As an adult with Ebstein’s anomaly, when will I need treatment?

Ebstein’s anomaly has the greatest variation in severity among all congenital heart defects. Some infants with the defect do not survive gestation, while other individuals live a normal life span and never need treatment.

As an adult with a mild Ebstein’s malformation, you may not need any treatment for years. If you have an arrhythmia, you may be treated with medication to control your heart rate and rhythm. Depending on the severity of the arrhythmia, you may need nonsurgical treatment such as radiofrequency ablation to fix your heart rhythm. If you develop heart failure, you may need other medications, such as a diuretic.

The course of Ebstein’s anomaly is unpredictable, however, and the condition could worsen to the point where your symptoms are bothersome, or your heart could enlarge, leading to decreased heart function. In either of these situations, surgical treatment may be necessary.

What type of surgery is used to treat Ebstein’s anomaly?

Ebstein’s anomaly is a rare heart defect, particularly among adults, so should you require surgery, you will want to choose a surgeon with experience in treating adults with this specific condition. The most likely place to find a heart surgeon with this type of experience will be at a large academic medical center.

There are several surgical procedures used to treat Ebstein’s anomaly:

Repair or replacement of the tricuspid valve

The goal of this surgery is to fix the defective valve between the right atrium and the right ventricle so that the leaflets open and close correctly.

When there is enough tissue present, the valve can be repaired. This is the preferred treatment because it uses your own tissue. When the existing valve cannot be repaired, it is possible to replace it with a mechanical valve or one made of biologic tissue. If you receive a mechanical valve replacement, you will need to take blood-thinning medication for the rest of your life.

Atrial septal defect repair

Many people with Ebstein’s anomaly have a hole in the septum (the tissue between the heart’s upper chambers [the atria]). This hole will be closed surgically at the same time the valve repair is performed.

Arrhythmia surgery (maze procedure)

Depending on the type of arrhythmia you have, you may undergo maze surgery in combination with valve surgery. During maze surgery, the surgeon creates new electrical pathways in the heart that restore the normal heart rhythm.

Heart transplant

In the most severe cases, when the valve is seriously deformed, heart function is poor and other treatments are not effective, a heart transplant may be the best treatment option.

In a recently published study of patients between the ages of 4 and 58 who had surgery for Ebstein’s anomaly, survival and heart function were very good 10 years later.

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