• Ebstein’s Anomaly—Child

    (Ebstein’s Malformation—Child; Anomaly, Ebstein’s—Child; Malformation, Ebstein’s—Child)


    Ebstein’s anomaly 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 returns to the left atrium and goes into the left ventricle. The blood moves out to the rest of the body.
    This defect occurs when the tricuspid valve develops lower than normal in the right ventricle. Also, the valve does not open and close normally. This allows blood to leak in the wrong direction. Ebstein’s anomaly can be mild to severe.
    Heart Chambers and Valves
    heart anatomy
    Copyright © Nucleus Medical Media, Inc.
    Blood Flow Through the Heart
    Copyright © Nucleus Medical Media, Inc.


    Ebstein's anomaly is present at birth. It is not known exactly why the heart does not develop normally.

    Risk Factors

    Specific risk factors for Ebstein’s anomaly are not clear. 2 possible risk factors include:
    • Chromosomal abnormalities
    • Environmental exposure
    • Certain medications taken by the mother during pregnancy, such as lithium


    Symptoms vary depending on how severe the defect is. In some cases, there may not be any symptoms. In other cases, symptoms may include:
    • Swelling in the abdomen and legs
    • Blue or pale skin color
    • Rapid heart beat or skipped heart beats
    • Decreased energy
    • Failure-to-thrive or gain weight as normal
    • Shortness of breath


    You will be asked about your child’s symptoms and medical history. A physical exam will be done. During the exam, the doctor may detect a heart murmur.
    Images may be taken of your child's chest. This can be done with:
    An electrocardiogram may also be done to measure the electrical activity of the heart.


    Talk with the doctor about the best treatment plan for your child. Often, surgery is needed right away. Treatment options include:


    Medications may be prescribed to:
    • Help restore normal heart rhythms
    • Reduce fluid in the body
    • Improve cardiac function


    Depending on your child’s condition, the doctor may recommend:
    • Surgery—Surgery may be needed to repair or replace the tricuspid valve. This will reduce leaking.
    • Ablation procedure—This procedure may be done if your child is having abnormal heart rhythms. A catheter is threaded up to the heart. Abnormal tissue is destroyed to stop the abnormal rhythms.

    Lifelong Monitoring

    Your child will have regular exams from a heart specialist. In some cases, your child may need antibiotics before some dental or medical procedures. This is to prevent infections.


    There are no current guidelines to prevent Ebstein's anomaly because the cause is unknown. Getting appropriate prenatal care is always important.


    Ebstein’s Anomaly Foundation http://www.ebsteinsanomaly.org

    Ebsteins Society http://www.ebsteins.org


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

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


    Ebstein’s anomaly. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Ebsteins-Anomaly%5FUCM%5F307025%5FArticle.jsp. Updated November 19, 2015. Accessed June 3, 2016.

    Ebsteins anomaly. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/e/ebstein. Updated January 2016. Accessed June 3, 2016.

    About Ebsteins. Ebsteins Society website. Available at: http://www.ebsteins.org/about. Accessed June 3, 2016.

    Ebstein anomaly of the tricuspid valve. EBSCO DynaMed Plus website. Available at:  http://www.dynamed.com/topics/dmp~AN~T114604/Ebstein-anomaly-of-the-tricuspid-valve. Updated January 25, 2016. Accessed June 3, 2016.

    Symptoms and diagnosis of congenital heart defects. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/SymptomsDiagnosisofCongenitalHeartDefects/Symptoms-Diagnosis-of-Congenital-Heart-Defects%5FUCM%5F002029%5FArticle.jsp. Updated October 26, 2015. Accessed June 3, 2016.

    7/14/2017 DynaMed Plus Literature Surveillance Update http://www.dynamed.com/topics/dmp~AN~T116076/Congenital-ventricular-septal-defect-VSD-in-children-and-adults: Patorno E, Huybrechts KF, et al. Lithium use in pregnancy and the risk of cardiac malformations. N Engl J Med. 2017 Jun 8;376(23):2245-2254.

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