Alternative Names
Pulmonary atresia – intact ventricular septum; PA/IVS
Definition of Pulmonary atresia
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.
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 (PDA).
Symptoms
Symptoms usually occur in the first few hours of life, although it may take up to a few days.
Signs and tests
The health care provider will use a stethoscope to listen to the heart and lungs. Persons with a PDA have a heart murmur that can be heard with a stethoscope.
Treatment
A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs. This medicine keeps a blood vessel open between the pulmonary artery and aorta. The vessel is called a patent ductus arteriosus (PDA).
Expectations (prognosis)
Most cases can be helped with surgery. However, how well a baby does depends on:
Review
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. – 12/21/2009
