Ventricular Septal Defect

Most common cardiac malformation

Accounts for 25% of congenital heart disease.
Any portion of the ventricular septum1. membranous type. -VSDs between the crista supraventricularis and the papillary
muscle of the conus may be associated with pulmonary stenosis and the other
manifestations of tetralogy of Fallot VSDs
2. supracristal - superior to the crista supraventricularis (supracristal) are found
just beneath the pulmonary valve and may impinge on an aortic sinus, causing
aortic insufficiency.
3. muscular -- midportion or apical region of the ventricular septum are muscular
in type and may be single or multiple (Swiss cheese septum).
PATHOPHYSIOLOGY.
Severity depends on
1. size of the VSD decides volume of the left-to-right shunt.
2. level of pulmonary vascular resistance compared with systemic vascular resistance.
SIZE Small
When a small communication is present (usually <0.5 sq.cm ), the VSD is called
restrictive and right ventricular pressure is normal. The higher pressure in the left ventricle
drives the shunt left to right
Large
In large VSDs (usually >1.0 sq.cm), right and left ventricular pressures are equal. The
direction of shunting and the shunt magnitude are determined by the ratio of pulmonary to
systemic vascular resistances
After birth in large VSD, the pulmonary vascular resistance may remain higher than normal,
and thus the size of the left-to-right shunt may initially be limited. As pulmonary vascular
resistance continues to fall in the 1st few weeks after birth because of the normal involution of
the media of small pulmonary arterioles, the size of the left-to-right shunt increases.
Eventually, a large left-to-right shunt ensues, and clinical symptoms become apparent.
In some infants with a large VSD, pulmonary arteriolar medial thickness never
decreases. Pulmonary vascular hypertension develops. When the ratio of pulmonary to
systemic resistance approaches 1:1, the shunt becomes bidirectional, signs of heart failure
decreases, and the patient becomes cyanotic (Eisenmenger physiology
Clinical manifestations.
Depends on
1. the size of the defect
2. the pulmonary blood flow and pulmonary pressure
Small VSD Most common
Minimal left-to-right shunts
Normal pulmonary arterial pressures
Asymptomatic

The cardiac lesion found during a routine physical examination.

Loud, harsh, or blowing pan systolic murmur heard best over the lower left sternal
border and frequently accompanied by a thrill.
Large VSD
There is Large pulmonary blood flow
Pulmonary hypertension
Findings Symptoms
1. Dyspnea,
2. feeding difficulties,
3. poor growth,
4. Head sweating,
5. recurrent pulmonary infections,
6. cardiac failure in early infancy.
Signs
1. Cyanosis is usually absent, -sometimes noted during infections or crying.
2. Prominence of the left precordium is common, as are a palpable parasternal lift, a
laterally displaced apical impulse and apical thrust, and a systolic thrill.
3. The pan systolic murmur of blowing in nature because of the absence of a significant
pressure gradient across the defect.
4. The second sound in pulmonary area may be increased, indicating pulmonary
hypertension.
5. The presence of a mid-diastolic, low-pitched rumble at the apex is caused by increased
blood flow across the mitral valve and indicates a Qp:Qs of 2:1 or greater.
6. This murmur is best appreciated with the bell of the stethoscope.
LABORATORY DIAGNOSIS.
Small VSD1. Chest radiograph is normal,
2. Electrocardiogram is usually normal The presence of right ventricular hypertrophy is a
warning that the defect is not small and that pulmonary hypertension is present or that
there is an associated lesion such as pulmonic stenosis.
3. 2D echo Large VSD
1. Chest radiograph shows gross cardiomegaly with prominence of both ventricles, the left
atrium, and pulmonary artery. The pulmonary vascular markings are increased and
frank pulmonary edema, including pleural effusions, may be present.
2. Electrocardiogram - biventricular hypertrophy; P waves may be notched or peaked.
3. Two-dimensional echocardiogram shows the position and size of the VSD.

o Small muscular defects, may be seen by color Doppler examination.

