St. Luke's Hospital
Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
Find a Physician Payment Options Locations & Directions
Follow us on: facebook twitter Mobile Email Page Email Page Print Page Print Page Increase Font Size Decrease Font Size Font Size
America's 50 Best Hospitals
Meet the Doctor
Spirit of Women
Community Health Needs Assessment
Home > Health Information

Multimedia Encyclopedia

    Print-Friendly
    Bookmarks

    Ebstein's anomaly

    Ebstein's malformation

    Ebstein's anomaly is a rare heart defect in which parts of the tricuspid valve are abnormal. The tricuspid valve separates the right lower heart chamber (right ventricle) from the right upper heart chamber (right atrium).

    The condition is congenital, which means it is present from birth.

    Causes

    The tricuspid valve is normally made of three parts, called leaflets or flaps. The leaflets open to allow blood to move from the right atrium (top chamber) to the right ventricle (bottom chamber) while the heart relaxes. They close to prevent blood from moving from the right ventricle to the right atrium while the heart pumps.

    In persons with Ebstein's anomaly, the leaflets are unusually deep in the right ventricle. The leaflets are often larger than normal. The defect usually causes the valve to work poorly, and blood may go the wrong way back into the right atrium. The backup of blood flow can lead to heart swelling and fluid buildup in the lungs or liver. Sometimes, not enough bloodgets out of the heart into the lungs and the person may appear blue.

    In most cases, patients also have a hole in the wall separating the heart's two upper chambers and blood flow across this hole may cause oxygen-poor blood to go to the body. There may be narrowing of the valve that leads to the lungs (pulmonary valve).

    Ebstein's anomaly occurs as a baby develops in the womb. The exact cause is unknown, although the use of certain drugs (such as lithium or benzodiazepines) during pregnancy may play a role. The condition is rare. It is more common in white people.

    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

    Exams 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

    Outlook (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.

    Possible Complications

    A severe leakage can lead to swelling of the heart and liver, and congestive heart failure.

    Other complications may include:

    • Abnormal heart rhythms (arrhythmias), including abormally fast rhythms (tachyarrhythmias) and abnormally slow rhythms (bradyarrhythmias and heart block)
    • Blood clots from the heart to other parts of the body
    • Brain abscess

    When to Contact a Medical Professional

    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.

    BACK TO TOP

    • Congestive heart failure...

      Animation

    • Congenital heart defect ...

      Animation

    • Ebstein's anomaly

      illustration

    • Congestive heart failure...

      Animation

    • Congenital heart defect ...

      Animation

    • Ebstein's anomaly

      illustration

    Tests for Ebstein's anomaly

      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 medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
      adam.com

      A.D.A.M. content is best viewed in IE9 or above, Fire Fox and chrome browser.


      Back  |  Top
      About Us
      Contact Us
      History
      Mission
      Locations & Directions
      Quality Reports
      Annual Reports
      Honors & Awards
      Community Health Needs
      Assessment

      Newsroom
      Services
      Brain & Spine
      Cancer
      Heart
      Maternity
      Orthopedics
      Pulmonary
      Sleep Medicine
      Urgent Care
      Women's Services
      All Services
      Patients & Visitors
      Locations & Directions
      Find a Physician
      Tour St. Luke's
      Patient & Visitor Information
      Contact Us
      Payment Options
      Financial Assistance
      Send a Card
      Mammogram Appointments
      Health Tools
      My Personal Health
      mystlukes
      Spirit of Women
      Health Information & Tools
      Clinical Trials
      Health Risk Assessments
      Employer Programs -
      Passport to Wellness

      Classes & Events
      Classes & Events
      Spirit of Women
      Donate & Volunteer
      Giving Opportunities
      Volunteer
      Physicians & Employees
      For Physicians
      Remote Access
      Medical Residency Information
      Pharmacy Residency Information
      Physician CPOE Training
      Careers
      Careers
      St. Luke's Hospital - 232 South Woods Mill Road - Chesterfield, MO 63017 Main Number: 314-434-1500 Emergency Dept: 314-205-6990 Patient Billing: 888-924-9200
      Copyright © St. Luke's Hospital Website Terms and Conditions  |  Privacy Policy  |  Patient Notice of Privacy Policies PDF Sitemap St. Luke's Mobile