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Treatments and drugs
By Mayo Clinic staffTreatment of Ebstein's 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. Treatments may include:
Regular monitoring
If you have no signs or symptoms or abnormal heart rhythms, your doctor may recommend only careful monitoring of your heart condition with regular checkups. Checkups typically include a physical exam, electrocardiogram, chest X-ray, echocardiogram and, if necessary, a Holter monitor test. Your doctor may also ask you to undergo an exercise test, such as walking on a treadmill. This test checks your heart's response to physical activity.
Medications
If you have heart rhythm disturbances, medications may help control heart rate and maintain normal heart rhythm. Doctors commonly use medications, including calcium channel blockers, beta blockers, digitalis and anti-arrhythmic drugs, such as amiodarone.
Your doctor may also prescribe medications for signs and symptoms of heart failure, if you need them. These may include diuretics and other medications.
Surgical heart repair
Your doctor may recommend surgical repair when your signs and symptoms are troublesome or when your heart begins to enlarge and overall heart function begins to decrease. Because Ebstein's anomaly is rare, choose a surgeon who's familiar with the defect and who has experience performing procedures to correct it. Different types of procedures can be used to surgically treat Ebstein's anomaly and associated defects, including:
- Tricuspid valve repair. This involves reducing the size of the valve opening and allowing the existing valve leaflets to come together to work properly. This procedure is usually done when there's enough valve tissue to allow for repair.
- Tricuspid valve replacement. If the existing valve can't be repaired, it may be replaced by removing the deformed valve and inserting either a mechanical valve or a specially treated biological tissue (bioprosthetic) valve. If a mechanical valve is used, you'll need to take a blood-thinning medication for the rest of your life.
- Closure of the atrial septal defect (ASD). If an ASD is present, your surgeon can close the hole during surgery to repair or replace the defective valve.
- Maze procedure. Another procedure that doctors may perform during valve repair or replacement surgery is called a maze procedure. It's used to help correct fast heart rhythms in the atrium (atrial tachyarrhythmias). To do the procedure, your surgeon makes a series of incisions in the right atrium with a scalpel or a device that destroys tissue by freezing it. When these incisions heal, the scars create a barrier of tissue in your heart that causes it to beat at a normal rate. The Maze procedure is an open-heart surgery.
Radiofrequency catheter ablation
Radiofrequency catheter ablation is another procedure that can help treat a fast heartbeat or WPW syndrome. In this procedure, one or more catheters are threaded through your blood vessels to your inner heart, where the electrical impulses that cause your heart to beat are generated. Electrodes at the catheter tips are heated with radiofrequency energy. This destroys (ablates) a small spot of heart tissue and creates an electrical block along the pathway that's causing your arrhythmia. Usually, this stops your arrhythmia.
Heart transplantation
If you have Ebstein's anomaly with a severely malformed valve and poor heart function, a heart transplant may be necessary.
- Attenhofer Jost CH, et al. Ebstein's anomaly. Circulation. 2007;115(2):277-285.
- Pflaumer A, et al. Symptomatic neonates with Ebstein anomaly. Journal of Thoracic and Cardiovascular Surgery. 2004;127(4):1208-1209.
- Kapusta L, et al. Ebstein's anomaly: Factors associated with death in childhood and adolescence: A multi-centre, long-term study. European Heart Journal. 2007;28(21):2661-2666.
- Brown ML, et al. Functional status after operation for Ebstein anomaly: The Mayo Clinic experience. Journal of the American College of Cardiology. 2008;52(6):460-466.
- Chen JM, et al. Early and medium-term results for repair of Ebstein anomaly. Journal of Thoracic and Cardiovascular Surgery. 2004;127(4):990-999.
- Palmen M. Long-term follow-up after repair of Ebstein's anomaly. European Journal of Cardio-Thoracic Surgery. 2008;34(1):48-54.
- Agarwala BN, et al. Ebstein's anomaly of the tricuspid valve. http://www.uptodate.com/index. Accessed Sept. 29, 2008.
- Ebstein's anomaly. American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=11075. Accessed Oct. 2, 2008.
