• Ebstein’s Anomaly—Child

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

    Definition

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

    Causes

    This is a congenital defect. This means that the heart forms incorrectly when the baby is developing in the womb. The baby is born with the condition. It is not known why the heart develops this way in some babies.

    Risk Factors

    Specific risk factors for Ebstein’s anomaly are not clear. Two possible risk factors include:
    • Genetic abnormalities
    • Environmental exposure

    Symptoms

    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
    • Palpitations or feeling of skipped beats
    • Decreased energy
    • Failure-to-thrive or gain weight
    • Shortness of breath
    During the exam, the doctor may detect a heart murmur .
    These symptoms may be due to other conditions. If your child has any of these, talk to the doctor right away.

    Diagnosis

    The doctor will ask about your child’s symptoms and medical history. A physical exam will be done. Tests may include:
    • Echocardiogram —an imaging test that uses sound waves to look at the size, shape, and motion of the heart
    • Cardiac catheterization —a test that uses a catheter (tube) and x-ray machine to assess the heart and its blood supply
    • Chest x-ray —an imaging test that uses low amounts of radiation to create an image of the chest
    • MRI scan —a test that uses magnetic waves to make pictures of structures inside the chest
    • CT scan —a type of x-ray that uses a computer to make pictures of structures inside the chest
    • Electrocardiogram —a test that measures the electrical activity of the heart
    • Other monitors (Halter or Event monitor) and tests (eg, stress test ) to measure the heart’s rhythm and function

    Treatment

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

    Medication

    The doctor may prescribe medicines to:
    • Help restore normal heart rhythms
    • Reduce fluid in the body

    Procedures

    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.

    Prevention

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

    RESOURCES

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

    Ebstein’s Society http://www.ebsteins.org/

    CANADIAN RESOURCES

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

    Heart and Stroke Foundation of Canada http://ww2.heartandstroke.ca/splash/

    References

    American Heart Association. Ebstein’s anomaly. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=11075 . Accessed July 1, 2010.

    American Heart Association. How your cardiologist diagnoses heart defects. American Heart Association website. Available at: http://www.americanheart.org/presenter.jhtml?identifier=152 . Updated June 2010. Accessed July 5, 2010.

    Cincinnati Children’s Hospital. Ebstein’s anomaly. Cincinnati Children’s Hospital website. Available at: http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/ebstein.htm . Updated July 2009. Accessed July 1, 2010.

    DynaMed Editorial Team. Ebstein anomaly of the tricuspid valve. EBSCO DynaMed website. Available at:  http://www.ebscohost.com/dynamed/what.php . Updated February 11, 2010. Accessed July 1, 2010.

    Ebstein’s Society. Ebstein’s anomaly. Ebstein’s Society website. Available at: http://www.ebsteins.org/?page%5Fid=2 . Accessed July 1, 2010.

    Mayo Clinic. Atrioventricular canal defect. Mayo Clinic website. Available at: http://www.mayoclinic.com/health/atrioventricular-canal-defect/DS00745/DSECTION=risk-factors . Updated June 3, 2010. Accessed July 7, 2010.

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