Skip to main content.

Pulmonary Atresia

What is it?

Pulmonary atresia (pronounced pull–mon–airy–ah–tree–sha) is a rare birth defect in which the pulmonary valve, the main door that allows blood to flow to the lungs, is missing or blocked.

Because the valve isn't working, blood cannot be pumped to the lungs from the right ventricle As a result, the right ventricle does not grow the way it should.

There are two different types of pulmonary atresia:

  1. One type has a hole in the wall between the lower two chambers of the heart. It is called pulmonary atresia with a ventricular septal defect.
  2. The other type does not have the hole in the heart. It is called pulmonary atresia with intact ventricular septum.

How is it treated?

Surgery is needed. If the right ventricle is too small, the Fontan procedure is necessary. Pulmonary atresia with intact ventricular septum is a single ventricle heart defect as there is only one pumping chamber in the heart.

These defects are among the most complex congenital heart problems known.