Pulmonary atresia - intact ventricular septum; PA/IVS

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).

Other treatments include:

• Heart catheterization to repair the problem
• Open heart surgery to repair or replace the valve, or to place a tube between the right ventricle and the pulmonary (lung) arteries
• Reconstructing the heart as a single ventricle (for some patients)
• Heart transplant

Most cases can be helped with surgery. However, how well a baby does depends on:

• Quality of the blood vessels supplying the heart
• How well the heart is beating
• Amount of leakiness of the other heart valves

Outcomes can vary because of the different forms of this defect. The baby could have only a single catheter-based procedure, or could need three or more surgeries and have only a single working ventricle. The outcome is difficult to predict without knowing all details about the patient's condition.

• Delayed growth and development
• Seizures
• Stroke
• Infectious endocarditis
• Heart failure
• Death

Call your health care provider if the baby has:

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

Images

Heart, front view

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.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)