Ebstein's anomaly of the tricuspid valve treatment

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]

Treatment

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.

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.

Surgery may include

- Tricuspid valve repair or replacement (when possible repair is preferable to replacement as it is associated with a lower mortality rate and fewer complications).

• Atrial septal defect repair.
• Right atrial plication (unclear whther this improves right ventricular function).
• Cardiac defibrillator implantation.
• Accessory pathways may require surgical intervention or ablation.

Hospital mortality at the Mayo for valve reconstruction is 6.7%. Only 1.6% in the Mayo series required reoperation. Long term follow up in the Mayo series showed 92% to have Class I or II symptoms and a 10 year mortality rate of 8%.

Generally, surgical treatment improves the exercise intolerance from NYHA-FC III or IV to NYHA-FC I or II.

Regardless of severity of the Ebstein’s Anomaly and type of treatment, risk of sudden death remains an important issue in patients with Ebstein's anomaly.