Left-Right Shunt�Natural history & Principles of Management

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

4. 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.CardiolYoung 2000; 10: 464–73.

5. Pathologic anatomy of IVS

6. 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%
Benignosotoet al. Br HeartJ1980; 43: 332-343

7. Restrictive-less than one third of aortic root
LVSP > > RVSP
Pulm/Aortic systolic pressure ratio < 0.3
Qp/ Qs<1.4:1
Moderately restrictive
RVSP high, but less than LVSP
Qp/Qs 1.4:2.2
Non restrictive -The size of aortic orifice
RVSP , LVSP, PA Aortic systolic pressures are equal
Qp/Qs >2.2
Flow determined by PVR

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

9. 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

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

11. 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

12. 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.Pediatrics1965;36:763-772.

13. 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
FusterV, et al.CardiovascClin. 1980;10:161–197.

14. Occurs in both perimembranous and muscular
Closure is documented in the fetus & in the adult
VSD diagnosed in the fetus -46% closed in utero&
23.1% in the first year, while 30.8% remained patent
In this study 15.8% of defects < 3 mm remained
patent in comparison to 71.4% > 3mm at 1yr
NirA et al.PediatrCardiol1990; 11: 208–10.

15. 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. TennMed 2000; 93: 136–8.
Farina MA et al . J Pediatr1978; 93: 1065–6.
Moe DG et al. Am J Cardiol1987; 60: 674–8.

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

17. 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 Cardiol1983; 52: 341–5.
Freedom et al. Circulation 1974; 49: 375–84.

18. 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 subaorticlocation by adhesion of the prolapsed aortic valve cusp

19. 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
NadasAS et al . Circulation 1977; 56(No.2, Suppl. I): 1–87.
CoroneP et al. Circulation 1977; 55: 908–15.
PongiglioneG et al . Am J Cardiol1982; 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.

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

21. 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

22. Pathogenesis
Anatomic factors for normal competence
Leaflet support by diastolic apposition
Infundibular support from below
Intrinsic structural abnormality
Progressive discontinuity between aortic valve annulus & media

23. Pathogenesis
Hemodynamic factor
‘’Venturieffect’’
VSD is restrictive, Qp/Qs<2, absence of PAH
Komai H et al.AnnThoracSurg64:1146-1149, 1997

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

25. 362 Patients. 37 (10.2%) had AR
Mean age 13.4 years ( 2-45),male to female ratio 5:1
31 (84%) had infracristal & 6 (16%) supracristalVSD
Infracristal VSD-RCC prolapse in 14 (48%) &NCC in 12 (41%) and both
RCC and NCC in 3 (11%)
SupracristalVSD -RCC prolapse in 5 (83%),NCC in 1
Two patients the AR was due to bicuspid aortic valve
PA pressure normal in 26(70.2%), L-R shunt 1.5:1 or less in 23 (62%)
No relationship-severity of AR & location of VSD
SomanathHS et al. Indian Heart J. 1990 Mar-Apr;42(2):113-6.

26. 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
ElgamalMA et al . Ann ThoracSurg68:1350-1355, 1999
LunK et al. Am J Cardiol87:1266-1270, 2001
Gabriel HM et al. J Am CollCardiol39:1066-1071, 2002

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

28. Three major structures are responsible
Posteriorly malaligned outlet septum
Septal deviation ( anteroseptaltwist)
Muscular protrusion of the left ventricular aspect of the septum
Anterolateral muscle bundle
Muscular protrusion between LCC & AML-present normally in 40%
(Very rarely “Mitral arcade”)
Careful echo assessment of the LVOT should be
performed in all cases of VSD before surgery
MoeneRJ et al. PediatrCardiol1982;2: 107–14.

29. 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.

30. 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 4
th
et al . Am Heart J. 1987;114:115-205

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

32. 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 endocarditisare
considered at increased risk for recurrent infection so
surgical closure may be recommended
GersonyWM et al.Circulation1993; 87(Suppl. I):I-121–I-126.

