Pulmonary Atresia | Treatments

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How is pulmonary atresia treated?

Babies with pulmonary atresia need some type of treatment soon after birth. The specific type of treatment your baby needs will depend on the severity of his or her condition.

Treatments for pulmonary atresia include:

  • Medication: The doctor may give your child an IV (intravenous) medication called prostaglandin to keep the ductus arteriosus open. The ductus arteriosis is the prenatal connection between the aorta and the pulmonary artery. This connection usually closes shortly after birth, but when kept open, it can serve as a temporary opening for blood flow until doctors decide on a more long-term solution.
  • Cardiac catheterization: This procedure can make some small changes to a baby’s heart to help improve blood flow.
  • Surgery: Many children with pulmonary atresia need one or more surgeries to fix the problem. These surgeries may include:
    • Blalock-Taussig shunt. This operation, done when the blood flow to the lungs is inadequate, is usually performed soon after birth to create a pathway for blood to reach the lungs. A connection is made between the first artery off the aorta and the right pulmonary artery. Some of the blood traveling through the aorta towards the body will “shunt” through this connection and flow into the pulmonary artery to receive oxygen.
    • Bi-directional Glenn procedure. This operation, often done when a child is between 4 and 12 months old, replaces the Blalock-Taussig shunt (which the baby's heart will outgrow) with another connection to the pulmonary artery. During the procedure, a large vein (vena cava) that returns blood back to the heart is surgically connected to the pulmonary artery instead. This allows blood to move into the lungs to receive oxygen and can help the right ventricle grow.
    • Fontan procedure. This operation, usually done in the first few years of life, is used when the right ventricle remains undeveloped, such as in severe forms of PA/IVS. This surgery directly connects returning blood from the lower body into the pulmonary arteries, bypassing the heart.
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