Cardiology , adult congenital heart disease,

1. VSD
SHABNAM.MOHAMMADZADEH, MD
FELLOW OF ADULT CONGENITAL HEART DISEASE

2. A: STRAIGHT HEART TUBE
B:BULGING
C: C- LOOP
a
b c d e
RV
LV
O
RV
LV
A
O
RV LV
A
O
RV LV
A

3. WHICH ONE IS INCORRECT
1. Prevalence of VSD s is not race dependent
2. A restrictive VSD may close spontaneously during adult life
3. Late development of subaortic stenosis are well described
4. A moderately restrictive VSD leads to left atrial and ventricular dilation and dysfunction as well
as a variable increase in pulmonary vascular resistance

4. PREVALENCE, ETIOLOGY
 Most common congenital heart defect. accounting for up to 40 % of cardiac anomalies .
 Chromosomal disorders associated with an increased incidence of VSD , (Down syndrome),
(Di George syndrome), (Turner syndrome).
 Familial forms , TBX5, GATA4, and NKX2.5 mutations .
 Children from an adult with a VSD that is not associated with a genetic disorder may have a
risk of VSD as high as 3 % if the father is affected and a 6 % risk if the mother is affected

6. TYPES OF VENTRICULAR SEPTAL DEFECTS
TYPE 1 :( 1 ) subarterial valve; defect: in the left
ventricular outflow tract just below the aortic valve
(conal,subpul, infundibular, supracristal, doubly
commited) ( 4 ) outlet
TYPE 2 :( 2 ) perimembranous defect, membranous
septum ( yellow , white , and blue dashed
circumference for outlet, trabecular and inlet subtype,
)
TYPE3 :( 3 ) inlet or atrioventricular defect, which lies
inferior to the septal leaflet of the tricuspid valve; and (
5 ) inlet and apical
TYPE 4 : ( 6 ) muscular defects, which are entirely
bounded by the muscular septum and are often
multiple
6
5
2
1
3
4

7. TYPE 1
 Conal,subpul, infundibular, supracristal, doubly commited ( 4 ) outlet
 Aortic regurgitation (87% IN 20Y)
 Prolapse of the anterior aortic valve leaflet. ( LCC,RCC
 6 % of defects 30% in Asian
 Spontaneous closure of this type of defect is uncommon
 Doubly committed subarterial :
 More common in Asian patients,
 In the outlet septum,
 Bordered by fibrous continuity of the aortic and pulmonary valves.

8. TYPE 2
 Synonyms: perimembranous, paramembranous,conoventricular
 SUBTYPES : Inlet, trabecular, outlet, and confluent. (multiple areas of the septum)
 Most common VSD, (80 % of defects)
 Bordered by fibrous continuity between the leaflets of an AV valve and
an arterial valve.
 AI (Prolapse of,RCC,NCC)

9. TYPE 3
 Synonyms: inlet, AV canal type, endocardial cushion
 May be associated with AV canal defect.
 Trisomy 21 syndrome.
 5–8 % of VSDs .

10. TYPE 4(MUSCULAR)
 Rim totally composed of septal muscle
 Subclassified as inlet, trabecular outlet, or confluent .
 20 % of VSDs in infants
 Spontaneous closure is common,.
 Frequently multiple.
 “Swiss-cheese” septum

11. GERBODE-TYPE VSD
• Left ventricle to right atrium.
• Extremely rare

12. OTHER ANOMALIES
 Ven septum psudoaneurysm
 BAV
 Sub AO stenosis
 Caoarctation
 Pulmonary Valve stenosis
 DCRV
 AV valve malformations

13. CASE 1
 22 y/o male
 DOE FCII
 Harsh systolic murmur ,LSB
 Load P2
 Diastolic rumble on apex

14. PATHOPHYSIOLOGY
 A restrictive VSD
1. Produces a significant pressure gradient between the left ventricle and the right ventricle
2. Pulmonary-to-aortic systolic pressure ratio < 0.3
3. Small (≤1.4 : 1) shunt.
4. Less than 5mm, or defect size <=25% of annulus diameter
5. Normal PA and branches
6. Normal LV, LA size

15. PATHOPHYSIOLOGY
 A moderately restrictive VSD
1. Qp/Qs of 1.4 to 2.2 :
2. pulmonary-to-aortic systolic pressure ratio less than 0.66.
3. Diameter of defect >25% <75% of annulus size or 5-10 mm
4. RVP,PAP normal or near normal
5. Mild to moderate PA,LA,LV dilation

16. PATHOPHYSIOLOGY
 A large or non-restrictive VSD
1. Qp/Qs > 2.2
2. pulmonary-to-aortic systolic pressure ratio greater than 0.66.
3. Defect diameter >75% of aortic diameter
4. PH in less than 2years

17. PATHOPHYSIOLOGY
An Eisenmenger VSD
 PAP/systolic pressure ratio of 1
 Qp/Qs less than 1 : 1
 Net right-to-left shunt.

18. NATURAL HISTORY
1. A restrictive VSD may close spontaneously during childhood and sometimes in adult life.
2. A perimembranous defect ,doubly committed VSD,
1. Progressive AR.
2. Subaortic and subpulmonary stenosis
3. Left ventricular to right atrial shunt
3. A moderately restrictive VSD
1. Left atrial and ventricular dilation and dysfunction
2. Variable increase in pulmonary vascular resistance.
4. A large or nonrestrictive VSD
1. Ventricular volume overload early in life
2. Progressive rise in pulmonary artery pressure
3. A fall in left-to-right shunting.

