Pulmonary Atresia

What is pulmonary atresia?

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.

What are the signs and symptoms of pulmonary atresia?

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:

• Fast breathing
• Problems breathing
• Poor feeding
• Extreme tiredness
• Cold, clammy skin

What causes pulmonary atresia?

Experts do not know what causes pulmonary atresia. The condition develops early in pregnancy when the heart is formed.

How is pulmonary atresia treated?

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:

• Medication to keep a connection in the heart, called the ductus arteriosus, open after birth. Usually it closes shortly after birth, but if kept open, it can help deliver blood to the lungs until the baby can have a permanent treatment.
• A catheterization procedure, called a balloon valvuloplasty. During this procedure, your child’s doctor places a thin tube (called a catheter) through a vein in the leg and into the heart. The doctor can then place another catheter with a balloon on the end of it into the valve to open it. The balloon is then deflated and removed.
• Surgery may be the only option for some babies with pulmonary atresia. In some cases, your baby may need more than one surgery.

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.