Pulmonary atresia

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Heart, front view

Heart, section through the middle

Atresia - pulmonary; PA/IVS

A medicine called prostaglandin E1 may be used to help the blood move (circulate) through the body.

Other treatments include:

• Interventional heart catheterization
• Open heart surgery to repair or replace the valve
• Heart transplant

Most cases can be helped with surgery. However, how well a baby does depends on the quality of the blood vessels supplying the heart, how well the heart is beating, and the amount of leakiness of the other heart valves.

Some patients who have PA/IVS where the right ventricle is extremely small usually have several surgeris to help simulate normal circulation, but the surgeries do not exactly re-create normal circulation. A subgroup of these patients also have abnormal blood supply to the heart.

• Delayed growth and development
• Seizures due to too little oxygen in the blood
• Infectious endocarditis
• Heart failure

Call your health care provider if the baby has:

• Problems breathing
• Skin or nails that appear blue (cyanosis)

Review Date: 07/06/2006
Reviewed By: Anne J. L. Chun, M.D., Assistant Professor of Pediatrics, Division of Pediatric Cardiology, New York University School of Medicine, New York, NY.Review provided by VeriMed Healthcare Network.