Pulmonary Atresia

Diagnosis

Pulmonary atresia is typically diagnosed shortly after birth. Newborns undergo testing to assess heart function and detect any signs of low oxygen levels. Several diagnostic tests help confirm the condition and determine its severity.

Tests that may be used include:

• Pulse oximetry, which measures the amount of oxygen in the blood using a small sensor placed on the baby’s finger. Low oxygen levels can indicate a heart or lung problem.
• Chest X-ray to show the size and shape of the baby’s heart and lungs.
• Electrocardiogram, a quick test that records the heart’s electrical activity and rhythm using small patches called electrodes placed on the chest, arms, or legs.
• Echocardiogram, the main test for pulmonary atresia, which uses sound waves to create images of the beating heart and show how blood moves through the heart structures. When done before birth, it is called a fetal echocardiogram.
• Cardiac catheterization, a procedure in which a thin tube is guided through a blood vessel to the heart. Dye is injected through the catheter so arteries show up clearly on imaging. It provides detailed information about blood flow and heart function, and certain treatments can also be done during the procedure.

Treatment

Pulmonary atresia requires emergency medical care soon after birth. Treatment is based on how severe the condition is and which heart structures are affected. Most babies need a combination of medications, catheter-based procedures, and multiple surgeries as they grow.

Medications may be given through an IV to keep the ductus arteriosus open. This temporary measure helps maintain blood flow to the lungs while healthcare professionals decide on the best long-term treatment.

Some babies may benefit from procedures done with a catheter guided through a major blood vessel to the heart. These options include:

• Balloon atrial septostomy, which enlarges the natural opening between the upper heart chambers to allow blood to move more easily from the right to the left side.
• Stent placement, which keeps the ductus arteriosus open by placing a small tube in the vessel to maintain blood flow to the lungs.

Babies often need different types of heart surgery over time. The choice of surgery depends on the size of the right ventricle and pulmonary artery.

Common surgical options include:

• Shunting to create a new pathway for blood to reach the lungs. A bypass shunt connects the aorta to the pulmonary arteries, although many infants outgrow this shunt within months.
• Glenn procedure, which connects one of the major veins returning blood to the heart directly to the pulmonary artery. This helps improve blood flow and can support the growth of the right ventricle.
• Fontan procedure for children whose right lower heart chamber remains too small. This surgery creates a pathway that directs most or all blood entering the heart straight into the pulmonary artery.
• Heart transplant, used when the heart is too damaged to repair.

If a ventricular septal defect is present, the opening is patched during surgery. The surgeon then creates a connection from the right pumping chamber to the pulmonary artery, sometimes using an artificial valve to complete the repair.