA
 TOF
 CONGENITAL AS
 CONGENITAL PS
 COARCTATION OF AORTA
 EBSTEINS ANOMALY
 CCTGA

4.  spontaneous diminution in size,
 remain stable for many years,
 operative or catheter based closure,
 go on to develop progressive pulmonary vascular
obstruction.

6. Samanek M et al. Pediatr Cardiol1999;20:411–7

7.  The natural history depends on
Size of the defect
Rt. & Lt. ventricular diastolic compliance
Pulmonary-to-systemic vascular resistance

8.  Shunt direction & magnitude are variable and age dependent
 In fetal life, RV noncompliance, a result of high pulmonary
vascular resistance, allows nearly unidirectional right-to-left
flow at the atrial level
 Immediately after birth, with RV compliance comparable to
that of the LV, there may be little net shunting through ASD
 With the physiological fall in pulmonary vascular resistance,
the RV thins, compliance increases, left-to-right shunt
develops

9.  Hemodynamic/anatomic abnormalities resulting
from a secundum atrial septal defect include
Right ventricular and atrial volume overload
Pulmonary vascular obstructive disease
Tricuspid valve and/or pulmonary valve
regurgitation
Supraventricular tachyarrhythmias

10.  Most infants with ASDs are asymptomatic
 They may present at 6 to 8 weeks of age with a soft
systolic ejection murmur and possibly a fixed and
widely split S2
 CHF rare in the first decades of life but it can become
common once the patient is older than 40 yrs

11.  With similarly sized ASDs, adults have larger shunts
 Four common clinical presentations of ASD in adult
 Progressive shortness of breath with exertion
 Pulmonary vascular obstructive disease
 Atrial arrhythmia
 stroke or other systemic ischemic event

13.  Spontaneous closure most likely in
 ASDs <7 to 8 mm
 Younger age at diagnosis
 A review of 101 infants -mean age of diagnosis 26 days
average follow-up of 9 months.
 Spontaneous closure in all 32 ASDs <3 mm
 87% of 3- to 5-mm ASDs
 80% of 5- to 8-mm ASDs
 None of 4 infants with defects >8 mm
Radzik D, et al. J Am Coll Cardiol. 1993;22:851-853.

15. Conclusion
 no follow-up -if a defect is <3 mm.
 defect 3- 5 mm - evaluated by the end of the 12th
month by which time >80% of the defects will be
closed
 defect 5 -8 mm, evaluated by the end of the 15th
month, by which time >80% of the defects will be
closed

16.  Uncommon in ASD
 Incidence is 5% to 10% of untreated ASDs
 Predominantly in females
 Sinus venosus ASDs have higher pulmonary artery
pressures & resistances than patients with secundum
Vogel M et al. Heart 1999; 82: 30–3.

17.  Atrial arrhythmia may be the first presenting sign (13%
in older than 40 & 52% in older than 60 yrs of age)
 Prevention of Atrial arrhythmia is one of the reasons
for repairing ASD in young asymptomatic patients
(Silversides CK et al. Heart. 2004;90:1194 –1198.)
 Subsequent development of AF may depend more on
the patient’s age at intervention and may occur
despite surgery in patients > 25 years of age
St. John Sutton MG,et al. Circulation 1981;64:402-409.
Murphy JG, et al. N Engl J Med. 1990;323:1645–1650

18.  Berger et al. reported an atrial fibrillation
prevalence of
 15% in those 40–60 years of age
 61% among those older than 60 years.

20.  Craig and Selzer in 1968 studied 128 adult patients
 Significant PAH developed in 22% of the series
 This complication usually develops when the patient is
between 20 and 40 years of age
 Cherian et al studied 709 pts.of isolated ASD
 PASP was > 50 mmHg in 17%
 PAH was present in 13% of patients under 10 yrs
 14% of those aged 11 to 20 years
 Eisenmenger syndrome 9%
Craig RJ et al. Circulation 1968; 37: 805–15.
Cherian G et al. Am Heart J 1983; 105: 952–7.

24.  Evidence of Rt. sided cardiac volume loading,Qp:Qs >
1.5:1
 Symptomatic patients (principally exercise related)
 PVR < 7 WU – closure is usually well tolerated
 Need to be mindful of elevated LVEPD
 Pts may need diuretic therapy after closure
 For PVR >7 WU and PA pressures >50%
 need to perform O2 and NO study

25.  Elective repair frequently has been deferred until the
child is at least 4 years of age.
 Early operation has been recommended for those
infants and young children who have unremitting
heart failure or associated pulmonary hypertension
 contraindication :-
 pulmonary hypertension with Rt. to Lt shunting at rest
 Pulmonary vascular resistance of 14 WU

27.  Most common congenital heart defect in children
 Incidence is 8 per 1000 live births
 Echo studies- 5 to 50 per 1,000 newborns
Ooshima A et al. Cardiology 1995;86:402-406.
 No sex preference , except in subarterial defect

28. ASIAN WESTERN
 Doubly commited
subarterial
 Multiple ventricular
septal defects are rare
 Doubly-commited
subarterial defect
requiring repair is 30%
 Muscular defects
 10% in the west
 5% in western
Ferreira Martins JD et al.Cardiol Young 2000; 10: 464–

30.  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

32.  Anterior more common than posterior
 Usually involves the infundibular septum
 Occurs as if there is a door that moves round on a
hinge

33.  The outcome and natural history influenced by
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

36.  Cardiac failure
 Spontaneous diminution in size or closure
 Right or Left ventricular outflow tract obstruction
 Aortic regurgitation
 Pulmonary vascular obstructive disease
 Infective endocarditis

37. 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

38.  After birth decline in PVR to adult level by 7to 10 days
 In large VSDs, the rate of this process is delayed
 Small VSD the shunt is small & remain asymptomatic
 Moderate sized VSD symptoms by 1to 6 months
 Rarely, adults present with new exercise intolerance
.)
Rudolph AM, et al.Pediatrics 1965;36:763-772.

