What is Ebstein's Anomaly? Symptoms, Tests & Treatment | MedStar Health

This rare condition accounts for less than 1 percent of all rare congenital heart defects

Ebstein’s anomaly is a congenital heart defect in which the tricuspid valve between the right chambers of the heart does not close correctly.

If you’ve been diagnosed with Ebstein’s anomaly, you’ll need to see a specialist regularly to monitor your heart function and treat symptoms if necessary. The team in our Adult Congenital Heart Center has specialized training in the lifelong care that adults with congenital heart defects such as Ebstein’s anomaly may require.

What are the symptoms of ebstein’s anomaly?

The tricuspid valve has three flaps. If you have Ebstein’s anomaly, the flaps aren’t shaped or positioned correctly or are stuck to the heart wall so they can’t move. This allows blood to leak back into the heart, which makes the heart work less efficiently. Over time, it can lead to heart enlargement or heart failure.

Severe forms of Ebstein’s anomaly will be diagnosed shortly after birth. However, mild forms may not cause symptoms until later in life. Symptoms can worsen over time and may include:

  • Arrhythmia, an irregular heartbeat

  • Bluish discoloration of the skin
  • Occasional chest pain

  • Shortness of breath

Tests

Cardiac catheterization

Cardiac catheterization is a minimally invasive way to diagnose and treat a variety of heart and vascular conditions by guiding thin, flexible tubes called catheters through blood vessels to problem areas.

Chest X-ray

Chest X-rays use a small dose of radiation to create pictures of the structures inside the chest, including the lungs, heart, and chest wall.

Computerized tomography (CT) scan

The cardiac computed tomography scan, or cardiac CT, uses X-rays to create three-dimensional images of your heart and blood vessels.

Echocardiogram

An echocardiogram uses high-frequency sound waves to create images of your heart.

Electrocardiogram (ECG)

An electrocardiogram, also known as an ECG, measures the heart’s electrical activity.

Magnetic resonance imaging (MRI)

Magnetic resonance imaging, better known as cardiac MRI, is a combination of radio waves, magnets, and computer technology to create images of your heart and blood vessels.

Stress tests

Stress tests are used to assess how your heart works during physical activity. There are several types of stress tests, including treadmill or bike stress tests, nuclear stress tests, stress echocardiograms, and chemically induced stress tests.

Transesophageal echocardiogram (TEE)

Transesophageal echocardiogram allows us to take very detailed images of your heart structure from a probe in your esophagus.

Treatments

Cardiac implantable electronic device replacement

We may need to replace a cardiac implantable electronic device such as a pacemaker or implantable cardioverter defibrillator if the battery wears out or the device malfunctions.

Congenital heart disease treatments

Treatments for congenital heart conditions range from atrial septal defect and patent foramen ovale closures to cardiac ablations and heart valve replacements to heart transplants.

Structural heart and valve disease treatments

Structural heart and valve disease treatments address defects or abnormalities with the heart’s muscle or valves with or without surgery.

Tricuspid valve surgery

Tricuspid valve surgery includes repair or replacement of a damaged valve using traditional or minimally invasive methods.

Additional information

When you’re born with a heart problem, you may need complex care throughout your life. Our experts tailor this specialized care to your unique needs.

Ask MedStar Heart & Vascular Institute

Have general questions for our heart and vascular program? Email us at AskMHVI@medstar.net. If you have clinically-specific questions, please contact your physician’s office.