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Pulmonary atresia - Treatment

Alternative Names

Atresia - pulmonary; PA/IVS

Treatment:

A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs.

Other treatments include:

Interventional heart catheterization to repair the problem.
Open heart surgery to repair or replace the valve. This surgery can also be used to place a tube between the right ventricle and the pulmonary (lung) arteries.
For some patients, the surgeon may reconstruct the heart as a single ventricle.
Heart transplant

Expectations (prognosis):

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.

Complications:

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

Calling your health care provider:

Call your health care provider if the baby has:

Problems breathing
Skin or nails that appear blue (cyanosis)

Heart, section through the middle

Heart, front view

Reviewed last on: 12/10/2007
David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.; and Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of Philadelphia.

References

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.

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