Treatment

Treatment of Ebstein anomaly depends on the severity of the defect and your signs and symptoms. The goal of treatment is to reduce your symptoms and avoid future complications, such as heart failure and arrhythmias.

Regular monitoring

If you have no signs or symptoms or abnormal heart rhythms, your doctor might recommend monitoring your heart condition with regular checkups.

Follow-up appointments generally include a physical exam and tests such as an electrocardiogram, echocardiogram, Holter monitor test and exercise stress test.

Medications

If you have heart rhythm disturbances, medications might help control your heart rate and maintain normal heart rhythm.

Your doctor might also prescribe medications to ease signs and symptoms of heart failure, such as drugs to prevent water retention (diuretics).

If you have certain heart rhythm problems or a hole (atrial septal defect) between the upper heart chambers, your doctor may prescribe medications to prevent blood clots.

Some babies are given an inhaled substance called nitric oxide to help improve blood flow to the lungs.

Surgery or other procedures

Your doctor might recommend surgery if your symptoms are affecting your quality of life. Surgery might also be recommended if your heart is enlarging and your heart function is decreasing. If you do need surgery, it's important to choose a surgeon who's familiar with the defect and who has training and experience performing procedures to correct it.

Several types of procedures can be used to surgically treat Ebstein anomaly and associated defects.

• Tricuspid valve repair. Surgeons reduce the size of the valve opening and allow the valve leaflets to come together to work properly. A band might be placed around the valve to keep it in place. This procedure is usually done when there's enough valve tissue to allow repair.

  A newer form of tricuspid valve repair is called cone reconstruction. Surgeons separate the leaflets of the tricuspid valve from the heart muscle. The leaflets are then rotated and reattached, creating a "leaflet cone."

  Sometimes, your valve might need to be repaired again or replaced in the future.

• Tricuspid valve replacement. If the valve can't be repaired, your surgeon might remove it and replace it with either a biological tissue (bioprosthetic) or mechanical valve. Mechanical valves aren't used often for tricuspid valve replacement.

  If you have a mechanical valve, you'll need a blood thinner to prevent blood clots. If you have any type of artificial valve, you'll need to take medication to prevent an inflammation of the inner lining of your heart (endocarditis) before dental procedures.

• Closure of the atrial septal defect. If there's a hole between the upper chambers of the heart (atrial septal defect), your surgeon can repair or replace the defective valve. Your surgeon can also repair other heart defects you have during this surgery.

• Maze procedure. If you have fast heart rhythms, your surgeon may perform the Maze procedure during valve repair or replacement surgery. In this procedure, your surgeon makes small incisions in the upper chambers of your heart to create a pattern, or maze, of scar tissue.

  Because scar tissue doesn't conduct electricity, it interrupts the stray heart signals that cause some types of arrhythmias. Extreme cold (cryotherapy) or heat (radiofrequency) energy also can be used to create the scars.

• Radiofrequency catheter ablation. If you have fast or abnormal heart rhythms, your doctor might perform this procedure. Your doctor threads one or more catheters through your blood vessels to your heart.

  Sensors at the tips of the catheters use heat (radiofrequency energy) to damage (ablate) a small area of heart tissue. This blocks the abnormal signals that are causing your arrhythmia. Some people may need repeat procedures.

• Heart transplantation. If you have severe Ebstein anomaly and poor heart function, a heart transplant might be necessary.