Congenital Heart Disease

Pulmonary Atresia

Signs and Symptoms
Diagnosis
Treatment

Treatment

Initial treatment is an intravenous drug called prostaglandin E-1 to keep the patent ductus arteriosus from closing and allow blood to flow between the pulmonary artery and aorta, thereby bypassing the valve and heart. But this only works temporarily. Your child also may be placed on a ventilator or oxygen to improve respiration.

Immediate surgery usually is necessary to increase blood flow to the lungs. Surgeons create a passageway by inserting a tiny piece of tubing called a shunt between the pulmonary artery and the aorta to increase flow to the lungs. Later, after the first year of life when the baby's lungs have developed more, a second operation called a Fontan procedure may be performed. It connects the right atrium, where deoxygenated blood collects from the body, directly to the pulmonary artery by creating a channel through or just outside the heart to bypass the undeveloped pulmonary valve.

Long-term treatment may include drugs to control heart rhythm disturbances and antibiotics may be needed to prevent infection during other surgery or dental procedures.

Reviewed by health care specialists at UCSF Children's Hospital.
Last updated May 8, 2007