39.  Large VSD -congestive heart failure in first few weeks
 Risk for recurrent pulmonary infection high
 If survives without therapy - pulmonary vascular
disease develop in the first few years of life
 Symptoms “get better” as Qp/Qs returns to 1:1
 Intervention at this time - a shorter life expectancy
than if the defect were left open
Fuster V, et al.Cardiovasc Clin. 1980;10:161–197.

40. Nir A et al.Pediatr Cardiol 1990; 11: 208–10.

41. SPONTANEOUS CLOSURE :
An inverse relation exists between the probability of
spontaneous closure and age at which the patient is
observed (Hoffman and rudolph)
Age % of spontaneous closure
1 month 80%
3 months 60%
6 months 50%
12 months 25%
Adolescence 23% (Onat and colleagues)
Between 21 – 31 yrs only 1 documented case

42.  More frequent in <10 yrs of age
 Isolated VSD ( 124 pts) - 34% at 1 yr & 67% at 5 yr
 Female predominance
 Decreases substantially after 1 year of age
Mehta AV et al. Tenn Med 2000; 93: 136–8.
Farina MA et al . J Pediatr 1978; 93: 1065–6.
Moe DG et al. Am J Cardiol 1987; 60: 674–8.

43.  Rare in malaligned VSD
 In outlet VSD closure only in 4%
All of the defects closed were initially < 4
mm
Tomita H et al. Jpn Circ J 2001; 65: 364–6

44.  Different for perimembranous and muscular
 Perimembranous
Reduplication of tricuspid valve tissue
Progressive adherence of the septal leaflet of the tricuspid
valve about the margins of the VSD
Aneurysmal transformation of the membranous septum
(appearance on angiography)
Early systolic click & late crescendo systolic murmur
Anderson RH et al . Am J Cardiol 1983; 52: 341–5.
Freedom et al. Circulation 1974; 49: 375–84.

45.  Muscular- direct apposition of muscular borders
 Large subarterial defect don’t close
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

46.  Incidence 3% to 7%.
 Mechanism:-
 Hypertrophy of malaligned infundibular septum
 Hypertrophy of right ventricular muscle bundles
 Prolapsing aortic valve leaflet
 High incidence in
Right sided aortic arch
Horizontal RVOT
Nadas AS et al . Circulation 1977; 56(No.2, Suppl. I): 1–87.
Corone P et al. Circulation 1977; 55: 908–15.
Pongiglione G et al . Am J Cardiol 1982; 50: 776–80.
Varghese PJ et al . Br Heart J 1970; 32: 537–46.
Tyrrell MJ et al. Circulation 1970; 41 & 42(Suppl. III): 113.

47.  VSD with direct contact with the aortic valve are
most prone to develop AVP
All the perimembranous defects
All doubly committed juxtaarterial defects
Most of muscular outlet defects
 Characteristic deformity of aortic cusp-nadir of the
cusp is elongated

48.  RCC (60-70%) , NCC (10-15%) , both in 10-20%
 Non-coronary cusp prolapse in perimembranous type
 Left coronary cusp prolapse extremely rare
 AR may be due to incompetent bicuspid aortic valve
 Rarely prolapsed valve cusp may perforate

49. Komai H et al.Ann Thorac Surg 64:1146-1149, 1997

50.  Unknown exact prevalence (2% to 7%)
 Rare before 2 years
 More severe - additional volume load
 Aneurysm of sinuses of Valsalva may develop
Nadas AS et al . Circulation 1977; 56(No.2, Suppl. I): 1–87.

51.  Indicated for both perimembranous and subarterial
VSDs when more than trivial AR
 Subarterial VSDs >5 mm - closed regardless of AVP
 Restrictive perimembranous VSD with AVP but
without AI, surgery indications are less clear
Follow up regularly
Surgery is indicated only if AI develops
Elgamal MA et al . Ann Thorac Surg 68:1350-1355, 1999
Lun K et al. Am J Cardiol 87:1266-1270, 2001
Gabriel HM et al. J Am Coll Cardiol 39:1066-1071, 2002

52.  Usually above the ventricular septal defect
Etiology:-
Progression of the pre-existing lesion
Acquired
Two types:-
Muscular
Fibromuscular

53.  Incidence - 5% to 22%
 Rare in small & Moderate-size VSDs
 Down syndrome – early development of PAH
 No overall sex predilection
Keith JD et al. Heart Disease in Infancy and Childhood. 1978: 320–79.

54.  Survival rate for patients with VSD by pulmonary
artery systolic pressure
 42 men and 37 women, 18 to 59 years (mean 34 yrs)
 67 patients treated medically and 12 surgically
 All patients were followed up for 1 month to 25 yrs ( mean 9yrs)
The solid line indicates a pressure less
than 50 mm Hg (n = 36)
Dashed line indicates a pressure of 50
mm Hg or greater (n = 17)
Ellis JH 4th
et al . Am Heart J. 1987;114:115-205

55.  Eisenmenger complex, develops in 10% to 15%
most commonly in the 2 nd
& 3rd
decades of life
common causes of death "sudden" or "unknown“
 Development of pulmonary vascular disease after
surgery depends on age at which procedure is done
 Infants with VSD and increased pulmonary artery
pressure - repair between 3 and 12 months

56.  18.7 per 10000 person-years in non operated cases
 Operated VSD 7.3 per 10000 person-years
 Higher in small defect & lower during childhood
 Patients with a proven episode of endocarditis are
considered at increased risk for recurrent infection
so surgical closure may be recommended
Gersony WM et al.Circulation 1993; 87(Suppl. I):I-121–I-126.

