Pulmonary atresia with ventricular septal defect

Diagnosis

Pulmonary atresia with ventricular septal defect, also called PA-VSD, is often diagnosed during pregnancy or soon after birth.

Tests

Tests that may be used to diagnose pulmonary atresia with ventricular septal defect include:

• Pulse oximetry. For this simple test, a small sensor clips onto a hand or foot. It checks the amount of oxygen in the blood.
• Chest X-ray. A chest X-ray shows the shape and size of the heart and lungs.
• Echocardiogram. This is an ultrasound of the heart. Sound waves make images of the beating heart. An echocardiogram of a baby's heart during pregnancy is called a fetal echocardiogram. It can diagnose pulmonary atresia.
• Electrocardiogram, also called an ECG or EKG. This quick test shows how the heart is beating. Patches with sensors, called electrodes, stick to the chest and sometimes to the arms or legs. Wires connect the patches to a computer, which prints or displays results. An ECG can find irregular heartbeats.
• Cardiac catheterization. A doctor places a flexible tube called a catheter in a blood vessel, usually in the groin or wrist. It's guided to the heart. Dye flows through the catheter to heart arteries. The dye helps the arteries show up more clearly on images.
• Heart CT scan, also called a cardiac CT scan. This test uses several X-rays to make pictures of the heart and blood vessels. It shows the shape of the heart and lungs. A cardiac CT can help diagnose major aortopulmonary collateral arteries, also called MAPCAs. Knowing about the MAPCAs is important for planning treatment.

Treatment

A baby with pulmonary atresia with ventricular septal defect, also called PA-VSD, needs treatment right away. Treatment may include one or more surgeries or procedures.

Surgeries or other procedures

A baby with pulmonary atresia with PA-VSD needs one or more surgeries or procedures to improve blood flow to the lungs. The treatments also help the heart work better. Treatment depends on the structure of the pulmonary arteries and whether there are major aortopulmonary collateral arteries, also called MAPCAs.

Surgeries or other procedures may include:

• Catheter procedure. Thin, flexible tubes called catheters are used to look at the baby's heart and blood supply to the lungs. Sometimes a rigid tube called a stent is placed in the patent ductus arteriosus. This keeps the vessel open and lets blood flow into the lungs.
• Systemic-to-pulmonary artery shunt. This surgery may be needed in the first few days of life to send more blood to the lungs. The surgeon makes a connection, called a shunt, using a small tube. An example is the Blalock-Taussig shunt, also called the BT shunt.
• Complete repair. This treatment may be done when as a baby is young or after a shunt or stent is placed. During complete repair, a surgeon closes the hole in the heart. The surgeon creates a pathway between the right lower heart chamber and the pulmonary artery.
• Unifocalization. Sometimes, when most of the blood going to the lungs is through MAPCAs, those blood vessels need to be connected. This surgery is called unifocalization. The surgery may be done in stages. It may be done as part of a complete repair. Sometimes it's the first step toward an eventual complete repair.

Babies with pulmonary atresia with ventricular septal defect need regular checkups by a doctor trained in heart conditions present at birth. This type of doctor is called a congenital cardiologist.

Clinical trials

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.

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