Back to Cardiovascular Diseases
Tetralogy of Fallot
It is a congenital disease of the heart that is composed of several
abnormalities. It is the commonest cyanotic heart disease.
1. Pulmonary stenosis (tight pulmonary valve). This
leads to obstruction to the right ventricular outflow.
2. Ventricular septal defect
3. The aorta is overriding the septum (straddle).
4. Right ventricular enlargement.
Hemodynamically the heart is one chamber.
The severity of obstruction to right ventricular
outflow is of fundamental significance. When the obstruction is severe,
the pulmonary blood flow is reduced markedly, and a large volume of deoxygenated
systemic venous blood is shunted from right to left across the ventricular
septal defect. Severe cyanosis (blue coloration of the skin) and erythrocytosis
occur, and symptoms and sequelae of decreased oxygen are prominent. In
many infants and children the obstruction is mild but progressive.
Radiologic examination characteristically reveals
a normal-sized, boot-shaped heart (coeur en sabot) with prominence of
the right ventricle and a concavity in the region of the pulmonary conus.
The pulmonary vascular markings are typically diminished, and the aortic
arch and knob may be on the right side.
1. Echocardiography: easily
diagnosis the condition.
1. Total surgical correction
Patients are usually diagnosed and treated during
childhood. Total surgical correction is ideal.
The size of the pulmonary arteries rather than the
age or size of the infant or child is the most important determinant in
establishing candidacy for primary repair. Pronounced thinning of the
pulmonary arteries is a relative contraindication for an early corrective
2. Shunt procedure
When pronounced thinning of the pulmonary
artery is present, a palliative operation,
such as creation of a systemic arterial-pulmonary arterial shunt, is carried
out and is usually followed by complete correction, which can be carried
out at a lower risk later in childhood.
Are you a doctor or a nurse?
Do you want to join the Doctors Lounge online medical community?
Participate in editorial activities (publish, peer review, edit) and
give a helping hand to the largest online community of patients.
Click on the link below to see the requirements:
Doctors Lounge Membership