VENTRICULAR SEPTAL DEFECT

A VSD is an abnormal communication between the right and left ventricles. It is the most
common type of congenital heart defect, accounting for approximately 25% of all CHDs. VSDs
vary in the size (small and restrictive to large and nonrestrictive defect), number (single versus
multiple), and type (perimembranous or muscular).
Pathophysiology and Etiology

• Blood flows from the high-pressure left ventricle across the VSD into the low-pressure
right ventricle and into the PA, resulting in pulmonary overcirculation.
• A left-to-right shunt because of a VSD results in increased right ventricular pressure and
increased PA pressure.
• The increased pulmonary venous return to the left side of the heart results in left atrial
dilation.
• Long-standing pulmonary overcirculation causes a change in the pulmonary arterial bed,
leading to increased pulmonary vascular resistance. High pulmonary vascular resistance
(PVR) can reverse the blood flow pattern that leads to a right-to-left shunt across the
VSD (Eisenmenger's syndrome), resulting in cyanosis. Once this develops, the child is no
longer a candidate for surgical repair.

Clinical Manifestations

• Small VSD usually asymptomatic; high spontaneous closure rate during the first year of
life.
• Large VSDs.
o CHF: tachypnea, tachycardia, excessive sweating associated with feeding,
hepatomegaly.
o Frequent URIs.
o Poor weight gain, failure to thrive.
o Feeding difficulties.
o Decreased exercise tolerance.

Diagnostic Evaluation

• Auscultation: harsh systolic regurgitant murmur heard best at the lower left sternal border
(LLSB); systolic thrill felt at LLSB, narrowly split S2.
• Chest X-ray: varies; normal or cardiomegaly and increased pulmonary vascular
markings. Pulmonary vascular markings are directly proportionate to the amount of left-
to-right shunting.
• ECG: varies; normal to biventricular hypertrophy.
• Two-dimensional echocardiogram with Doppler study and color flow mapping to identify
the size, number, and sites of the defects, estimate pulmonary artery pressure, and
identify associated lesions.
• Cardiac catheterization usually not needed for initial diagnosis; may be needed to
calculate the size of the shunt or to assess PVR. May be performed if defect can be closed
using a ventricular occlusion device (device can be used only in muscular defects).
Management
Small VSD

• Medical management:
o Usually no anticongestive therapy is needed.
o Infective endocarditis prophylaxis for 6 months after surgical implantation of a
ventricular occlusion device.
• Cardiac catheterization for placement of a ventricular occlusion device for muscular
defects (for Qp:Qs > 2:1).
• Surgical intervention is usually not necessary.

Moderate to Large VSD

• Medical Management:
o CHF management: digoxin and diuretics (furosemide, spironolactone) and
afterload reduction.
o Avoid oxygen; oxygen is a potent pulmonary vasodilator and will increase blood
flow into the PA.
o Increase caloric intake: fortify formula or breast milk to make 24 to 30 cal/oz
formula; supplemental nasogastric feeds as needed.
o Infective endocarditis prophylaxis for 6 months after surgery/ventricular device
occluder.
• Cardiac catheterization for placement of a ventricular occlusion device for muscular
defects (for Qp:Qs > 2:1).
• Refer for surgical intervention.
o Usually repaired before age 1.
o One-stage approach: preferred surgical plan; patch closure of VSD.
o Two-stage approach: first surgery is to band the PA to restrict pulmonary blood
flow; second surgery is to patch close the VSD and remove the PA band.

Long-Term Follow-Up

• Monitor ventricular function.

• Monitor for subaortic membrane and double-chamber RV.

Complications

• CHF.
• Frequent URIs.
• Failure to thrive; poor weight gain.
• Infective endocarditis.
• Eisenmenger's syndrome.
• Pulmonary hypertension.
• Aortic insufficiency.