046 * EBSTEIN'S ANOMALY IN ADULTS: MODIFIED CONE RECONSTRUCTION OF THE TRICUSPID VALVE CARRIES PROMISING OUTCOME

Abstract

Objectives: Ebstein's anomaly is a complex malformation involving not only the tricuspid valve but also both ventricles. Justification of a procedure in mildly symptomatic adults still remains debatable: repair techniques are demanding and valve replacement associated with poorer outcome. We reviewed our experience with the cone procedure. Methods: Since 2007, 40 adults under medical follow-up for Ebstein's anomaly were enrolled: median age 34.6 (range 16-70) years. A procedure was planned in 16 patients (group 1) presenting with severe tricuspid valve regurgitation (mean grade 3.3 +/- 0.7) because of impairment of functional capacity: 11 were in NYHA class II and 5 in class III. One had undergone a prior repair attempt elsewhere. All underwent modified cone repair including prosthetic ring insertion and leaflet patch augmentation. From the remaining 24 (group 2), 7 underwent percutaneous ASD closure. Median follow up was 2.8 years (range 4 months-5 years). Results: In group 1, no mortality occurred. One patient required subsequent repair for suture dehiscence six months postoperatively. All patients presented with mild or less tricuspid regurgitation at last echocardiographic control. NYHA functional class was significantly improved at follow-up (P < 0.001): 13 were in NYHA class I and 3 in class II. From group 2, two patients died, one 10 days after percutaneous ASD closure and one because of stroke. Conclusions: Cone repair of the tricuspid valve in adults with Ebstein's anomaly provides excellent mid-term results and significantly improves the functional status. This procedure might be considered, even in mildly symptomatic patients, in the presence of severe valve regurgitation