Ebstein's anomaly of the tricuspid valve medical therapy

and Claudia P. Hochberg, M.D. [mailto:chochber@bidmc.harvard.edu]

Associate Editor-In-Chief:

Mechanical and Device Based Therapy
The protracted natural history of this disease in contrast to the uncertainty of long-term results of surgical intervention supports the policy of postponing surgical intervention until the onset of disability.

Pharmacological Management
Ebstein's cardiophysiology typically presents as an (antidromic) AV nodal reentrant tachycardia with associated pre-excitation. In this setting, the preferred pharmacological treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs like 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.