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Pulmonary atresia

  • Alternative Names

    Pulmonary atresia - intact ventricular septum; PA/IVS


    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).

    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

    Support Groups


    Expectations (prognosis)

    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.


    Complications
    • Delayed growth and development
    • Seizures
    • 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)