Pulmonary atresia is a congential condition (a condition present at birth) that affects a newborn’s heart. It limits blood flow from the heart to the lungs. A baby with this condition may need surgery soon after birth.

Pulmonary atresia affects a newborn’s pulmonary valve. This valve allows blood to flow from the heart into the lungs through the pulmonary artery. If a baby has pulmonary atresia, the pulmonary valve does not form correctly.

Read on to learn more about pulmonary atresia, including the types, causes, risk factors, symptoms, and treatments.

There are two typesTrusted Source of pulmonary atresia: pulmonary atresia with an intact ventricular septum (PA-IVS) and pulmonary atresia with a ventricular septal defect (PAVSD).

PA-IVS

PA-IVS occurs when a newborn has pulmonary atresia but does not have a hole in the ventricular septum. The ventricular septum is the wall separating the ventricles, which are the bottom two chambers of the heart.

If a newborn has PA-IVS, blood cannot enter the pulmonary artery, which carries deoxygenated blood from the right side of the heart to the lungs. Instead, it enters the left atrium through a hole called the foramen ovale. This is a natural opening between the two atria that usually closes after a baby is born.

PA-IVS may also cause a newborn to have an underdeveloped pulmonary artery and right ventricle. This may cause the heart to have difficulty pumping blood to the lungs and the rest of the body.

PAVSD

PAVSD occurs when a newborn has pulmonary atresia and has a hole in the ventricular septum. This allows blood to flow directly from the right ventricle into the left ventricle.

A newborn with PAVSD may have a more developed right ventricle and pulmonary artery.

Learn more about the septum of the heart, which contains the ventricular septum.

The cause of pulmonary atresia is unclearTrusted Source in most cases. However, the condition may result from mutations in the chromosomes or genes.

There may be a connection between PAVSD and certain genetic syndromes. Some research suggests that PAVSD may occur in 45% of childrenTrusted Source who have DiGeorge syndrome. DiGeorge syndrome is a congenital condition in which a baby is missing part of chromosome 22.

Factors that may increase the risk of pulmonary atresia includeTrusted Source:

  • a history of congenital heart disease in either parent
  • a pregnant person’s use of alcohol, drugs, or certain medications that may cause developmental issues in a fetus
  • cigarette smoking before or during pregnancy
  • pregnancy at an older age
  • a lack of effective treatment for diabetes

A newborn with pulmonary atresia will show signs and symptoms of the condition at birthTrusted Source or shortly after. These may include:

  • cyanosis (bluish skin discoloration)
  • heart murmur
  • tiredness or fatigue
  • breathing difficulties
  • difficulty with feeding
  • failure to thrive (a condition in which a baby does not grow as expected)

A healthcare professional may diagnose pulmonary atresia before or afterTrusted Source a baby is born.

During pregnancy, a healthcare professional may detect pulmonary atresia using an ultrasound. An ultrasound uses sound waves to create an image of the fetus.

If a healthcare professional suspects that a fetus may have pulmonary atresia, they may perform a fetal echocardiogram, which is an ultrasound that shows the fetus’s heart in greater detail.

If a doctor notices signs of pulmonary atresia once a baby is born, they may request an echocardiogram or other tests, such as:

  • Cardiac catheterization: This test involves inserting a long, thin tube into the heart. It allows the doctor to look inside the heart and measure the baby’s blood pressure and oxygen levels.
  • Electrocardiogram (EKG): An EKG uses sensors placed on the body to track the electrical activity of the baby’s heart.
  • Pulse oximetry screening: This test uses sensors to measure a baby’s blood oxygen levels.

Doctors treat most casesTrusted Source of pulmonary atresia with medication to keep the ductus arteriosus open. The ductus arteriosus is a blood vessel that connects the pulmonary artery and the aorta.

However, treatment may depend on the severity of the condition. A doctor may treat certain cases by inserting a stent into the baby’s ductus arteriosus. This prevents it from closing and may help improve blood flow.

A baby with pulmonary atresia may need surgery soon after birth. During these procedures, a surgeon may do any of the following:

  • widen or replace the pulmonary valve
  • enlarge the passage to the pulmonary artery
  • patch the hole between ventricles (if a baby has PAVSD)

In severe cases, a baby may need a heart transplant.

Because the exact cause of pulmonary atresia is unclear, it may not be possible to prevent the condition.

However, by avoiding risk factors, a pregnant person may be able to help reduce the chances that the condition will develop. It may be helpful for a person to:

  • avoid smoking
  • avoid drinking alcohol during pregnancy
  • seek proper treatment for diabetes, if applicable

Children with pulmonary atresia may be more likelyTrusted Source to develop endocarditis, which is an infection of the inner lining of the heart.

Other possible complications includeTrusted Source:

The life expectancy of a child with pulmonary atresia may vary based on factors such as the severity of the condition and any additional complications.

In a 2020 studyTrusted Source, researchers looked at the survival rates of 88 children who received surgery for PAVSD. They found that after surgical treatments, the children had the following survival rates:

  • 95% at the age of 1 year
  • 83.7% at the age of 5 years
  • 79.6% at the age of 10 years

However, the outlook will be different for each person. The baby’s doctor can provide more accurate information based on their individual circumstances.

There is no cure for pulmonary atresia, but treatments are available that may help a person manage the condition.

A person with pulmonary atresia may needTrusted Source lifelong follow-ups with a cardiac specialist. An adult with pulmonary atresia may needTrusted Source additional surgeries or medical care.

Pulmonary atresia is a congenital condition that affects the pulmonary valve. The condition has two types: pulmonary atresia with an intact ventricular septum (PA-IVS) and pulmonary atresia with a ventricular septal defect (PAVSD).

The condition is usually noticeable at birth or soon after, and in some cases a doctor may be able to diagnose it during pregnancy.

Signs and symptoms of pulmonary atresia can include cyanosis, heart murmur, breathing difficulties, feeding difficulties, tiredness, and failure to thrive.

A baby with pulmonary atresia may need surgery soon after birth and may need additional surgeries or treatments as they get older.

While the exact cause of pulmonary atresia is unclear, a person can take steps to reduce the likelihood that the condition will develop. For example, a person can avoid smoking, avoid drinking alcohol or taking certain drugs during pregnancy, and make sure that they receive proper treatment for diabetes if necessary.