VENTRICULAR SEPTAL DEFECT

• The ventricular septum consists of:

1. A small membranous septum and a

2. Larger muscular septum:

1. The inlet - 5% to 8%

2. Infundibular - 30%

3. Trabecular (muscular) septa - 5% to 20%

4. Perimembranous: membranous & muscular part - 70%

often with PDA and COA
 Embryology

Septum is formed by:

1. Muscular ridge of the
ventricle
2. endocardial cushion
3. conal cushion
 Embryology

• Failure of ventricular septation causes various

forms of ventricular septal defects (VSD).
• If the ventricular ridge does not enfold, the result.
is a single ventricle defect.
• A lack of contribution of endocardial or conal tissue
result’s in either a high inlet, an outlet, or a
membranous type VSD
The inlet:
 Septum from endocardial cushion tissue separates the mitral
and tricuspid valves. It is called "inlet" since it forms the area
that allows blood to enter the ventricles.
The outlet or infundibulam:
 Septum is formed by conal tissue. It is called "outlet" because it
forms the outflow part of the right ventricle, or "infundibular"
because it forms the area below the pulmonary artery.
The membranous septum:
 Is formed by the joining of the inlet and outlet septum near the
mitral and tricuspid valve. It is called "membranous" because of
the type of tissue it appears to be.
 Types
• Membranous, (70%)
– Perimembranous inlet (atrioventricular [AV] canal
type),
– Perimembranous outlet
• Muscular or Trabecular : 5% to 20%
1.anterior
2.midmuscular
3.posterior
4.apical
5.“Swiss cheese” type of multiple muscular defect
 Crista supraventricularis
• It is a muscular ridge in RV that separates tricuspid valve and
pulmonic valve
 Types of VSD
supra and infracrystal
 Epidemiology
1. Incidence of CHD is 0.8%, and this incidence increases to
2–6% for a 2nd birth.
1. Two 1st-degree relatives with CHD, the risk for a
subsequent child may reach 20–30%
2. 25 % of CHDs; commonest CHD;
3. Prevalence rate of 2.5 per 1000 live births; 1:1 M:F ratio
4. May run in families
5. Gene mutations result in ventricular septal defects - holt-
oram syndrome.
6. Chromosomal abnormalities (trisomy)
7. Part of other CHD: TOF
 Hemodynamics

1. No symptoms in infants up to 8 weeks as high pulmonary

vascular resistance prevents L to R shunt
2. LV overworks by pumping into aorta and VSD and
hypertrophies.
3. LA gets more venous return and works against high pressure
LV and hence hypertrophies.
4. RV gets shunt only during its systole transmitting the blood to
PA and hence no strain - no hypertrophy
5. More blood to pulmonary circulation and hence gradual
increase in pulmonary hypertension.
 Clinical

1. <0.5 cm2 : restrictive and right ventricular pressure is normal.

2. >1.0 cm2: non restrictive; right and left ventricular pressure is equalized; PH
deciding factor
3. Small VSD:
1. Child is asymptomatic
2. normal growth and development.
3. Pulmonary vascular congestion is minimal.
4. Minimal left ventricular hypertrophy
5. The shunt produces a harsh (loud) heart systolic murmur
6. Intensity of the P2 is normal because the PA pressure is normal.
 Large VSD
Moderate VSD:
1. There will be LVH
2. RVH is absent.
3. Pansystolic heart murmur is produced by the left-to-right
shunt.
Large VSD
1. Delayed growth and development,
2. Decreased exercise tolerance; suck-rest-suck cycle
3. Repeated pulmonary infections
4. Precardial bulge
5. CHF in early infancy
6. With long-standing VSD: pulmonary hypertension & cyanosis;
clubbing
1. Eisenmenger's syndrome:
1. overall heart size decrease
2. Because of severe PH, there is persistence of RVH and
prominent PA
3. Because the shunt is small, the loudness of the murmur
decreases,
4. The S 2 is loud and single owing to pulmonary
hypertension
2. High altitude:
1. Hypoxia (high altitude) increases pulmonary vascular
resistance and decreases the amount of left-to-right
shunt.
2. At high altitudes, children with ventricular defects
develop congestive heart failure less commonly than at
sea level
 Signs of large VSD
1. Respiratory rate is 80 to 100 breaths/minute
2. The liver edge is palpable well below the right costal margin
3. Peripheral pulses are good
4. The cardiac impulse is hyperdynamic
5. Pan systolic thrill and murmur over Lt. Para sternum;
6. Because there is no pressure gradient across the ventricular
defect, the systolic murmur is not loud
7. More flow through mitral orifice: mid diastolic rumble at the
apex
8. PH: P2 is single and loud ; Second heart sound is narrowly split
9. Wheeze due to pressure by the enlarged left atrium on the
bronchus
 Eisenmenger complex
1. Pulmonary hypertension
2. RVH
3. Accentuated P2; Pul. Incompetent murmur
4. Eisenmenger complex
1. Rt to Lt shunt
2. RVH more; LVH is less
3. There is no systolic murmur,
4. The pulmonary component of the second heart sound is
very loud,
5. The patient is mildly cyanosed.
6. Inoperability
Mild VSD
Large VSD
 CXR
1. Cardiomegaly of varying degrees is present and
involves the LA, left ventricle (LV), and sometimes
RV.

