Pulmonary atresia is a form of congenital heart disease in which the pulmonary valve does not form properly. The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs.
In pulmonary atresia, a solid sheet of tissue forms where the valve opening should be, and the valve remains closed. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.
Pulmonary atresia - intact ventricular septum; PA/IVS
Causes, incidence, and risk factors
As with most congenital heart diseases, there is no known cause of pulmonary atresia. The condition is associated with another type of congenital heart defect called a
Persons with pulmonary atresia may also have a poorly developed tricuspid valve. They may also have an underdeveloped right ventricle and abnormal blood vessels feeding the heart.
Pulmonary atresia may occur with or without a
Review Date: 12/21/2009
Reviewed By: Kurt R. Schumacher, MD, Pediatric Cardiology, University of Michigan Congenital Heart Center, Ann Arbor, MI. Review provided by Verimed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.