Ebsteins anomaly of the tricuspid valve medical therapy
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], Maneesha Nandimandalam, M.B.B.S.[4]
Overview
Medical management of patients with Ebstein’s anomaly consists of supportive care such as control of the heart rhythm with antiarrhythmic drugs, inotropic agents and diuretics for heart failure, anticoagulation in patients with atrial fibrillation and paradoxical embolization.
Medical therapy
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[1][2]
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)[3][4]
| 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
- ↑ Luo M, Lin J, Qin Z, Du L (January 2017). “Prevention preferable to treatment: 3 case reports of patients experiencing right-sided heart failure after Ebstein anomaly correction”. Medicine (Baltimore). 96 (1): e5627. doi:10.1097/MD.0000000000005627. PMC 5228659. PMID 28072699.
- ↑ Kim HY, Jang SY, Moon JR, Kim EK, Chang SA, Song J, Huh J, Kang IS, Yang JH, Jun TG, Park SW (November 2016). “Natural Course of Adult Ebstein Anomaly When Treated according to Current Recommendation”. J. Korean Med. Sci. 31 (11): 1749–1754. doi:10.3346/jkms.2016.31.11.1749. PMC 5056206. PMID 27709852.
- ↑ 3.0 3.1 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.
- ↑ 4.0 4.1 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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