19. WHICH ONE IS INCORRECT?
1. Infants with large nonrestrictive defects tend to present later than restrictive ones
2. Diastolic murmur of MV in neonates with large VSD may occur without any other anatomical
disorder
3. Cyanosis is rare in early childhood,
4. Medical management of the symptomatic infant is directed at improving symptoms before
surgery
5. Most adult patients with a small restrictive VSD are asymptomatic

20. CLINICAL FEATURE IN NEONATE
 Restrictive defect, the murmur becomes apparent only as the pulmonary vascular resistance
falls.
 Large nonrestrictive defects tend to present later. breathlessness, congestive heart failure, and
failure to thrive in the second and third months a pulmonary ejection murmur and a mitral
rumble
 Cyanosis is rare in early childhood, and if it is present, other causes of a raised pulmonary
vascular resistance should be excluded (e.g., mitral stenosis or coexisting lung disease).
 Medical management of the symptomatic infant : diuretics , ACE inhibition

21. Harrison’s grooves caused by the thoracic
retractions of chronic dyspnea

22. CLINICAL FEATURE IN ADULT
 Small restrictive VSD :
 Asymptomatic.
 Harsh or high-frequency pansystolic murmur, usually grade 3 to 4/6, maximal intensity at the left sternal border
in the third or fourth intercostal space.
 Moderately restrictive VSD
 Dyspnea in adult life, perhaps triggered by atrial fibrillation
 Displaced cardiac apex with a similar pansystolic murmur as well
 An apical diastolic rumble
 Third heart sound at the apex from the increased flow through the mitral valve.
 Large nonrestrictive Eisenmenger VSDs
 Central cyanosis and clubbing of the nailbeds
 Right ventricular heave, a palpable and loud P2, and a right-sided S4. A
 Pulmonary ejection click, a soft and scratchy systolic ejection murmur,
 High-pitched decrescendo diastolic murmur of pulmonary regurgitation (Graham Steell).
 Peripheral edema usually reflects right-sided heart failure.

23. MODERATELY RESTRICTIVE
1. LA enlargement
2. LV volume
overload

24. ECG
 Mirrors the size of the shunt and the degree of pulmonary hypertension.
 Small, restrictive VSDs: normal tracing.
 Moderate-sized VSDs :left atrial overload ,left ventricular volume overload, deep Q and tall R
waves with tall T waves in leads V5 and V6 and perhaps eventually atrial fibrillation.
 After repair, : usually normal with right bundle branch block.

25. NONRESTRICTIVE,PH
1. RVH
2. LV volume overload

26. NONRESTRECTIVE ,EISENMENGER
RVH

27. 1
2
3

29. R
LN
AVS
MS
TS DC

36. QP/QS
 Diameter of RVOT:23
 RVOT VTI: 24
 Diameter of LVOT: 20
 LVOT VTI: 21

38. SUBPULMONIC VSD

39. INLET TYPE VSD

40. AVSD

41. PERIMEMBRANOUS VSD

42. MUSCULAR VSD

45. CARDIAC CATHETERIZATION.
1. Hemodynamic signicance of a VSD is questioned
2. Assessment of pulmonary artery pressures and resistances.

49. CHAMBER SATURATION% PRESSURE(mmH
g)
IVC 78
HSVC 65
LSVC 67
HRA 67 6
MRA 70
LRA 71
P RV 80 100
DRV 83 100
MPA 84 100
PCWP 12
LV 98 120
AO 98 120

55. INDICATIONS FOR INTERVENTION
1. The presence of a significantVSD
1. The symptomatic patient shows a Qp/Qs > 1.5 : 1,
2. Pulmonary artery systolic pressure > 50 mm Hg,
3. Increased left ventricular and left atrial size, or deteriorating left ventricular function
in the absence of irreversible pulmonary hypertension.
2. Presence of a perimembranous or outlet VSD with more than mild aortic regurgitation
3. History of recurrent endocarditis.
4. In children, a nonrestrictive VSD and a smaller VSD with significant symptoms failing to respond
to medication.

56.  Elective surgery is usually performed between 3 and 9 months of age.
 PVR< 7 Wood units, closure :safely undertaken if there is a net left-to-right shunt of at least 1.5 :
1 or
strong evidence of pulmonary reactivity on challenge with a pulmonary vasodilator (oxygen,
nitric oxide).

57. REPRODUCTIVE ISSUES
• well tolerated :small or moderate VSDs and in women with repaired
VSDs.
• Pregnancy is contraindicated in Eisenmenger syndrome ,high
maternal (!50%) and fetal (!60%) mortality.

58. FOLLOW-UP
• Good to excellent functional class and good left ventricular function before surgical closure,
life expectancy after surgical correction is close to normal.
• The risk of progressive aortic regurgitation is reduced after surgery,
as is the risk of endocarditis, unless a residual VSD persists.
• Yearly cardiac evaluation :
• with right ventricular outflow tract obstruction,
• left ventricular outflow tract obstruction,
• aortic regurgitation not undergoing surgical repair;
• patients with Eisenmenger syndrome
• adults with significant atrial or ventricular arrhythmias.
• late repair of moderate or large defects, which are often associated with left ventricular
impairment and elevated pulmonary artery pressure at the time of surgery.