The treatment for Ebstein’s anomaly depends on the severity of the condition. If symptoms do not appear until adulthood, the defect may be managed with medications. If the defect is caught during childhood, but is not especially severe, the physician may choose to postpone surgery until the child has grown some.

Severe Ebstein’s, however, may require open-heart surgery at the earliest possible date. The surgery to repair the defect involves several steps, including:

Because this condition is so rare, treatment success depends in part on the skill of the medical personnel who are involved in the surgery. It is important to consult with pediatric cardiologists, surgeons and hospitals that specialize in the diagnosis, repair and medical management of these types of congenital defects. The patient’s outcome after surgery is also dependent on:

• Age at the time of surgery (generally the younger, the better).
  
  
• The extent to which the tricuspid valve is displaced, and the amount of blood that has been leaking back in the wrong direction.
  
  
• The extent to which the heart muscle sustained damage from overexertion.
  
  
• The patient’s symptoms.

The degree of enlargement of the right atrium can affect the overall ability of the heart to function over the long term. Also, the earlier symptoms present themselves, the more severe the condition and the higher the chance of developing early heart failure.

If leakage from the tricuspid valve is mild and tests reveal no abnormal heart rhythms (arrhythmias), most patients can benefit from exercise, according to the American Heart Association. Patients are advised to first consult their cardiologist, who may rule out certain high-intensity activities.

Like some other congenital heart defects, the cause of Ebstein’s anomaly is unknown. However, pregnant women who have been taking lithium (a treatment for bipolar disorder) are encouraged to speak with their physician or psychiatrist about the benefits and risks of this medication. Some data have suggested that taking lithium during pregnancy can increase the risk of birth defects, including Ebstein’s anomaly.