57.  Patients with VSD have a high incidence of arrhythmia
Ventricular tachycardias in 5.7%
Sudden death is 4.0%
SVT, mostly AF, is also prevalent
 Age and pulmonary artery pressure are the best
predictors of arrhythmias
 The odds ratio of serious arrhythmias increases
1.51 for every 10-year increase in age
1.49 for 10mm Hg increase in mean PA pressure
Wolfe RR et al. Circulation. 1993;87:I89-101

59.  Heart failure not controlled by medical therapy VSD
should be operated with in 6 month of life
 Qp/Qs is 2 or more surgical closure needed
regardless of PA pressure
 VSD with PVR more than 4 unit during 6-12 months
 VSD with elevated PVR first seen after infancy
 Moderate VSD with no size change in childhood

60.  Right bundle branch block
 33.3% undergoing transatrial repair
 78.9% to 11% in repair via a right ventricular incision
 Transpulmonary approach has the lowest incidence
 Complete heart block in 1 to 2%
 Pulmonary hypertension in (4% )
 Sinus node dysfunction (4%)
 Progressive aortic valve insufficiency (16%)
Roos-Hesselin JW et al. Eur Heart J 2004;25:1057-1062.
Abe T et al . Jpn Circ J 1983; 47: 328–35.

64. Clinical evaluation
 Of the patients, 92% were in NYHA class 1, and 8%
in class 2.
 Five patients (5%) were taking medication: oral
anticoagulation in 1 (artificial aortic valve), b-
blockers in 2, and ace-inhibitors in 2 patients.
Oxygen saturation (measured with Nellcor) was
98% (range 94–100%).
No patient showed signs of heart failure.

65. Risk factors for late death
 A median pulmonary artery pressure of more
than 70 mmHg before operation .
 peri-operative complications (re-operation,
arrhythmia, infection) were predictors for late
mortality.

66.  This series is unique in that it comprises a cohort
of consecutive patients operated on at young age
at a single institution with longitudinal follow-up
of 22–34 years.
 Apart from a considerable historical early
mortality (13%), we experienced only a 4% (6
patients) late mortality.

67. Second natural history study of congenital heart defects –
circulation – 1993.
 25 yr survival – 87%
 In medically managed pts,64.5% deaths are cardiac, 35% -
sudden deaths, 3.3% due to IE, 35% due to CHF and 27 %
due to unspecified cardiac cause
 In pts who underwent surgery, 89.8% deaths are cardiac,
6.3% - peri-operative, 39.2% - sudden, 24% - CHF, 30% -
unspecified cardiac cause
 Addl. Surgeries in surgically managed pts – 5.5%
 Pts with eisenmenger syndrome – 10-12 fold higher risk of
death
 AR – 0.7 %, IE - rare
 10 pts among medically managed group developed
eisenmengers & none in surgically managed group

68.  Surgical closure is not indicated in pts with small VSDs &
N.PAP
 If the VSD is large and the pulm. Resistance is increased,
closure early in life is indicated
 If the VSD is moderate in size with an increase in PAP and
N. PVR, decision is less clear. However such pts are at 4-
fold the risk of death and so closure may be reasonable.
Factors that influence course of disease :
Worse prognosis with males, elderly, elevated PAP, RVH,
medical center

69. Patent Ductus ArteriosusPatent Ductus Arteriosus

70.  Incidence of isolated PDA in term infants - 1 in 2,000
 Female predominance - 3:1
 High incidence- Prematurity, Maternal rubella
 Genetic inheritance- Autosomal recessive with
incomplete penetrance

72. Campbell series :
SPONTANEOUS CLOSURE :
Occurs at a fairly constant rate of 0.6 %/yr through
the 1st
4 decades of life (Campbell’s study)
It is generally agreed that spontaneous closure is
uncommon in full term infants beyond 3 – 5
months age.
However, in preterm infants, delayed closure of PDA
is common

73. DEATH :
 30 % die within 1st
yr of life. Risk highest in the 1st
few months
 After infancy, the annual death rate is 0.5 % / yr.
 By 3rd
decade, death rate is 1 %/yr
 By 4th
decade – 1.8 %/yr
 Later, 4 %/yr
 60 % of pts die by 45 yrs age
(natural h/o PDA – Campbell)
 Death is due to CHF or recurrent LRTI and
sometimes due to IE

75.  Congestive heart failure
 Infective endarteritis
 Pulmonary vascular disease
 Aneurysmal formation
 Thromboembolism
 Calcification

76.  CHF resulting from an isolated PDA either develops in
infancy or during adult life
 HF in infancy usually occurs before of 3 mths of age
 Initially left heart failure, later right heart failure
 Good response to drugs initially, but is not maintained

77.  Major cause of death in earlier era
 Incidence - 0.45% to 1.0% per annum
 Vegetations usually found at the PA end of the duct
May cause recurrent pulmonary embolism
 Infection may also cause a ductal aneurysm
Cosh JA. Br Heart J 1957; 19: 13–22.