33. Patients with VSD have a high incidence of arrhythmia
Ventricular tachycardiasin 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

34. Closing defect -soft S2, high frequency & shorter
murmur
Increasing PVR : increased RV pulsations ,S2 loud &
narrow split
Infundibular hypertrophy & resulting decreased L to
R shunt : S2 decreases in intensity ,crescendo-
decrescendo systolic murmur in the ULSB

35. 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

36. Right bundle branch block
33.3% undergoing transatrialrepair
78.9% to 11% in repair via a right ventricular incision
Transpulmonaryapproach 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-HesselinJW et al. EurHeart J 2004;25:1057-1062.
Abe T et al . JpnCirc J 1983; 47: 328–35.

37. Patent Ductus Arteriosus

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

39. Congestive heart failure
Infective endarteritis
Pulmonary vascular disease
Aneurysmalformation
Thromboembolism
Calcification

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

41. 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
CoshJA. Br Heart J 1957; 19: 13–22.

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

43. Described either pre-or postnatally
Likely develops in the third trimester due to abnormal
intimal cushion formation or elastinexpression
Incidence varies from 1.5% to 8.8%
DyamenahalliUet al. J Am CollCardiol2000; 36: 262–9.
Jan SLet al. J Am CollCardiol2002; 16: 342–7.

44. Complications
Thromboembolism
Dissection
Rupture
Tracheal compression
Left recurrent laryngeal nerve palsy
Pulmonary artery obstruction
Regression can occur, presumably due to thrombosis
and subsequent organization

45. As many ducts will eventually close in premature
infants approach 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.Heart1968;30:4–13.

46. Indomethacin
Rates of success varying between 18% to 89%
Major determinants of success were gestational and
postnatal age
Less chance of closure-Extreme prematurity, very low
birth weight, and advanced postnatal age
The rate of re-opening is highest in the extreme
premature( 1/3) & less than 1/10 those weighing 1500 g
Mahonyet al. N EnglJ Med 1982;306: 506-510.

47. Ibuprofen
Comparable rate of ductal closure to Indomethacin
Less effect on renal function
Increased incidence of Pulmonary hypertension
GournayVet al. Lancet 2004;364:1939-1944.

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

49. Symptomatic moderate or large PDA with L to R shunt
Prior history endarteritis [class1]
Reasonable in small L to R shunt with normal-sized
heart chambers when the PDA is audible by
auscultation[IIa]
May be considered in bidirectional PDA shunt due to
PAH and obstructive pulmonary vascular disease but
reversible to pure L to R shunting with pulmonary
vasodilators [IIb]

51. Incidence is 1 child per 1,500 live births
More frequent in females than males by about 2:1
Most ASDs occur sporadically; however, a few families
have the defect as a genetic abnormality.
Mutation in-TBX5, NKX2.5 , GATA4, Myosin heavy chain 6
SamanekM et al. PediatrCardiol1999;20:411–7

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

53. Hemodynamic/anatomic abnormalities resulting
from a secundumatrialseptal defect include
Right ventricular and atrialvolume overload
Pulmonary vascular obstructive disease
Tricuspid valve and/or pulmonary valve regurgitation
Supraventriculartachyarrhythmias

54. 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 atriallevel
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

55. 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

56. 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

57. 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,etal. Circulation 1981;64:402-409.
Murphy JG, et al. N Engl J Med. 1990;323:1645–1650

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

59. Craig and Selzerin 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
Cherianet al studied 709 pts.ofisolated 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
Eisenmengersyndrome 9%
Craig RJ et al. Circulation 1968; 37: 805–15.
CherianG et al. Am Heart J 1983; 105: 952–7.

60. 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
RadzikD, et al. J Am CollCardiol. 1993;22:851-853.

61. 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 a 5-to 6-mm defect
7% with a defect >6 mm
HelgasonH, et al. PediatrCardiol.1999;20:195-199.
Even infants with CHF can have spontaneous
reduction in the size of the ASD years after the
diagnosis
Occasionally, spontaneous closure will occur as late as
16 years.

62. 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

63. 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

66. 1.In large VSDs, the rate of decline in pulmonary
vascular resistance after birth is delayed
2.In Down syndrome the development of CHF is
delayed
3.CHF symptoms get worsen initially as pulmonary
vascular resistance increases
4.Adults may present with new symptom of exercise
intolerance

67. 1.Usually moderate sized VSDs can be managed medically
without surgical intervention during infancy
2.Intervention to close the VSD with PAH would shorter life
expectancy than if the defect were left open
3.There is enlargement of LA, LV, RV and PA in moderate sized
VSDs
4.Development of PAH may occur in as many as a quarter of
patients with large defects who undergo surgery after 2 years
of age.

