Ebstein's Anomaly

​Ebstein's Anomaly is a congenital heart defect involving the right-sided valve (tricuspid valve), which opens from the right atrium (top chamber) to the right ventricle (bottom chamber). The tricuspid valve is abnormally formed, with one or two of the three leaflets being stuck lower than its usual place on the right atrium. In normal circumstances, the tricuspid valve allows blood to flow one way from right atrium to the right ventricle. If tricuspid leaflets are malformed, then blood may leak backward to the right atrium (regurgitation).

Ebstein Anomaly may not show symptoms if the condition is mild. Symptoms exhibited include:

• Shortness of breath, especially with exertion
• Fatigue
• Heart palpitations or abnormal heart rhythms (arrhythmias)
• A bluish discoloration of the lips and skin caused by low oxygen (cyanosis)

Risks

Activity

Activity restrictions are not necessary when the tricuspid leakage is mild and there are no associated problems. If it is severe, you may need to restrict your physical activity due to increasing strain on the right ventricle or presence of abnormal rhythms. Your cardiologist will advise you on your physical restrictions.

Prevention of Infective Endocarditis (IE)

The incident of IE in congenital patient is higher than general population. Therefore it is important for congenital patient to maintain good oral, dental and skin hygiene as primary prevention. Cosmetic tattooing and piercing are discouraged due to the risk of IE. Antibiotic prophylaxis is considered for patient at highest risk for IE before surgery and dental procedures. Please discuss with your congenital team for the latest update on the antibiotic prophylaxis.

Pregnancy and family planning

Most women with mild form of Ebstein Anomaly who are not blue usually carry through pregnancy without any significant problems. However, do consult your cardiologist if you become pregnant.

Others

Patient with Ebstein Anomaly may develop irregular heart rhythm, causing the heart to beat faster (palpitations), fainting, giddiness or chest discomfort. Severe malformation of the valve may cause fatigue, decrease effort tolerance and heart pump failure, which may require medical treatment.

Your doctor may review your signs and symptoms and conduct a physical examination. If your doctor suspects Ebstein Anomaly, your doctor may recommend several tests, including:

• Echocardiogram assesses the structure of your heart, the tricuspid valve and the blood flow through your heart.
  

Your doctor may also order a transesophageal echocardiogram to provide a detailed image of your heart.

• Electrocardiogram (ECG) can help your doctor detect irregularities in your heart's rhythm and structure, and offer clues as to the presence of an extra accessory pathway.
• Chest X-ray can reveal if your heart is enlarged, which may be due to Ebstein Anomaly.
• Cardiac MRI may be used to determine the severity of your condition, get a detailed view of the tricuspid valve, and assess the size and function of your lower right heart chamber (right ventricle).
• Holter monitor may be used to diagnose rhythm disturbances that occur at unpredictable times. You wear the monitor under your clothing and it records information about the electrical activity of your heart as you go about your normal activities for a day or two.

​Mild form of Ebstein Anomaly usually does not require surgery. When the tricuspid valve leaks severely causing right-sided heart chambers to be dilated and further weakened, then surgical intervention may be considered.

Patients with Ebstein Anomaly require follow-up care to monitor any irregular heart rhythm (arrhythmia) that may occur. Therefore, routine test such as electrocardiogram (ECG) may be performed during the medical check-up.