Various Repair Techniques to Correct Tricuspid Valve Incompetence in Ebstein Anomaly and Their Impact on Long-Term Ventricular Function and Functional Outcome

Objective: We describe a repertoire of repair techniques according to types of Ebstein anomaly to correct the tricuspid valve (TV) incompetence, and report the long-term ventricular function and functional outcome.

Methods: Sixty-eight patients (mean age: 26.9 ± 7.3 years) with Ebstein anomaly (Types A = 21, B = 23, C = 15, D = 9) underwent correction of TV incompetence. In all, the atrialized right ventricle (RV) is incorporated into the contractile RV using the most mobile leaflet for valve competence. Posterior annulorrhaphy was performed for Types A, B, and C. The double orifice valve technique was employed mostly in types C and D. Sebening stitch was applied in combination in most types. A combination of anterior and posterior annulorrhaphy was also performed for types A and B. In three patients with type C, additional bidirectional Glenn anastomosis was performed.

Results: Mean follow-up duration was 13.25 ± 1.3 years (median: 9.34, range: 1 – 24). Mean NYHA class improved from 3.4 to 1.3 (p < 0.001). Severity of TV incompetence was reduced from 3.2 to 1.3 (p < 0.001). Exercise tests demonstrated improvement in maximal oxygen uptake (p < 0.02). Mean basal, middle, and apical ventricular strain significantly improved to 25.7% (p < 0.011), 23.7% (p < 0.001), and 19.36% (p < 0.05), respectively. Freedom from reoperation was 100% at 1 year, 98.3% and 92.9% at 5 and 20 years, respectively. Early and late mortality was 5.8% and 2.9%, respectively. Overall survival rate was 94.2%, 92.7%, and 91.26% at 30 days, 5, and 20 years, respectively.

Conclusion: The various repair techniques, which preserve the atrialized chamber and are employed individually according to morphology, provide satisfactory long-term ventricular function and functional outcome even in severe types of Ebstein anomaly.