Heart disease

Ebstein’s Anomaly


Ebstein’s anomaly is congenital heart disease (present at birth). In this condition, the blood in the heart flows backward through the valve and can also lead to heart enlargement or heart failure. There are no specific signs and symptoms of Ebstein’s anomaly, but regular heart monitoring may help you to get early detection and treatment of this disease.

Causes of Ebstein’s Anomaly

Genetic and environmental factors may cause Ebstein’s anomaly, but the primary reason is still unknown.

What happens to the tricuspid valve in Ebstein’s anomaly?

The people who suffer from Ebstein’s anomaly typically have the following conditions of the tricuspid valve:

  • Misplaced: When the tricuspid valve is in the wrong position and sits more than usual in the heart.
  • Malformed: The leaflets of the tricuspid valve are abnormally shaped and unable to open or close properly.

Diseases associated with Ebstein’s Anomaly

  • Arrhythmias: Abnormal or irregular heartbeats are also associated with Ebstein’s anomaly, making it difficult for the heart to work correctly.
  • Holes in the heart: In many babies, a hole is present in the upper heart’s chambers called Patent Foramen Ovale. It is ordinarily present in all babies before birth and automatically closes after birth, but it remains open in some babies even after delivery. This hole decreases the oxygen level in the patient’s blood, resulting in discoloration of the skin and lips.
  • Wolff-Parkison-White (WPW) syndrome: WPW syndrome is associated with Ebstein’s anomaly in which the patient experiences an abnormal heartbeat.

Symptoms of Ebstein’s Anomaly

Ebstein’s anomaly may not show symptoms in its early stage but gradually appear in adulthood. The signs include:

  • Fatigue
  • Cyanosis
  • Dizziness
  • Shortness of breath during physical activities
  • Chest pain
  • Discoloration of skin and lips
  • Abnormal heart rhythm
  • Heart failure symptoms
  • Slow weight gain

When to see a doctor?

If your child has skin discolorations, arrhythmias, fluid retention, or shortness after every physical activity, contact your doctor immediately for a proper checkup. Early detection and treatment of this disease help you to improve the symptoms.

Diagnosis of Ebstein’s Anomaly

During diagnosis, your cardiologist inquired about the symptoms of your disease, performed a physical checkup, and advised you for the following test:

  • Cardiac MRI: A Cardiac MRI is a safe, non-invasive, and painless method to get the internal picture of your heart. It is primarily used to diagnose heart diseases, detect heart damage, and notice blood flow in heart valves and chambers.
  • Echocardiogram: An echocardiogram is ultrasound testing to detect the heart valves and chambers functioning. In this test, the doctor uses sound waves to create pictures of the internal heart structure to see any defect in the heart’s functioning.
  • Stress Echo: The stress Echo or stress Echocardiography is a test used to determine the working of your heart and the blood vessels. During these tests, the doctor may ask you to exercise on a treadmill or on a stationary bicycle to monitor your heartbeat and blood pressure.
  • Electrocardiogram: An electrocardiogram is a standard, painless test that records the heart’s electrical activity. It is used to detect the position and size of your heart chambers.
  • Holter Monitor: A Holter monitor is a small electrical device that detects heart rate and rhythm over an extended period. During the test, you must wear this device for 24 to 48 hours to record heart activity, and later, your doctor examines the results.

Treatment of Ebstein’s Anomaly

The treatment usually depends on the severity of the disease. If the patient has mild or no symptoms, the doctor monitors the heart and looks at the changes. While, in case of arrhythmia or heart failure, the patient receives non-invasive treatment, which includes:

  • Limiting physical activities: The doctor must be asked to restrict the patient’s physical movement, which may increase its severity.
  • Medication: The doctor may also recommend a medicine to manage your arrhythmia and heart failure.

Surgery: If the patient’s condition gets worse over time, doctors will advise tricuspid valve surgery to provide long-lasting results. However, if it’s not possible, he may replace your valve with another mechanical valve. In rare cases, your doctor moves forward for multiple treatments to combine your tricuspid valve surgery with other heart surgical procedures, such as arrhythmia treatment or repair holes in the heart’s upper chambers. If none of these treatments worked out, your doctor might perform a heart transplant.

Risk factors of Ebstein’s Anomaly

Ebstein’s anomaly usually develops in the baby’s heart during pregnancy. The actual risk factor behind this disease is still unknown, but some genetic and environmental factors can play a critical role in its development. A family history of heart disease and the mother’s use of medications, including lithium, can also increase the risk of Ebstein’s anomaly in the child.


The complications of Ebstein’s anomaly are rare, but some possible difficulties include:

  • Stroke
  • Heart failure
  • Sudden cardiac arrest


The actual cause behind Ebstein’s anomaly is still unknown; there isn’t any tip or medication that helps you to prevent this disease.


  • https://www.mayoclinic.org/diseases-conditions/ebsteins-anomaly/symptoms-causes/syc-20352127
  • https://my.clevelandclinic.org/health/diseases/16946-ebsteins-anomaly-for-adults
  • https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.106.619338
  • https://medlineplus.gov/ency/article/007321.htm
  • https://emedicine.medscape.com/article/154447-overview#a1
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