Treatment

Your baby will need urgent medical attention once pulmonary atresia symptoms develop. The choice of surgeries or procedures depends on the severity of your child's condition.

Medications

An intravenous drug called prostaglandin will prevent closure of the natural connection (ductus arteriosus) between the pulmonary artery and the aorta. This is not a permanent solution, but it can buy your doctors enough time to determine what type of surgery or procedure might be best for your child.

Procedures via catheterization

In some cases, repairs can be made via a long, thin tube (catheter) inserted into a large vein in your baby's groin and threaded up to the heart. These procedures include:

  • Radiofrequency ablation and balloon valvotomy. If your baby's pulmonary valve features fused leaflets, your doctor might apply a small amount of energy (radiofrequency ablation) or use a wire to create a small hole at the center of the valve and then use a balloon to open the leaflets.
  • Balloon atrial septostomy. A balloon can also be used to enlarge the natural hole (foramen ovale) in the wall between the upper two chambers of the heart. This hole usually closes shortly after birth. Enlarging it increases the amount of blood available to travel to the lungs.
  • Stent placement. Your baby's doctor may place a rigid tube (stent) in the natural connection between the aorta and pulmonary artery (ductus arteriosus). This opening also usually closes a few days after birth. Keeping it open allows blood to travel to the lungs.

Heart surgery

The type of surgical repair needed will depend on the size of your child's right ventricle and pulmonary artery. Babies with pulmonary atresia often require a series of heart operations over time. Some examples include:

  • Shunting. Creating a bypass (shunt) from the main blood vessel leading out of the heart (aorta) to the pulmonary arteries allows for adequate blood flow to the lungs. However, babies usually outgrow this shunt within a few months.
  • Glenn procedure. In this surgery, one of the large veins that normally returns blood to the heart is connected directly to the pulmonary artery instead. Another large vein continues to provide blood to the right side of the heart, which pumps it through the surgically repaired pulmonary valve. This can help the right ventricle grow larger.
  • Fontan procedure. If the right ventricle remains too small to be useful, surgeons may use a Fontan procedure to create a pathway that allows most, if not all, of the blood coming to the heart to flow directly into the pulmonary artery.
  • Heart transplant. In some cases, the heart is too damaged to repair and a heart transplant may be necessary.
Jan. 31, 2016
References
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