78.  No definite data on incidence
 “Differential cyanosis”
 Eisenmenger patients do not tolerate PDA closure

79.  As many ducts will eventually close in premature
infants approach to treatment is different - preterm
infant Vs mature, child
 Beyond infancy, closure reported in 0.6% per year
 Medical therapy
 Treatment of Heart failure
 Ductal closure with drugs
 Surgical Therapy
Campbell M et al.Heart 1968;30:4–13.

80.  Coils closure for <3 mm, >97% success, zero mortality
 Larger PDAs - specialized devices
 >98% complete closure rate at 6 months

81. No spontaneous closure
h/ similar to persistently large VSD
Rarely seen in childhood or adult life
Those who survive beyond early life have severe
PVOD

82.  1.5 – 3 % of all CHDs
 50 % survive beyond 3 months
 20 % survive the 1st
yr of life.
 Survival beyond the 1st
yr of life with out surgical
Rx usually have a large ASD. They tend to have a
stable hemodynamic state for 10 -20 yrs with little
change in PVR after which some pts develop
eisenmenger complex.

83. Hazelrig and colleagues – data from 183 autopsied cases of
surgically untreated TAPVC reported in literature :
Median survival – 2 months (range – 1 day to 49 yrs)
90 % of deaths by 1st
yr of life
Obstruction of the pulmonary venous pathway reduced
median survival from 2.5 months in the non obstructed
group to 3 weeks in the obstructed group
Patients with supra cardiac and cardiac connections had a
similar history,with median survival of 2.5 & 3 months
respectively, whereas those with infracardiac type – 3
weeks
Presence of an ASD (rather than a PFO) was associated with
increased survival particularly when the connection is not
infracardiac

85.  Gender distribution is approximately equal.
 malformation recurs in families .
 reported in siblings including triplets, and in
parents and offspring.
 Birth weight lower than normal
 growth and development are generally retarded.

86.  Usually comes to light in neonates and infants.
 Shunt is left-to-right, initial suspicion is a
prominent systolic murmur.
 Shunt is balanced, the murmur persists in addition
to mild, intermittent, or stress-induced cyanosis.
 Shunt is reversed, the prominence of the systolic
murmur is inversely proportional to the degree of
cyanosis.

87.  Early infancy is often benign.
 Mild to moderate neonatal cyanosis tends to increase.
 Cyanosis may be delayed for months.
 Coupled with increased oxygen requirements of the
growing infant rather than with progressive
obstruction to right ventricular outflow.
 By 5 to 8 years of age, most children are conspicuously
cyanotic, with cyanosis closely coupled to the severity
of pulmonary stenosis.
 Infants with TOF and pulmonary atresia are mildly
cyanotic or acyanotic when collateral flow is
abundant.

88.  Analysis of survival patterns based on 566
necropsy cases of Fallot’s tetralogy,
 two thirds of patients reached their first birthday,
 approximately half reached age 3 years,
 approximately a quarter completed the first
decade of life.
 The attrition rate was then 6.4% per year with
 11% alive at age 20 years,
 6% at age 30 years,
 3% at age 40 years

89.  According to data compiled by Bertranou,
 66% of patients not treated surgically live to age 1
year,
 48% to age 3 years
 24% to age 10 years

91.  Samanek et al (central Bohemia.)
 88% survived the first week.
 84% to the first month.
The actuarial survival rate
 at 1 year was 64%,
 49% at 5 years,
 23% at 10 years
 4% at 15 years.
 survival overall is considerably worse in those
patients with tetralogy and pulmonary atresia

92.  Most common CCHD after 4 years of age
 Constitutes a large proportion of adults with
cyanotic congenital heart disease.
 Patients without repair have lived to age 75 years,
78 years, 84 years and 86 years.
 In TOF with pulmonary atresia
 life expectancy without surgery
 as low as 50% in 1 year and 8% in 10 years
 adequate collateral blood flow occasionally
permits survival into adolescence and adulthood.

93.  Pregnancy is poorly tolerated in females who
reach childbearing age.
 The gestational fall in systemic vascular resistance
increases the right-to-left shunt, and labile
systemic vascular resistance during labor and
delivery results in abrupt oscillations in
hypoxemia.
 Fetal wastage is high
 live born infants are dysmature.

94.  Neonatal right ventricle - well equipped to eject
against systemic vascular resistance because the
nonrestrictive VSD permits decompression into
the aorta.
 Right ventricular failure is uncommon
 Biventricular failure in the first few weeks of life
seen in pulmonary atresia with excessive flow
through large systemic arterial collaterals.

95.  Accessory tricuspid leaflet tissue -partially occludes
the VSD results in supra systemic RVSP and right
ventricular failure.
 Absence of the pulmonary valve results in volume
overload of the pressure-overloaded right ventricle.
 Systemic hypertension- increases left and right
ventricular afterload and can induce right
ventricular or biventricular failure.

96.  Acquired calcific stenosis of the biventricular
aortic valve imposes increased afterload on both
the right and the left ventricles.
 Regurgitation of a biventricular aortic valve sets
the stage for right ventricular failure by imposing
volume overload on the already pressure
overloaded right ventricle.
 Infective endocarditis on an incompetent aortic
valve can result in catastrophic acute severe
biventricular aortic regurgitation.

97.  Isotonic exercise – causes
 a fall in systemic vascular resistance in the face of
fixed obstruction to right ventricular outflow
 increasing venoarterial mixing.
 significantly influencing the dynamics of O2
uptake and ventilation.
 Stimulates the respiratory center and the carotid
body, provoking hyperventilation that is
subjectively perceived as dyspnea.

