Ebstein's Anomaly

Treatment

Mayo Clinic doctors with training in heart disease (cardiologists), surgeons and other specialists with experience in treating people with all types of congenital heart disease work with you to determine the most appropriate treatment. Doctors at Mayo Clinic evaluate and treat many people each year who have Ebstein's anomaly.

Treatment of Ebstein's anomaly depends on the severity of your condition and your heart size. Doctors choose treatment to minimize your symptoms and avoid future complications, such as progressive heart enlargement, heart dysfunction and arrhythmias.

Your treatment may include:

• Observation and monitoring. If you have a normal heart size and aren't experiencing any symptoms or irregular heartbeats, you may need only regular visits with a congenital cardiologist. A typical evaluation generally includes a physical exam and other tests, possibly including a stress test. Your doctor may advise you to take antibiotics prior to dental procedures to prevent infection of the heart lining or valves (bacterial endocarditis).
• Medications. Your doctor may prescribe medications to control abnormal heart rhythms or other symptoms.
• Surgery. Your doctor may recommend surgery if your symptoms are serious, your heart begins to enlarge or your overall heart function begins to decline. The type of surgery needed depends on the size of your lower-right heart chamber (right ventricle) and the condition of your tricuspid valve. Surgery may not be appropriate for everyone who has Ebstein's anomaly.
• Tricuspid valve repair. In this procedure, your surgeon makes the tricuspid valve opening smaller, which allows the existing flaps (leaflets) of the valve to create a working valve. If you have enough valve tissue to allow for repair, your doctor may recommend this surgery.
• Cone repair. Mayo Clinic surgeons perform cone repair, a new form of tricuspid valve repair available at only a few medical centers. In this surgery, your surgeon fashions the three leaflets of the tricuspid valve into a cone shape.
• Tricuspid valve replacement. Your surgeon removes the deformed valve and replaces it with a mechanical or biological valve. People who have a mechanical valve require blood thinning medications for life. Artificial and, to a lesser extent, repaired valves may not last a lifetime, so you may eventually need further surgery. Read more about heart valve surgery.
• Closure of the atrial septal defect (ASD). If you have a hole in the wall (septum) between your upper heart chambers (atria), your surgeon closes this atrial septal defect. Closure of the atrial septal defect prevents mixing of unoxygenated blood, which should be going to your lungs, and oxygenated blood, which comes from your lungs and goes to your limbs.
• Maze procedure. Some people who have heart rhythm problems are candidates for a right-sided Maze procedure, which your surgeon usually performs during a tricuspid valve repair or replacement. During the operation, your surgeon creates a series of incisions in your upper-right heart chamber (right atrium) to interrupt the abnormal electrical pathways that may be causing heart rhythm problems.
• Heart transplant. If you have severe Ebstein's anomaly with significant valve involvement and reduced heart function, your doctor may consider heart transplantation.

• Evaluation and treatment of heart rhythm conditions and abnormal electrical pathways. People who have Ebstein's anomaly often have heart rhythm conditions or an abnormal extra electrical pathway that causes heart rhythm problems. Your Mayo Clinic doctor evaluates you and treats these conditions so you can avoid other complications.

  Your doctor may recommend medication, surgery or catheter radiofrequency ablation to control heart rhythm problems. In catheter radiofrequency ablation, your doctor threads long, thin tubes (catheters) through your blood vessels to your heart to destroy (ablate) abnormal heart tissue causing your heart rhythm condition.

• Pregnancy and family planning. If you are considering pregnancy, you should discuss your plans with your doctor before you become pregnant. Your congenital heart disease cardiologist will work closely with your pregnancy doctor (obstetrician) to monitor you during your pregnancy, labor, delivery and after the pregnancy.