2. Pulmonary vascular markings increase.

3. In Eisenmenger's complex, the main PA and the

hilar PAs enlarge noticeably, but the peripheral lung
fields are ischemic. The heart size is usually normal
ECG:
1. With a small VSD, the ECG is normal.
2. With a moderate VSD, left ventricular hypertrophy
(LVH) and left atrial hypertrophy (LAH) may be seen.
3. With a large defect, the ECG shows biventricular
hypertrophy (BVH) with or without LAH
Echo:
1. Selected two-dimensional echo views the ventricular
septum and the site of a VSD.
2. colour-flow Doppler mapping identifies colour-flow
jets across the ventricular defect
 Natural History
1. Spontaneous closure:
1. 30-50% of small defects close spontaneously,
during the 1st 2 yr of life. majority before the
age of 4 yr
2. Small muscular VSDs are more likely to close (up
to 80%) than membranous VSDs
3. Outlet and Infundibular defects do not close
2. Child with small defect:
1. Most children with small defects remain
asymptomatic,
2. A long-term risk is infective endocarditis.
Child with moderate to large VSD:
1. May become smaller and up to 8% may close
completely.
2. More commonly, repeated episodes of respiratory
infection and heart failure
3. Failure to thrive.
4. Pulmonary hypertension
Child with supracristal VSD:
Patients with VSD are also at risk for the
development of aortic valve regurgitation, esp in
supracristal VSD.
 Medical treatment
1. CHF: digoxin and diuretics; captopril reduces after load;
spironolactone to minimize potassium loss
2. Oral iron to correct anemia
3. Diet:
1. Frequent feedings of high calorie diet;
2. Expressed breast milk or formula contains 20 calories/ounce;
can be supplemented with carbohydrate or fat to provide 30
calories/ounce
3. Corn oil can be added to EBM or formula milk given as extra
milk after breast feeding
4. Exercise as per tolerance if there is no PH
5. Dental hygiene and AB prophylaxis during extraction
1. Non surgical: umbrella device closure through catheter

2. Surgical: Indications for surgical closure:

1. By the end 1 year; Sub pulmonary defects soon after diagnosis

2. PA pressure greater than 50% of systemic pressure,

3. significant left-to-right shunt with Qp/Qs ratio > 2:1

4. Aortic valve prolapse into outlet VSD

5. Single episode of endocarditis

3. Procedure: transatrial or trans PA or ventriculotomy and patching with

synthetic or pericardial tissue.

Amplatzer Membranous VSD device
Procedure
 Eisenmenger syndrome
• Refers to any untreated congenital cardiac defects with
intracardiac communications that lead to pulmonary
hypertension, reversal of flow and cyanosis.
• The previous left-to-right shunt is converted into a right-to-
left shunt secondary to the elevated pulmonary artery
pressures
• Pulmonary hypertension is defined by a mean pulmonary
artery pressure of more than 25 mm Hg at rest or more than
30 mm Hg during exercise