98.  Five mechanisms are therefore involved in the
pathogenesis of Fallot spells:
 (1) an acceleration in heart rate.
 (2) an increase in cardiac output and venous
return.
 (3) an increase in right-to-left shunt.
 (4) vulnerable respiratory control centers.
 (5) infundibular contraction.

99.  Squatting for relief of dyspnea –time honored
hallmark of Fallot’s tetralogy
 Taussig described the preference for certain
postures other than squatting
 knee-chest position,
 lying down,
 sitting with legs drawn underneath

100.  Recurrent hypoxic spells lead to brain damage and
mental retardation.
 Cerebral venous sinus thromboses and small
occult thromboses may become manifest after
prolonged hypoxic spells.
 Velopharyngeal insufficiencymay develop.

101.  Brain abscess and cerebral embolism.
 Iron deficient erythrocytosis -less than 4 years
increases the risk of cerebral venous sinus
thrombosis.
 Wheezing and stridor-attributed to tracheal
compression by an enlarged aorta.
 A stenotic pulmonary valve and incompetent
aortic valve -substrates for infective endocarditis.

102.  Physiologic consequences and clinical course
 Non restrictive VSD –
 favorably influenced by mild to moderate acquired
obstruction to right ventricular outflow
 The clinical picture initially resembles an isolated
nonrestrictive VSD with large left-to-right shunt .
 With the development of RVOT obstruction, excessive
pulmonary blood flow and volume overload of the left
ventricle are curtailed symptoms related to the left-
to-right shunt diminish, and physical development
improves.
 Obstruction RVOT may progress sufficiently to reverse
the shunt, resulting in late onset cyanosis.

103.  restrictive VSD - accompanied by severe
pulmonary valve stenosis, the clinical picture
resembles isolated pulmonary stenosis with intact
ventricular septum.
 restrictive VSD with mild pulmonary stenosis
-associated with a conspicuous murmur and few
or no symptoms but with the risk of infective
endocarditis.

104.  Fortunes of patients with TOF were altered by a
series of primarily surgical maneuvers:
• Blalock–Taussig shunt 1945.
• Potts shunt 1946.
• primary repair 1954
• Waterston shunt 1962
• prostaglandin therapy 1976
• primary repair of the infant 1973.

107.  Shunt failure was more common in patients < 3.0
kg
 The central and Waterston shunts had the highest
30 day mortality of 17%,
 modified Blalock–Taussig shunt had a 7%
mortality.
 Their data also showed an inverse relationship
between mortality and weight.

108.  This approach should avoid the deleterious effects
of a shunt procedure, reduce right ventricular
hypertrophy and hypertension, ideally as a
corollary reducing the risk of late ventricular
arrhythmias.

110.  survival
 impact of pulmonary regurgitation and
requirement for pulmonary valve replacement
 ventricular arrhythmias and sudden death
 atrial arrhythmias
 complete heart block
 ventricular function
 bacterial endocarditis

111.  Older age at surgery
 Higher ratio of RVSP/LVSP
 Transannular patch
 Pulmonary regurgitation
 Uncorrectable lesion in RVOT
 Prolonged CPB time
 Residual shunt>1.5:1 with PVOD
 Pre op- polycythemia

114. • Some degree of pulmonary regurgitation
-inevitable after repair of TOF
• In the long term pulmonary regurgitation if not
trivial-to mild will not be tolerated and will impact
on the form and function of the right ventricle.
• Important pulmonary regurgitation of long
duration will cause right ventricular dilatation,
impair right ventricular performance, lead to
tricuspid regurgitation, and will predispose to
atrial flutter/fibrillation, ventricular arrhythmias,
and sudden cardiac death

115. • Sudden, unexpected death -incidence of from 0.5%-
6%.
• This event is likely related to ventricular arrythmias
or a history of CHB
• Ventricular arrhythmias are seen in the older TOF
patient before repair, and thus may be a
consequence of the disease.
• Sudden, unexpected death - more common when
the patient was repaired at an older vs. younger
age.

117.  Not completely known
 owing largely to the fact that techniques for
diagnosing and quantitating aortic stenosis have
developed simultaneously with the evolution of
surgical and transcatheter treatment modalities.

118.  1993 Report from the Second Joint Study on the
Natural History of Congenital Heart Defects (NHS-
2) presents data from the largest number of
patients and longest follow-up period
 This report provides complete data on only 235
patients (51% of the original NHS-1 cohort) with
median follow-up of 22.5 years.

119.  Congenital aortic valve disease encompasses a
wide spectrum from critical infantile aortic
stenosis to normally functioning bicuspid aortic
valve.
 Only about 1 in 50 of the 1.3% of the population
with congenitally bicuspid aortic valves will
develop significant valve dysfunction by
adolescence.

120.  Patients who present in infancy with aortic valve
stenosis generally have more severe stenosis and
higher mortality with or without treatment.
 25% of the patients in the original NHS-1 cohort
were younger than 2 years old; the 1-year survival
rate was 64%, and most had undergone surgical
intervention.
 In contrast, the 25-year survival in patients who
were 2 years of age or older at the time of original
enrollment was 85%.

121.  50% of infants with severe valvular aortic stenosis
-require hospitalization within the first week of
life because of failure.
 Symptomatic infants require prompt relief of
obstruction by balloon valvuloplasty, the
procedure of choice.

122.  1.Most infants beyond the newborn period and
children with mild aortic valvular stenosis
 (PSPG at catheterization <25 mm Hg or a Doppler
mean pressure gradient <25 mm Hg)
 remain stable
 only a 21% likelihood of progression in severity and
the need for intervention within the subsequent 25
years.
 2.patients with a PSPG between 25 and 49 mm Hg,
 the likelihood of significant progression rises to 41%,
 3.patients with PSPG >50 mm Hg
 it rises to 71%.