o In membranous VSD, a thin membrane (called a ventricular septal aneurysm but

consisting of tricuspid valve tissue) can partially cover the defect and limit the
volume of the left-to-right shunt.
o The echo can estimate the shunt size by examining Volume overload of the left atrium and left ventricle- extent of their
increased dimensions shows the size of the left-to-right shunt.
o Pulsed Doppler examination - pressure gradient across the defect.
We can find of right ventricular pressure and determine whether the
patient is at risk for the development of early pulmonary vascular
disease.
o Determine the presence of aortic valve insufficiency or leaflet prolapse in the
case of supracristal VSDs.
Cardiac catheterization. A good ECHO is most often sufficient
1. Oximetry shows an increase in oxygen content in the right ventricle;
2. Small, restrictive VSDs - normal right-sided heart pressures and pulmonary vascular
resistance.
3. Large, nonrestrictive VSDs - equal or near-equal pulmonary and systemic systolic
pressures.
4. Pulmonary blood flow may be two to four times systemic blood flow.
5. Eisenmenger syndrome if present, pulmonary artery systolic and diastolic pressures are
elevated, the degree of left-to-right shunting is minimal, and desaturation of blood in
the left ventricle is encountered.
6. The size, location, and number of ventricular defects are demonstrated by left
ventriculography.
7. Contrast medium passes across the defect or defects to opacify the right ventricle and
pulmonary artery.
Prognosis and complications.
natural course of a VSD depends on the size of the defect.
small defects
close spontaneously, most frequently during the 1st 2 years of life.
Small muscular VSDs are more likely to close than membranous VSDs
majority of defects that close do so before age 4 yr,
children with small defects remain asymptomatic,
One of the long-term risks for these patients is infective endocarditis.
Isolated, small, hemodynamically insignificant VSD is not an indication for surgery.
Moderate or large VSDs
1.
2.
3.
4.
5.
6.
7.

Do not close spontaneously,

Repeated respiratory infection and heart failure
Heart failure causes failure to thrive.
Pulmonary hypertension occurs due to high pulmonary blood flow.
They need surgery
development of aortic valve regurgitation, - in with supracristal VSD
acquired infundibular pulmonary stenosis, which then protects fromthe long-term
effects of pulmonary vascular disease. In these patients, the clinical picture changes

from that of a VSD with a large left-to-right shunt to a VSD with pulmonary stenosis.
The shunt may diminish in size, become balanced, or even become a net right-to-left
shunt
TREATMENT.
Small VSD
1. Parents should be reassured of the benign nature of the lesion,
2. Child should be encouraged to live a normal life, with no restrictions on physical
activity.
3. Surgical repair not recommended.
4. Protection against infective endocarditis, - primary and permanent teeth - antibiotic
prophylaxis should be provided for dental visits (including cleanings), tonsillectomy,
adenoidectomy, and other oropharyngeal surgical procedures - instrumentation of the
genitourinary and lower intestinal tracts
5. Electrocardiogram is a good for screening patients for possible pulmonary hypertension
or pulmonic stenosis -- look for right ventricular hypertrophy.
6. Echocardiography is used to screen for the development of left ventricular outflow tract
pathology (subaortic membrane or aortic regurgitation) and to confirm spontaneous
closure.
large VSD
two aims:
1. control heart failure
2. prevent the development of pulmonary vascular disease.
surgical closure can be carried out at low risk in most infants, medical management should not
be pursued in symptomatic infants after an initial unsuccessful trial. Pulmonary vascular
disease can be prevented when surgery is performed within the 1st yr of life.
Indications for surgical closure of VSD -1. Any age with large defects with failure to thrive which cannot be controlled medically
2. Infants between 6 and 12 mo of age with large defects associated with pulmonary
hypertension,
3. Older than 24 mo of age with a Qp:Qs greater than 2:1.
4. Supracristal VSD of any size because of the high risk of development of aortic valve
regurgitation
Severe pulmonary vascular disease is a contraindication to closure of a VSD.
complications are rare
1. Residual ventricular shunts requiring re-operation
2. Heart block requiring a pacemaker.
Pulmonary arterial palliative banding with repair in later childhood is for complicated cases or
very premature infants.
Surgical risks are higher for defects in the muscular septum, particularly apical defects and
multiple (Swiss cheesetype) VSD.

After surgery
1. Hyper dynamic heart becomes quiet,
2. Cardiac size decreases toward normal
3. Thrills and murmurs are abolished,
4. Pulmonary artery hypertension regresses.
5. Infant begin to thrive, Catch-up growth occurs over the next 12 yr
6. Cardiac medications are not required..
7. Systolic ejection murmurs may persist for months.