Pulmonary atresia is a type of congenital heart condition, which means it’s a condition a baby is born with. Babies born with pulmonary atresia have a pulmonary valve that does not work properly.
Normally, the pulmonary valve allows blood to move from the right ventricle in the heart to the pulmonary artery, which brings the blood to the lungs for oxygen. But with pulmonary atresia, this valve does not open, so blood cannot flow to the lungs. Sometimes, other blood vessels or openings in the heart develop to carry some blood to the lungs. But they can’t provide all the oxygen a baby needs. Because of a lack of oxygen, babies born with pulmonary atresia may have a blue tint to their skin.
Many babies with pulmonary atresia also have a hole in between the two lower chambers (ventricles) of the heart, called a ventricular septal defect (VSD). If a baby does not have a VSD, they may have a small right ventricle that does not work properly.
Sometimes, pulmonary atresia is found before birth during an ultrasound or fetal echocardiogram.
Symptoms of pulmonary atresia are often noticeable right after birth. The most common symptom is a blue tint to the skin, called cyanosis. Other symptoms may include:
Experts do not know what causes pulmonary atresia. The condition develops early in pregnancy when the heart is formed.
The treatment for pulmonary atresia may depend on your baby’s specific heart anatomy. Most babies need some type of treatment shortly after birth. Your care team at Arkansas Children’s is experienced in treating pulmonary atresia and will work with you to develop the best treatment plan for your child. Treatment options may include:
After treatment, babies with pulmonary atresia will need to have follow-up visits with a cardiologist (a heart doctor) into adulthood. Some children may need additional treatments or surgeries.