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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. There, 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.

Heart Chambers and Valves
heart anatomy
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Blood Flow Through the Heart
IMAGE
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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, such as Down Syndrome

Symptoms

Symptoms may include:

  • Blue skin color
  • Rapid or difficult breathing
  • Fatigue
  • Irritability

Diagnosis

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

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

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, will 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, especially when the ventricular septum is intact. 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.

Prevention

There is no way to prevent this condition. Getting appropriate prenatal care is always important.

Revision Information

  • FamilyDoctor.org - American Academy of Family Physicians

    http://familydoctor.org

  • American Heart Association

    http://www.heart.org

  • Canadian Cardiovascular Society

    http://www.ccs.ca

  • Heart and Stroke Foundation of Canada

    http://ww2.heartandstroke.ca

  • Pulmonary atresia. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php. Updated July 15, 2013. Accessed July 16, 2013.

  • Pulmonary atresia. Johns Hopkins University, Cove Point Foundation website. Available at: http://www.pted.org/?id=pulmonaryatresia1. Updated May 16, 2011. Accessed July 16, 2013.

  • Single ventricle defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Single-Ventricle-Defects%5FUCM%5F307037%5FArticle.jsp. Updated January 8, 2013. Accessed July 16, 2013.