Ebsteins anomaly of the tricuspid valve surgery

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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]

Surgery

- 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.

ACC / AHA Guidelines- Recommendations for Surgical Interventions (DO NOT EDIT)

“

Class I 1. Surgeons with training and expertise in CHD should perform tricuspid valve repair or replacement with concomitant closure of an ASD, when present, for patients with Ebstein’s anomaly with the following indications: a. Symptoms or deteriorating exercise capacity. (Level of Evidence: B) b. Cyanosis (oxygen saturation less than 90%). (Level of Evidence: B) c. Paradoxical embolism. (Level of Evidence: B) d. Progressive cardiomegaly on chest x-ray. (Level of Evidence: B) e. Progressive RV dilation or reduction of RV systolic function. (Level of Evidence: B) 2. Surgeons with training and expertise in CHD should perform concomitant arrhythmia surgery in patients with Ebstein’s anomaly and the following indications: a. Appearance/progression of atrial and/or ventricular arrhythmias not amenable to percutaneous treatment. (Level of Evidence: B) b. Ventricular preexcitation not successfully treated in the electrophysiology laboratory. (Level of Evidence: B) 3. Surgical rerepair or replacement of the tricuspid valve is recommended in adults with Ebstein’s anomaly with the following indications: a. Symptoms, deteriorating exercise capacity, or New York Heart Association functional class III or IV. (Level of Evidence: B) b. Severe TR after repair with progressive RV dilation, reduction of RV systolic function, or appearance/ progression of atrial and/or ventricular arrhythmias. (Level of Evidence: B) c. Bioprosthetic tricuspid valve dysfunction with significant mixed regurgitation and stenosis. (Level of Evidence: B) d. Predominant bioprosthetic valve stenosis (mean gradient greater than 12 to 15 mm Hg). (Level of Evidence: B) e. Operation can be considered earlier with lesser degrees of bioprosthetic stenosis with symptoms or decreased exercise tolerance. (Level of Evidence: B)

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