Definition

Pulmonary atresia (PA) is a rare heart defect. With PA, 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.

Heart Chambers and Valves
heart anatomy
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Blood Flow Through the Heart
IMAGE
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Causes

PA is present at birth. It is not known exactly why the heart does not form as it should.

Risk Factors

These factors raise the chance of PA in your child:

  • Other family members with a congenital heart defect
  • Other heart defects
  • Certain problems with chromosomes, such as Down Syndrome

Symptoms

Your child may have:

  • Blue skin color
  • Problems breathing
  • Lack of energy
  • Irritability

Diagnosis

You will be asked about your child's symptoms and health history. A physical exam will be done. Your child's doctor may also detect a heart murmur during the exam.

Pictures may be taken of your child's chest. This can be done with:

Treatment

Talk with the doctor about the best plan for your child. Some defects may be so severe that they are hard to treat. Your child may have:

Medications

Medicine will be given to keep a vessel that connects the pulmonary artery and the aorta open. This opening lets some blood continue to reach the lungs, especially when the ventricular septum is healthy. This is a short term treatment.

Surgery

Sometimes a shunt can be placed between the aorta and pulmonary artery. This is done to help blood flow to the lungs.

Many 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 short term shunt
  • Close any holes between the chambers of the heart, if they are there
  • Make the pulmonary artery larger, if needed
  • Place an artificial valve, if needed
  • Reconnect veins and arteries for proper blood flow

When the right ventricle is too small to pump blood, 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 before certain medical or dental procedures. This is to prevent heart infections.

Prevention

PA can't be prevented.

Revision Information

  • Reviewer: Kari Kassir, MD
  • Review Date: 05/2018 -
  • Update Date: 06/29/2018 -