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. There are a few types of treatments for pulmonary atresia.

Medication

The doctor may give your child an IV (intravenous) medication called prostaglandin to keep the ductus arteriosus open. The ductus arteriosis is the connection between the aorta and the pulmonary artery that is present in all babies before birth. This connection usually closes shortly after birth, but when kept open with medication, it can allow blood to continue to flow to the lungs until doctors decide on a long-term solution.

Cardiac catheterization

A cardiac catheterization is often performed to determine how blood is supplied to the heart in patients with pulmonary atresia with intact ventricular septum. Using this test, doctors can tell if the blood supply to the heart is dependent upon flow directly from the right ventricle through abnormal coronary connections, called fistulae. If the blood supply is dependent upon the right ventricle, surgery is recommended. 

If the cardiac catheterization shows that the blood supply is not dependent upon the right ventricle, and the blockage is short, your doctor may recommend that the pulmonary valve is reopened in the catheterization laboratory, which involves crossing the pulmonary valve with a wire or a radiofrequency ablation catheter. 

A special balloon can then be expanded in the middle of the valve to reestablish blood flow from the right ventricle to the lungs, allowing the right ventricle to grow over time. In many cases, it can allow for a biventricular repair and may reduce the number of surgeries. In some cases, it may eliminate the need for surgery altogether. If the pulmonary valve cannot be opened in the catheterization lab, then the connection between the right ventricle and lungs may be reestablished with surgery.

Surgery

Many children with pulmonary atresia need one or more surgeries to fix the problem. These surgeries may include:

  • Right ventricular outflow reconstruction: This operation, often done shortly after birth, involves surgical reconstruction of a right ventricular outflow tract in order to allow blood to reach the lungs. Options for the reconstruction involve enlarging the connection from the right ventricle to the lungs with a patch or placement of a valved right ventricle to pulmonary artery conduit. The goal of this procedure is to achieve a biventricular repair after one or two operations. Sometimes this procedure is combined with a Blalock-Taussig shunt.
  • 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.
  • Heart transplantA careful evaluation is performed in order to decide if this is the best treatment option. Heart transplant is rarely needed for children with PA/IVS.

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