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.

Pulmonary Atresia Causes

The problem is caused during the first eight weeks of your baby’s development. Some congenital heart defects are believed to be hereditary, causing heart problems to run in some families. In the majority of cases, this pulmonary atresia occurs randomly, with no clear reason that points back to a common cause.

Genetic factors are also thought to contribute to the likelihood of additional children developing the disease. According to emedicine there’s a 2.5 to 3 percent increased risk of occurrence in siblings. Similarly, if either parent has tetralogy of Fallot, the baby has an increased risk of 1.2 to 8.3 percent of developing pulmonary atresia.
This heart defect is related to similar anomalies in the spine, anus, esophagus and kidneys.

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.

Pulmonary Atresia Prevention

Since scientists don’t know the cause of pulmonary atresia, prevention isn’t yet possible. You can, however, reduce the risk of your baby getting pulmonary atresia by taking the following precautions.

Before you become pregnant, get a German measles (Rubella) vaccine. If you contract German measles while you’re pregnancy, it can affect your child’s heart development. A vaccination almost eliminates this possibility.

If you have chronic medical conditions, make sure to get them under control. For example, if you suffer from diabetes, keep a close eye on your blood sugar to reduce the risk of heart defects. For other chronic conditions like epilepsy that require medication, make sure you understand all the risks and benefits of taking these drugs while you are pregnant.

If you smoke or drink, this is a great time to quit. Smoking cigarettes and drinking alcohol increases the risk of pulmonary atresia in your baby.
If one or both parents has a family history of heart disease, or if you already have a child with pulmonary atresia you may want to see a genetic counselor and talk to your doctor before becoming pregnant again.

Last Reviewed:
October 10, 2016
Last Updated:
January 17, 2018