Definition
Pulmonary atresia is a rare heart defect. In a normal heart, the blood flows in from the body to the right atrium. It then goes into the right ventricle. Next, the blood travels to the lungs through the pulmonary valve. Here, it picks up fresh oxygen. The blood then returns to the left atrium and goes into the left ventricle. The blood moves out to the rest of the body.
With this defect, there is no pulmonary valve in the heart. Blood cannot flow into the pulmonary artery. This is the artery that brings blood to the lungs. Other heart problems, like a small right ventricle, may also be present. This is a serious condition that requires care from a doctor.
Causes
A direct cause is not known. This defect develops while the baby is forming in the womb. The baby is born with the condition.
Risk Factors
These factors increase the chance of pulmonary atresia in your child:
- Family history of congenital heart defect
- Other heart defects
- Certain chromosomal disorders (eg, DiGeorge syndrome )
Symptoms
Symptoms may include:
- Blue skin color
- Rapid or difficult breathing
- Fatigue
- Irritability
Your child’s doctor may also detect a heart murmur during the exam.
These symptoms may be due to other conditions. If your child has any of these, tell the doctor right away.
Diagnosis
Your doctor will:
- Ask about your child’s symptoms and medical history
- Do a physical exam
Tests may include the following:
- Echocardiogram —a test that uses sound waves (ultrasound) to examine the size, shape, and motion of the heart
- Chest x-ray —imaging test that uses low amounts of radiation to get a picture of the chest
- Electrocardiogram (ECG, EKG)—a test that records the heart’s activity by measuring electrical currents through the heart muscle
Treatment
Talk with the doctor about the best treatment plan for your child. Some defects may be so severe that they are difficult to treat. Treatment options include:
Medications
Medicines, like Prostaglandin E1, may be given to keep a vessel that connects the pulmonary artery and the aorta open. This opening allows some blood to continue to reach the lungs. This is a temporary treatment.
Surgery
Sometimes a shunt can be placed between the aorta and pulmonary artery. This is done to improve blood flow to the lungs.
Several surgeries may be considered depending on:
- The size of the pulmonary artery and right ventricle
- Other heart abnormalities that your child may have
Open heart surgery aims to:
- Remove the temporary shunt
- Close any holes between the chambers of the heart (if they are present)
- Enlarge the pulmonary artery (if needed)
- Insert an artificial valve (if needed)
- Reconnect veins and arteries for proper circulation
When the right ventricle is too small to pump blood effectively, other surgeries may be done. These can reroute blood to the lungs.
Lifelong Monitoring
Your child will need to see a heart specialist regularly. Your child may need to take antibiotics prior to certain medical or dental procedures. This is to prevent heart infections.