68. 1.In outlet VSD 25 % will close by 2yrs
2.Isolated VSD closure rate -34% at 1 yr & 67% at 5 yr
3.Closure is rare in malaligned VSD
4.Closure more frequent in <10 yrs of age

69. 1.Rare before 2 years
2.Prevalence 2% to 7%
3.Right coronary cusp prolapse extremely rare
4.Non-coronary cusp prolapse in perimembranous
type

70. 1.Posteriorlymalaligned outlet septum
2.Septal deviation or anteroseptaltwist
3.Anterolateral muscle bundle
4.Mitral arcade

71. 1.Incidence higher in large defect
2.Incidence lower during childhood
3.Operated VSD 7.3 per 10000 person-years
4.Surgical closure may be recommended in Patients
with a proven episode of endocarditis

72. 1.2 days
2.2 weeks
3.2 months
4.6 months
5.12 months

73. 1.Anterolateralmuscle bundle present normally in 60% of
infants
2.Anteriorly malalignedoutlet septum is a major cause
3.Usually occurs below the ventricular septal defect
4.Septal deviation is a muscular protrusion of the left
ventricular aspect of the septum

74. 1.It is a shunt from the pulmonary artery to the aorta in the
fetus
2.Blood flow may take place from the aorta to the pulmonary
artery after birth, prior its closing
3.Right to left blood flow increases with the first breath after
birth due to falling pulmonary vascular resistance
4.The smooth muscle in its walls is sensitive to rising PO2

75. 1.Breathlessness
2.Failure to thrive
3.Asymptomatic murmur
1.Recurrent chest infections
1.Arrhythmias

77. 1.In large VSDs, the rate of decline in pulmonary
vascular resistance after birth is delayed
2.In Down syndrome the development of CHF is
delayed
3.CHF symptoms get worse initially as pulmonary
vascular resistance increases
4.Adults may present with new symptom of exercise
intolerance

78. 1.Usually moderate sized VSDs can be managed medically
without surgical intervention during infancy
2.Intervention to close the VSD with PAH would shorter life
expectancy than if the defect were left open
3.There is enlargement of LA, LV, RV and PA in moderate sized
VSDs.
4.Development of PAH may occur in as many as a quarter of
patients with large defects who undergo surgery after 2 years
of age.

79. 1.In outlet VSD 25 % will close by 2yrs
2.Isolated VSD closure rate -34% at 1 yr & 67% at 5 yr
3.Closure is rare in malaligned VSD
4.Closure more frequent in <10 yrs of age

80. 1.Rare before 2 years
2.Prevalence 2% to 7%
3.Right coronary cusp prolapse extremely rare
4.Non-coronary cusp prolapse in perimembranous
type

81. 1.Posteriorlymalaligned outlet septum
2.Septal deviation or anteroseptaltwist
3.Anterolateral muscle bundle
4.Mitral arcade

82. 1.Incidence higher in large defect
2.Incidence lower during childhood
3.Operated VSD 7.3 per 10000 person-years
4.Surgical closure may be recommended in Patients
with a proven episode of endocarditis

83. 1.2 days
2.2 weeks
3.2 months
4.6 months
5.12 months

84. 1.Anterolateralmuscle bundle present normally in 60% of
infants
2.Anteriorly malalignedoutlet septum is a major cause
3.Usually occurs below the ventricular septal defect
4.Septal deviation is a muscular protrusion of the left
ventricular aspect of the septum

85. 1.It is a shunt from the pulmonary artery to the aorta in the
fetus
2.Blood flow may take place from the aorta to the pulmonary
artery after birth, prior its closing
3.Right to left blood flow increases with the first breath after
birth due to falling pulmonary vascular resistance
4.The smooth muscle in its walls is sensitive to rising PO2

86. 1.Breathlessness
2.Failure to thrive
3.Asymptomatic murmur
1.Recurrent chest infections
1.Arrhythmias