123.  Patients with a PSPG >50 mm Hg are at risk for
serious ventricular arrhythmias and sudden death.
 Infective endocarditis on the aortic valve poses
 systemic arterial emboli
 aortic regurgitation with congestive failure, shock,
and death.

124.  Campbell series , published in 1968,
 mean age of death was 35 years,
 40% mortality by age 30
 60% mortality by age 40.
 50% of the patients who died had sudden
unexpected death,
 50% of sudden death cases from aortic stenosis
occurred during or immediately after exercise .
 most of the remaining deaths -due to progressive
CCF.

125.  Clinical presentation
 varies from severe valve disease in infancy to
 asymptomatic valve or thoracic aorta disease in
the older child
 symptoms usually develop in adulthood (Siu &
Silversides, 2010).

126.  A study performed on adult patients with bicuspid
aortic valves showed a median increase of 0.7
mmHg per year in peak Doppler gradient (Tzemos
et al., 2008).
 Pure aortic incompetence due to a prolapsed
leaflet may occur in childhood but is more likely to
develop and progress later in time

127.  Aortic root dilatation has been documented in
childhood, suggesting that this process begins
early in life (Beroukhim et al., 2006; Gurvitz et al.,
2004).
 Its progression is more likely in children with a
larger aorta at baseline, but it is extremely rare to
necessitate intervention before adulthood
(Holmes et al., 2007).

128.  Bacterial endocarditis risk -1% per year,
 NHS-2 data where the incidence rate was 27.1
cases per 10,000 person-years.
 Bacterial endocarditis risk is present even in very
mild aortic valve stenosis, the incidence of
endocarditis is higher in patients with more severe
stenosis.

130.  Bimodal presentation.
 produces significant symptoms in early infancy and
after age 20 to 30 years.
 Neonates with severe coarctation become acutely
symptomatic when the ductus closes.
 Most who survive the hazards of infancy reach
adulthood
 25% die by age 20 years,
 50% die by age 30 years
 75% die by age 50 years.
 Survival has been reported at age 74 years and 76
years.

131.  Mild coarctation is not always benign, and severe
coarctation is not always asymptomatic.
 Except for symptomatic infants, patients tend to
be clinically well when the diagnosis is first made.

132.  Epistaxis .
 Muscular fatigue-When coarctation compromises
the orifice of the left subclavian artery in left-
handed patients.
 Leg fatigue occurs in about half of patients, but
claudication is reserved for abdominal coarctation.
 Dysphagia occurs when a retroesophageal right
subclavian artery originates distal to the
coarctation and passes behind the esophagus or
when coarctation is a component of a vascular
ring.

133. Four eventualities:
 (1) congestive heart failure;
 (2) rupture or dissection of the aorta;
 (3) infective endarteritis or endocarditis; and
 (4) cerebral hemorrhage.
 Hypertension is chiefly responsible for morbidity
and mortality with advancing age.

134.  Highest in infants and is high again after the fourth
decade.
 Review of 234 patients -aged 1 day to 72 years,
heart failure occurred in
 67% of patients -less than 1 year of age and >age 40
years
 4% of patients between 1 year and 40 years of age.
 Many neonates and infants with congestive heart
failure have a coexisting VSD or PDA
 In brief, more than 90%of infants and children with
uncomplicated coarctation experience little or no
difficulty

135.  Dramatic complication
 peak incidence -third and fourth decades.
 Rupture originates either in a paracoarctation aneurysm
or above a coexisting bicuspid aortic valve because of an
inherent medial abnormality of the ascending aorta.
 Rupture of a postcoarctation aneurysm may be
accompanied by bleeding into the esophagus that is
announced by hematemesis and melena.
 In XO Turner’s syndrome, rupture or dissection of an
ascending aortic aneurysm occurs because of an
inherent medial abnormality, whether or not a
coexisting coarctation or a bicuspid aortic valve exists .

136.  Major complication of coarctation of the aorta
 More susceptible site is a coexisting bicuspid
aortic valve.
 Saccular septic aneurysms are occasional
sequelae of infective endarteritis

137.  Fourth major eventuality
 Hypertension is not a necessary precondition because
cerebral complications can occur with normotensive
conditions long after successful repair.
 An aneurysm of the circle of Willis-chief offender and
sets the stage for rupture and cerebral hemorrhage.
 Less common are aneurysms in other cerebral
arteries.
 Infective endocarditis on a bicuspid aortic valve can
give rise to septic cerebral aneurysms that rupture.

139.  Typical mobile dome-shaped pulmonary valve
stenosis
 relatively common, with a prevalence rate as high
as 10% of cases of congenital heart disease.
 Gender distribution is equal
 Also the case in dysplastic pulmonary valve
stenosis.

140.  Neonates with pinpoint pulmonary valve stenosis
experience rapidly progressive cardiac failure and
early death.
 mobile domeshaped- experience little or no difficulty
in infancy and childhood.
 In a review of 69 cases,
 Average age at death was 26 years;
 Seven patients survived to age 50 years
 three survived to 70 and 75 years.
 In 21 adults, the average follow-up period was 50
years.

141. Longevity depends on three variables:
1. the initial severity of stenosis;
2. whether a given degree of stenosis remains
constant or progresses;
3.whether the function of the after loaded
right ventricle is preserved.

