Pulmonary valve atresia

Pulmonary valve atresia frequently occurs in conjunction with other congenital heart defects that largely determine the course of action. The most common association is with a ventricular septal defect.

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More information about pulmonary valve atresia without a ventrical septal defect is on the next page

When accompanied with a septal defect, pulmonary valve atresia may be diagnosed in infancy or childhood, or may not be diagnosed until adolescence or even adulthood. Treatment for these patients aims at normalizing blood circulation in the heart by closing the septal defect and connecting the right ventricle to the pulmonary artery. This involves open-heart surgery to insert a conduit with a new valve that channels blood from the right ventricle into the pulmonary artery for oxygenation in the lungs.

If the pulmonary arteries are too small, an additional operation will be needed prior to inserting the conduit. In this procedure, the surgeon uses a shunt to create a connection between the aorta, or a branch of the aorta, and the pulmonary artery. The shunt is a palliative measure that increases blood flow to the lungs, facilitating growth of the pulmonary arteries until the more permanent conduit can be surgically placed.

This section also contains information about pulmonary valve atresia without a ventricular septum defect.