• Pulmonary Atresia—Child


    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
    Copyright © Nucleus Medical Media, Inc.
    Blood Flow Through the Heart
    Copyright © Nucleus Medical Media, Inc.


    Pulmonary atresia is present at birth. It is not known exactly why the heart does not develop normally.

    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 may include:
    • Blue skin color
    • Rapid or difficult breathing
    • Fatigue
    • Irritability


    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:


    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 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.


    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.


    There are no current guidelines to prevent pulmonary atresia because the cause is unknown.. Getting appropriate prenatal care is always important.


    American Heart Association http://www.heart.org

    Healthy Children—American Academy of Pediatrics http://www.healthychildren.org


    Canadian Cardiovascular Society http://www.ccs.ca

    Heart and Stroke Foundation of Canada http://www.heartandstroke.ca


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

    Pulmonary atresia. Johns Hopkins Children's Center website. Available at: http://www.hopkinschildrens.org/Pulmonary-Atresia.aspx. 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.

    Revision Information

  • LiveWell personal health survey

    How healthy are you really? Find out – free.Learn more

    It's time to stop guessing. If you want to make some changes but just aren't sure how, the free personal health survey from LiveWell is a great place to start.

  • HeartSHAPE Spotlight

    At risk for a heart attack? Learn more

    Fight heart disease and prevent heart attacks. HeartSHAPE® is a painless, non-invasive test that checks pictures of your heart for early-stage coronary disease.

  • Calories and Energy Needs

    Calorie NeedsLearn more

    How many calories do you need to eat each day to maintain your weight and fuel your physical activity? Enter a few of your stats into this calculator to find out.

  • Ideal Body Weight

    Ideal Body WeightLearn more

    Using body mass index as a reference, this calculator determines your ideal body weight range. All you need to do is enter your height.

  • Body Mass Index

    Body Mass IndexLearn more

    This tool considers your height and weight to assess your weight status.

  • Can we help answer your questions?

    Wellmont Nurse Connection is your resource for valuable health information any time, 24 hours a day, seven days a week. Speak to a Nurse any time, day or night, at (423) 723-6877 or toll-free at 1-877-230-NURSE.