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Ebstein's anomaly


Alternative Names:

Ebstein's malformation

Symptoms:

Symptoms range from mild to very severe. Often, symptoms develop soon after birth and include bluish-colored lips and nails due to low blood oxygen levels. In severe cases, the baby appears very sick and has trouble breathing.

Symptoms in older children may include:

  • Cough
  • Failure to grow
  • Fatigue
  • Rapid breathing
  • Shortness of breath
  • Very fast heartbeat
Signs and tests:

Newborns who have a severe leakage across the tricuspid valve will have very low levels of oxygen in their blood and significant heart swelling. The doctor may hear abnormal heart sounds, such as murmur, when listening to the chest with a stethoscope.

Tests that can help diagnose this condition include:

  • Chest x-ray
  • Magnetic resonance imaging (MRI) of the heart
  • Measurement of the electrical activity of the heart (EKG)
  • Ultrasound of the heart (echocardiogram)
Treatment:

Treatment depends on the severity of the defect and the specific symptoms. Medical care may include:

  • Medications to help with heart failure
  • Oxygen and other breathing support
  • Surgery to correct the valve may be needed for children who continue to worsen or who have more serious complications
Expectations (prognosis):

In general, the earlier symptoms develop, the more severe the disease.

Some patients may have either no symptoms or very mild symptoms. Others may worsen over time, developing blue coloring (cyanosis), heart failure, heart block, or dangerous heart rhythms.

Calling your health care provider:

Call your health care provider if your child develops symptoms of this condition. Seek immediate medical attention if breathing problems occur.

Prevention:

There is no known prevention, other than talking with your doctor before a pregnancy if you are taking medicines that are thought to be related to developing this disease. You may be able to prevent some of the complications of the disease. For example, taking antibiotics before dental surgery may help prevent endocarditis.

References:

Bernstein D. Ebstein anomaly of the tricuspid valve. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 424.7.


Review Date: 2/7/2012
Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine; and Michael A. Chen, MD, PhD, Assistant Professor of Medicine, Division of Cardiology, Harborview Medical Center, University of Washington Medical School, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

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