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Pulmonary Atresia with Ventricular Septal Defect

What is pulmonary atresia with ventricular septal defect (PA-VSD)?

Pulmonary atresia (PA) is when the pulmonary valve did not form, and no blood can go from the right ventricle into the lungs.

A ventricular septal defect (VSD) is a hole between the two bottom chambers (ventricles) of the heart. In pulmonary atresia, this opening allows for mixing of the oxygen-rich and oxygen-poor blood between the right and left sides of the heart.

An extra blood vessel called a patent ductus arteriosus (PDA) is required for blood to be able to reach the lungs. The PDA is a normal blood vessel that connects the pulmonary (lung) artery to the aorta (large blood vessel that supplies blood to the body). During fetal development, the PDA is used to bypass the baby’s lungs as they are not used prior to birth. Typically, the PDA then closes within a day or two after birth.

For babies with pulmonary atresia, a medication called prostaglandin will be infused through an IV to keep the PDA open to provide pulmonary blood flow.

How often does it occur?

Pulmonary atresia occurs in about every 1 in 10,000 live births and occurs in the first 8 weeks of fetal development.

Causes of pulmonary atresia

The exact cause is not known, though it can be related to certain genetic conditions.

Signs and symptoms

Babies born with pulmonary atresia may have a bluish tint to their skin (cyanosis), they may breathe fast or appear to be struggling to breathe, have poor feeding or be lethargic.

Testing for PA-VSD


  • Pulse oximetry: a way to monitor the oxygen content of the blood via a light probe placed on your baby’s hand or foot. This test is not painful. It is typical for a baby with pulmonary atresia to have lower than normal blood oxygen levels.
  • Electrocardiogram (ECG): a visual representation of the heart's electrical activity captured via monitors placed on the skin. This test is not painful.
  • Echocardiogram (echo): an ultrasound of the heart that evaluates the structure and the function of the heart by using sound waves. Still and moving pictures of the heart structures, heart valves, and heart function are recorded for review by a cardiologist. This test is not painful.
  • Chest X-ray: a test that uses a small amount of radiation to create an image (or picture) of the inside of the chest, including the heart, lungs, blood vessels and bones. This test is not painful.
  • Cardiac catheterization: a procedure where a catheter (small tube) is inserted into your child’s heart through a large vein or artery in the leg to take pictures and pressure measurements. There may be some soreness at the insertion site following this procedure.

Treatment options for PA-VSD


  • Catheter based: During a cardiac catheterization, doctors can place a stent in the PDA so that blood can continue to flow to the lungs without the prostaglandins. There are certain types of pulmonary atresia where a catheter can be used to create an opening in the valve.
  • Surgical Interventions:
    • A surgical shunt can be placed to connect the pulmonary artery and aorta to act as a PDA
    • Complete repair includes closure of the ventricular septal defect and placement of a valved conduit (tube) that connects the right ventricle to the pulmonary arteries for pulmonary blood flow

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