142.  Normal pulmonary valve orifice
 increases linearly with age and body surface area.
 Orifice of a stenotic mobile dome-shaped pulmonary valve
increases with age, but not necessarily at the rate of
somatic growth.
 Mild pulmonary valve stenosis in infancy usually remains
mild
 Moderate to severe pulmonary stenosis tends to progress.
 Stenosis of the pulmonary artery and its branches is not
progressive.
 Fibrous thickening and occasionally calcification
-responsible for increasing the degree of stenosis in older
adults.

143.  Dyspnea and fatigue- mild as long as the right
ventricle maintains a normal stroke volume at rest
and augments its stroke volume with exercise.
 Relatively asymptomatic patients can have rapid
deterioration.
 Cardiac output is inadequate even at rest when
the hemodynamic burden imposed on the right
ventricle leads to right ventricular failure, which is
the commonest cause of death.

144.  Giddiness and light-headedness with effort prefigure
syncope.
 Children and adults occasionally experience the chest
pain of right ventricular myocardial ischemia.
 Sudden death has been associated with right
ventricular infarction and an abnormal right coronary
artery.
 Dilated thin-walled intrapulmonary artery aneurysms
distal to the stenoses of pulmonary artery
branchesare sources of hemoptyses that can be
intermittent and mild or recurrent and brisk.

145.  Mobile dome-shaped pulmonary valve stenosis
 substrate for infective endocarditis that can
induce a medical valvotomy when tissue is
interrupted and orifice size increases.
 Jet lesions in pulmonary artery stenosis can serve
as substrates for infective endarteritis

147.  Ranges from intrauterine death to asymptomatic
survival to late adulthood.
 The most common presentations are
 detection of the anomaly in a routine fetal
echocardiogram;
 neonatal cyanosis
 heart failure in infancy
 murmur in childhood
 arrhythmias in adolescents and adults.

148.  In utero, severe EA may lead to cardiomegaly,
hydrops and tachyarrhythmias.
 EA is a common lesion referred for foetal
echocardiography by the obstetrician.
 The intrauterine mortality rate is high in the
severe forms of EA.

149. Neonates with Ebstein’s anomaly,
 20% to 40% do not survive 1 month
 < 50% survive to 5 years.
 Neonates not only have high mortality rates but
also a significant ongoing risk of morbidity and
death.
 Neonatal right-to-left interatrial shunt disappears
as pulmonary vascular resistance normalizes.
 Shunt subsequently reappears as filling pressure
rises in the functionally abnormal right ventricle.

150.  subjects <2 years old at presentation, a
haemodynamic problem is more common than in
older patients
 In subjects >10 years old at presentation, an
electrophysiological problem is more common
than in younger.

152.  Symptomatic children with EA may have
progressive right heart failure, but most will reach
adolescence and adulthood.
 In adulthood, patients usually present with
arrhythmias, progressive cyanosis, decreasing
exercise tolerance or right heart failure.
 In the presence of an interatrial communication,
the risk of paradoxical embolisation, brain abscess
and sudden death is increased

153.  Celermajer et al. reviewed 220 cases with 1–34
years’ follow-up.
 Actuarial survival for all live-born patients was
 67% at 1 year
 59% at 10 year
 Predictors of death were
 echocardiographic grade of severity at
presentation
 foetal presentation
 right ventricular outflow tract obstruction

154.  Tachyarrhythmic sudden death -responsible for
the decline in survival rate in the fifth decade.
 WPW syndrome
 healthy individuals carries an estimated sudden
cardiac death risk of 0.02%,
 Ebstein’s anomaly-atrial flutter or fibrillation with
accelerated conduction is accompanied by a major
increase in the risk of sudden death.

155.  Pregnancy incurs the risks inherent in a functionally
inadequate volume overloaded right ventricle that
copes poorly with the additional hemodynamic
burden of gestation.
 Paroxysmal atrial tachyarrhythmias -potential
hazards especially the rapid rates associated with
accessory pathways.
 Cyanosis may first become manifest during pregnancy
because of a rise in filling pressure in the volume-
overloaded right ventricle.
 Hypoxemia increases the risk of fetal wastage
 Right-to-left interatrial shunt incurs a puerperal risk
of paradoxical embolization

156.  The male: female ratio is approximately 1.5:1.
 Symptoms and clinical course depend chiefly on
the presence and degree of coexisting
malformations.
 longevity principally hinges on the vulnerability of
the sub aortic morphologic right ventricle, even
with no coexisting malformations.

157.  Infant mortality is related to congestive heart
failure.
 Survival is then relatively constant, with an
attrition rate of approximately 1% to 2% year.

158.  Young patients with CCTGA-often overlooked
because symptoms are absent and clinical signs
are subtle.
 The diagnosis may come
 abnormalities in an x-ray or an electrocardiogram
 symptomatic complete heart block .
 age-related risk of development of complete heart
block is about 2% per year

159.  Left atrioventricular valve regurgitation is closely
coupled to long-term survival.
 Regurgitation is usually occult in infants, so late
appearance prompts a mistaken diagnosis of
acquired mitral regurgitation.
 Survival to the sixth or seventh decade is
infrequent

160.  In isolated CCTGA – CARDAIC FAILURE
 failure of subaortic right ventricle is uncommon
 May occur during pregnancy in previously
asymptomatic women.
 Myocardial perfusion defects are prevalent.
 Angina pectoris is attributed to a supply-demand
imbalance between a thick-walled systemic right
ventricle and its blood supply from a morphologic
right coronary artery.

