Treatments and drugs

By Mayo Clinic Staff

Your baby will need urgent medical attention once pulmonary atresia symptoms develop. Treatment of pulmonary atresia depends on the severity of your child's condition. After diagnosis, a pediatric cardiologist can help you to manage your child's condition as you decide about treatment. Initial treatments after diagnosis may include:

  • Giving your baby a drug called prostaglandin E1 to prevent closure of the connection between the left and right side of the heart (ductus arteriosus)
  • Connecting your baby to a ventilator to assist with breathing
  • Giving your baby intravenous (IV) fluids and drugs to help his or her heart beat stronger

In addition to medications, your baby will need medical procedures using catheters and possibly surgery to correct pulmonary atresia.

Cardiac catheterization

In cardiac catheterization, a doctor inserts a long, thin tube (catheter) in your baby's groin and guides it to the heart using X-ray imaging. Cardiac catheterization usually is performed to check for any further heart defects or abnormalities, and to see whether natural connections between the upper heart chambers and blood vessels (foramen ovale or ductus arteriosus) are still open. Your baby's doctor can also check the amount of oxygen-rich (red) and oxygen-poor (blue) blood flowing through your child's heart. Additional procedures that can be done via catheterization include:

  • Radiofrequency ablation and balloon valvotomy. Your baby's doctor may apply a small amount of energy (radiofrequency ablation) through a catheter or use a small wire to create a small hole to open the valve that allows your baby's blood to flow from the right ventricle of the heart to the lungs (pulmonary valve). Your baby's doctor may then insert a catheter with a balloon in the tip and inflate the balloon to open the valve (balloon valvotomy), allowing blood to flow through the valve to the lungs. Your child may still need open-heart surgery, which your doctor sometimes can perform at the same time as the catheterization (hybrid surgery).
  • Balloon atrial septostomy. In this procedure, your baby's doctor inserts a tube (catheter) with a balloon in the tip through the connection (foramen ovale) in the wall between the left and right atria (atrial septum) and inflates the balloon. Your baby's doctor may perform this procedure to improve the proportion of oxygenated blood and oxygen-poor blood between the upper chambers of your child's heart (right and left atria).
  • Stent placement. Your baby's doctor may place a tube (stent) in the natural connection between the aorta and pulmonary artery (ductus arteriosus) to keep the connection open and allow blood to pass through into the lungs. If your baby's condition is severe, the doctor may place a stent between the right ventricle and pulmonary artery to help blood flow to the lungs.

Heart surgery

Your baby may need heart (cardiac) surgery within his or her first week of life, depending on the size and condition of your child's lower right heart chamber (right ventricle) and the artery that delivers blood to the lungs (pulmonary artery). Options include:

  • Shunt placement. If your baby's heart's right ventricle is smaller than it should be, your baby's doctor may place a tube (shunt) between the large artery that exits the heart (aorta) and the pulmonary artery to keep blood flowing to the lungs.

Additional heart surgery

Your baby may need additional surgery later on in life, particularly if he or she has an underdeveloped right ventricle. The type of surgery depends on the size and condition of your child's right ventricle and pulmonary artery. Later surgeries may include:

  • Bidirectional Glenn procedure or hemi-Fontan procedure. In this procedure, your doctor connects some of the blood vessel carrying blue blood from the body to blood vessels carrying blood to the lungs. This surgery allows most of the blue blood to flow directly from the body into the lungs. The heart pumps blood containing more oxygen through the aorta to supply oxygen to organs and tissues. This approach reduces the work of the right ventricle by allowing it to pump blood only to the body.
  • Fontan procedure. If the right ventricle is small and unable to pump, doctors may perform the Fontan procedure. In this surgery, doctors connect the remaining blood vessels carrying blue blood from the body to the blood vessels carrying blood to the lungs. This approach helps blood coming from the body to flow to the lungs and further reduces the work of the right ventricle.
  • Right ventricular outflow tract reconstruction. Some children may need reconstruction of the area of the right ventricle where blood exits to the pulmonary valve (right ventricular outflow tract), depending on their condition.
  • Valve repair or replacement. Some children may need a pulmonary valve repair or replacement later in life.

Follow-up care

Your child will need monitoring and testing before and after surgery. Your child will also need long-term monitoring by a congenital heart specialist, including throughout adulthood.

People who have had surgery for pulmonary atresia may require antibiotics before dental work and certain surgeries to prevent endocarditis, a bacterial infection of the heart's walls or valves.

Jan. 24, 2013