Ebsteins anomaly of the tricuspid valve medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] and Claudia P. Hochberg, M.D. [2]Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [3]

Overview

Medical management of patients with Ebstein’s anomaly consists of supportive care such as:

Antiarrhythmics

Some Ebstein's anomaly patients present with an (antidromic) AV nodal reentrant tachycardia with associated pre-excitation. Among these patients, the preferred pharmacological treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers and digoxin are contraindicated.

If there is atrial fibrillation with pre-excitation, treatment options include procainamide, flecainide, propafenone, dofetilide and ibutilide since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response.

Anticoagulation

Warfarin is recommended for patients with Ebstein’s anomaly with a history of paradoxical embolus or atrial fibrillation.

Heart Failure Management

Diuretics may aid in reducing volume overload. Cardiac glycosides like digoxin can be used to maintain proper heart rhythm and increase contractility.

ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1][2]

Medical Therapy (DO NOT EDIT)[1][2]

Class I
"1. Anticoagulation with warfarin is recommended for patients with Ebstein’s anomaly with a history of paradoxical embolus or atrial fibrillation. (Level of Evidence: B)"

References

  1. 1.0 1.1 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: Executive Summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease)". Circulation. 118 (23): 2395–451. doi:10.1161/CIRCULATIONAHA.108.190811. PMID 18997168.
  2. 2.0 2.1 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.


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