Pulmonary atresia (1582)

Key points below


Pulmonary atresia is a problem with the heart valve that lets blood flow from the heart to the lungs. It is congenital, which means your baby was born with it. The exact cause is not known.

The normal heart

normal heart

What is pulmonary atresia?

The pulmonary valve is between the right ventricle and the pulmonary artery. When it does not form correctly, it is called pulmonary atresia. Because the valve does not open, blood can’t flow into the
lungs. This is not a problem before the baby is born because the lungs are not working yet. The PFO lets blood go from the right to the left side of the heart. Pulmonary atresia can happen with or without a hole in the septum. If there is a hole, it is called a ventricular septal defect (VSD).

There may also be problems with the coronary arteries. These are the blood vessels that bring blood to the heart muscle itself.

heart with pulmonary atresia

What are the symptoms?

The symptoms of pulmonary atresia are normally seen just after birth. These babies are often very sick. Symptoms include:

How is it diagnosed?

These include:

- Chest X-ray. Shows the chest, including the lungs and the heart.
- Electrocardiogram (EKG). Records the heart’s electrical activity.
- Echocardiogram (echo). Looks at a moving picture of the heart.
- Pulse oximeter or oxygen saturation test. Measures the oxygen level in the blood.

How is it treated?

What are the long term concerns?

Home Monitoring Program

Your baby may go home with a baby scale and a machine that measures the amount of oxygen in your baby’s blood. It is called a pulse oximeter. You will be given a special book to keep a record of
your baby’s feeding schedule, oxygen level, and weight. The cardiology nurse practitioners will teach you how to do this before your baby leaves the hospital. They will let you know when and who to call if you have questions or concerns.

ALERT

Call your child’s doctor, nurse, or clinic if you have any questions or concerns or if your child has special health care needs that were not covered by this information.