Pulmonary Atresia

Treatment

At Mayo Clinic in Minnesota, doctors with training in treating children who have heart disease (pediatric cardiologists) care for children who have congenital heart disease, including pulmonary atresia with intact ventricular septum (PA/IVS). Cardiologists, cardiac surgeons and other specialists trained in treating heart conditions present at birth (congenital heart disease) coordinate your child's care.

A child who has PA/IVS requires urgent medical evaluation once symptoms develop. Treatment of PA/IVS depends on the severity of your child's condition and your family's wishes. After diagnosis of PA/IVS, a cardiologist helps you to manage your child's condition as you decide about treatment. A cardiologist may:

• Give your child a drug to prevent closure of the connection between the left and right side of the heart (ductus arteriosus)
• Place your child on a ventilator to assist with breathing
• Give your child intravenous (IV) fluids and drugs to help his or her heart beat stronger

Your child's treatment may include:

• Cardiac catheterization. In cardiac catheterization, a doctor inserts a long, thin tube (catheter) in your child's arm or groin and guides it to your child's heart using X-ray imaging. Cardiac catheterization usually is performed to evaluate your child for any heart defects or coronary abnormalities, to check whether natural connections between the upper heart chambers (foramen ovale or patent ductus arteriosus) are still open and to evaluate the amount and proportions of oxygen-rich (red) and oxygen-poor (blue) blood flowing through your child's heart.
• Radiofrequency ablation and balloon valvotomy. Your child'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 blood to flow from the lower-right chamber (right ventricle) of your heart to your lungs (pulmonary valve). Your 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 (hybrid surgery).
• Balloon atrial septostomy. In this procedure, your child's doctor inserts a tube (catheter) with a balloon in the tip through the natural connection (foramen ovale) in the wall between the left and right atria (atrial septum) and inflates the balloon. Your child's doctor may perform this procedure to improve the proportion of red blood and blue blood between the upper chambers of your child's heart (right and left atria).
• Cardiac surgery. Your child 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 or pumping chamber (right ventricle) and the artery that delivers blood to the lungs (pulmonary artery).
• Shunt placement. If your child's heart's right ventricle is smaller than usual, your child'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.
• Stent placement. In some children, your child's doctor may place a tube (stent) in one of the natural connections (patent foramen ovale or patent ductus arteriosus) between the upper heart chambers (atria) to keep the connection open and allow blood to pass through. In children with more severe conditions, your child's doctor may place a stent between the right ventricle and pulmonary artery to help blood flow to the lungs.
• Additional cardiac surgery. Your child may need additional surgery when older, particularly if your child has an extremely underdeveloped right ventricle. The type of surgery depends on the size and condition of your child's right ventricle and pulmonary artery.
• Bidirectional Glenn procedure or hemi-Fontan procedure. In this procedure, your doctor connects the blood vessel carrying blood from the body to blood vessels carrying blood to the lungs. This surgery allows most of the 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 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. Your child will need monitoring and testing before and after surgery. Your child also will need long-term monitoring by a congenital heart specialist.
  
  People who have had surgery for PA/IVS may require antibiotics before dental work and certain surgeries to prevent endocarditis, a bacterial infection of the heart's walls or valves.