Congenital Pulmonary Valve Stenosis

When the pulmonary valve, which allows the blood in the right ventricle to pass into the pulmonary artery which leads to the lungs, is narrowed, it is referred to as pulmonary valve stenosis.

  • The stenosis affects the valve in the right ventricle which allows blood to be pumped out into the pulmonary artery that carries it to the lungs.
  • A heart murmur is the most common symptom, but in some cases, cyanosis may be present, and heart failure can occur.
  • The symptoms lead to diagnosis, which is confirmed by echocardiography.
  • The valve may be opened up by balloon valvuloplasty, but surgical reconstruction may be necessary in some cases.

Mild to moderate stenosis, which is more common, causes the right ventricle to work harder to get the blood pumped into the pulmonary artery through the narrowed valve opening. The blood is pumped at higher than normal pressure too. If the valve is severely narrowed, the right ventricle can pump out almost no blood into the lungs. Pressure builds up in the right ventricle, and when it becomes too high, the deoxygenated blood gets pumped through alternate pathways such as a septal defect in the atrial wall. This results in right-to-left shunting.

A heart murmur may be the only symptom displayed by most of the children who have congenital pulmonary valve stenosis. Cyanosis may be present in a few, and heart failure may occur in some. When the children grow older, fatigue and breathing difficulties may develop on exertion. Echocardiogram can confirm the condition, but sometimes, a procedure called cardiac catheterization is done to determine the severity and extent of stenosis.

Balloon valvuloplasty can successfully enlarge a valve with moderate stenosis, but if there are structural abnormalities, reconstructive surgery may be necessary. The fetal blood vessel ductus arteriosus is kept open with the administration of a prostaglandin drug like alprostadil, till surgical correction of the pulmonary valve can be done, or a an alternate route bypassing the valve can be created. This relieves severe cyanosis occurring due to pulmonary valve stenosis. When the infant grows older, surgery may have to be repeated.

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Yasser Elnahas

MD, PHD, Professor Of CardioVascular Surgery
Dr. Yasser Elnahas, Is an associate Professor of Cardiovascular Surgery. Dr. Elnahas was trained as a fellow At Texas Heart Institute And Mayo Clinic Foundation.Dr. Elnahas is dedicated to educating the general public about different disease conditions and simplifying the medical knowledge in an easy to understand terminology.

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