161.  nonrestrictive VSD +CCTGA -clinical course analogous
to a VSD of analogous size in normally formed hearts.
 Pulmonary stenosis exerts a protective effect by
curtailing excessive pulmonary blood flow.
 Inverted subpulmonary left ventricle adapts to the
systemic systolic pressure incurred by a nonrestrictive
VSD.
 Isolated pulmonary stenosis varies from mild to
severe and has a clinical course analogous to
equivalent pulmonary stenosis in hearts without
ventricular inversion

162.  Cyanosis begins as early as the first day of life in
more than 90% of infants with an intact IVS.
 Severe pulmonary stenosis or atresia results in
intense neonatal cyanosis.
 Mild cyanosis with delayed onset is a feature of
D-TGA +nonrestrictive VSD or PDA.
 Large isolated ductus -associated with severe CCF
in the first few days of life, and spontaneous
closure results in a sudden fall in systemic arterial
oxygen saturation, rapid clinical deterioration, and
death.

163.  Neonatal survival - tightly coupled to the delicate
interplay between the inter circulatory
communications and pulmonary bloodflow.
 An older infant depends for survival on adequate
pulmonary blood flow.
 MORTALITY:
 30% in the first week,
 50% in the first month,
 90% in the first year.

164.  Survival is poorest
 when the foramen ovale is restrictive,
 the ventricular septum is intact
 ductus is closed.
 balloon septostomy results in;
 75% of patients survive for 6 months,
 65% survive for 1 year,
 many survive into their teens.
 A nonrestrictive VSD with pulmonary vascular disease
carries a 6-month survival rate of about 30% and a 1-
year survival rate of about 20%.

165.  Moderate pulmonary stenosis improves longevity
by regulating pulmonaryblood flow,
with 75% surviving for a year or more.
 Most of those who reach their teens have a
nonrestrictive VSD with pulmonary vascular
disease or pulmonary stenosis.

168.  The murmur of VSD dates from birth
 Flow from the left ventricle through the defect is
obligatory and does not await the neonatal fall in
pulmonary vascular resistance.
 Increased pulmonary blood flow results in volume
overload of the left ventricle, congestive heart
failure, and poor growth and development.

169.  Cyanosis is mild or absent because left ventricular
blood preferentially enters the aorta and right
ventricular blood preferentially enters the
pulmonary artery
 A rise in PVR curtails pulmonary blood flow and
relieves the left ventricular of volume overload.
 Patients occasionally reach young adulthood.

170.  The clinical manifestations closely resemble
Fallot’s tetralogy.

171.  DORV+sub pulmonary VSD
 resemble complete transposition of the great
arteries with a nonrestrictive ventricular septal
defect

172.  Cyanosis dates from birth or early infancy because
flow from right ventricle into aorta is obligatory.
 As neonatal pulmonary vascular resistance falls,
left ventricular blood preferentially enters the
pulmonary trunk across the subpulmonary VSD.
 Systemic arterial oxygen saturation is relatively
high but at the price of increased pulmonary blood
flow, left ventricular volume overload, and
congestive heart failure.

173.  The clinical course is especially poor when
coarctation of the aorta elevates systemic systolic
pressure, which augments already abundant
pulmonary blood flow by diverting still more right
ventricular blood into the pulmonary trunk .
 A rise in pulmonary vascular resistance
occasionally regulates pulmonary blood flow and
ameliorates congestive heart failure.
 Cyanosis increases, but longevity improves; and an
occasaional patient reaches the second, third, or
fourth decade.

175.  Equal frequency in males and females.
 Truncus arteriosis has been reported with
chromosome 22q11 deletion, trisomy 18,and
trisomy 21.

176.  Comes to attention in the first few weeks of life.
 Tachypnea, diaphoresis, poor feeding, and failure to
thrive.
 As pulmonary resistance falls, pulmonary blood flow
increases and neonatal cyanosis diminishes or may
virtually vanish.
 Truncal valve regurgitation is responsible for
biventricular failure because regurgitant flow is
received by both ventricles.
 A systolic murmur awaits the fall in neonatal
pulmonary vascular resistance analogous to the time
course with VSD.

177.  seldom reach their first birthday.
 Most die of congestive heart failure in the first few
months of life.
 In van Praagh and van Praagh’s necropsy series,
 mean age at death was 5 weeks.
 A rise in PVR occasionally regulates pulmonary
blood flow and relieves the volume-overloaded
left ventricle, so symptoms of congestive heart
failure improve while cyanosis deepens.

178.  A small but not insignificant number of patients
reach their third, fourth, or fifth decade with an
occasional survival into the sixth or seventh
decade.
 Rarely, death is from sudden intramural
dissection and rupture of the common trunk.
 Morbidity and mortality are also influenced by a
host of noncardiac abnormalities that coexist.

179.  depends on the specific morphology of the defect,
 the size of the ventricular septal defect
 degree of ventricular hypoplasia,
 degree of atrioventricular valve regurgitation
 presence or absence of left ventricular outflow
tract obstruction
 presence or absence of coarctation of aorta, and
associated syndromes (cardiac and noncardiac).

180.  Patients with the complete form of AVSD and
large VSD not undergoing repair die in infancy
with congestive heart failure and pulmonary
artery hypertension.
 Those who survive without surgery into childhood
usually develop pulmonary vascular obstruction
and eventually die with Eisenmenger’s syndrome.

181.  Berger and his colleagues found that in complete
AVSD
 only 54% were alive at 6 months of age,
 35% at 12 months
 15% at 24 months
 4% at 5 years.
 These data alone would support surgical
intervention in the first 3–6 months of age.

182.  Prognosis better when compared to complete
AVCD especially with minimal LAVR(left AV
regurgitation).