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Ebstein Anomaly

Overview

Ebstein anomaly is a rare heart defect that occurs when the tricuspid valve does not form properly before birth. A normal tricuspid valve separates the upper right and lower right heart chambers and opens to allow blood to flow from the top to bottom chamber while the heart is relaxed. For people with Ebstein anomaly, the tricuspid valve does not work well. It is often positioned incorrectly and the flaps of the valve are stuck to the heart wall and cannot close completely. Instead of blood flowing to the lungs, blood leaks backwards into the right chamber and causes backup.

Although an infant may not initially show symptoms, the defect can cause complications later in life that require treatment. Adults must seek a cardiologist trained in congenital heart defects for diagnosis and treatment.

  • Symptoms

    • Cyanosis, or bluish discoloration of skin
    • Decreased stamina
    • Fatigue
    • Heart rhythm problems that cause palpitations, dizziness and chest discomfort
    • Swelling of abdomen and legs
  • Prevention

    There is no known way to prevent the condition.

  • Diagnosis

    • Clinical examination. Your healthcare provider will evaluate you for signs of the condition, such as a heart murmur or an enlarged heart. Your provider will determine which tests are needed to diagnose the condition.
    • Imaging tests. Your physician may order tests to assess the severity of the defect, which could include an echocardiogram, chest X-ray or heart MRI.
  • Treatment

    • Drug treatment. Medications will not treat Ebstein anomaly, but may help alleviate symptoms. Your physician may prescribe diuretics to treat fluid retention or a blood thinner for heart rhythm abnormalities.
    • Valve repair. This surgery will repair the flaps of the tricuspid valve so they open and close properly. A surgeon can use your own tissue to repair the valve.
    • Valve replacement. This is an option for those who have more severe symptoms of the condition. If the valve cannot be repaired, you may need to have it replaced completely with a tissue valve.
  • Follow-up Care

    • You will most likely have routine visits with an adult congenital heart disease specialist, like those at the Kentucky Adult Congenital Heart Program. He or she will monitor you for further complications from the condition, as well as other cardiac disease risk factors.
    • You may need to check with your cardiologist before starting an exercise program.