Pulmonary Atresia

What is Pulmonary Atresia?

Pulmonary Atresia is a birth defect where the valve that releases blood from the heart into the lungs does not form properly. Oxygenated blood can’t travel between the heart and lungs and the baby doesn’t get enough oxygen.

In some cases, pulmonary atresia can be diagnosed in the womb. If you have a family history of the problem or a routine ultrasound shows a potential issue, you can have a fetal echocardiogram done to image the baby’s heart and identify any malformations.

Other cases are diagnosed at birth. Your doctor will be able to hear a heart murmur that indicates an issue.

Congenital heart defects like pulmonary atresia are more common in mothers who smoke, drink alcohol during pregnancy, have diabetes or lupus, or who have had some types of viral illnesses like German measles in early pregnancy. Children with Down’s Syndrome are also more likely to have a heart defect.

What are the Symptoms of Pulmonary Atresia?

Infants with pulmonary atresia often appear slightly blue because they are not getting enough oxygen into their tissues. This is called cyanosis. Other symptoms include fast or labored breathing and getting too fatigued to eat properly.

Left untreated, most babies will eventually die from pulmonary atresia.

How is Pulmonary Atresia Treated?

It’s likely that your baby will need surgery to treat pulmonary atresia. The exact surgical procedure will vary depending on the structural problem. Often, multiple surgeries are required during the first few years of your child’s life.

Initially, your doctor may surgically place a shunt that will move blood between the heart and lungs. Subsequent procedures will depend on how well developed the child’s right ventricle of the heart is and whether it can reliably pump blood to the lungs. Extremely serious cases may require a heart transplant.

Last Reviewed:
October 10, 2016
Last Updated:
August 24, 2017