New trend: Surgical correction of Ebstein's anomaly using modified cone repair and a combination of different techniques.

Abstract

Ebstein's anomaly,a rare cardiac disease that accounts for <1% of all congenital heart diseases, is a right ventricular myopathy with varying degrees of delamination in the endocardium under the tricuspid valve. Cone reconstruction, first described by da Silva in 2004, is a modification of the Carpentier technique. An alternative surgical treatment for Ebstein's anomaly, it is now the preferred surgical corrective technique. This study demonstrates that our modifications of the cone repair procedure to correct Ebstein's anomaly improve valve coaptation and stabilization in the early and midterm. Of the 134 patients diagnosed with Ebstein's anomaly in our clinic between January 2012 and October 2020, 10 underwent a cone procedure and its modifications and were thus included in the study. The mean age of these patients was 28.6 ± 18.79 years and ranged from 1 to 61 years. Thirty percent of the patients were male and 70% were female. The additional procedures performed were an isolated cone procedure (two patients), anomalous pulmonary venous return repair in addition to a cone procedure (one patient), single-vessel coronary artery bypass (one patient), Glenn procedure (two patients), and tricuspid ring annuloplasty (four patients). Based on the results of this study, which assesses the outcomes of patients who underwent cone repairs with surgical modifications, we argue that right ventricular oblique plication, tricuspid valve delamination, surgical valve rotation, and tricuspid ring annuloplasty protect valve coaptation and function in the early and midterm, thereby having beneficial effects on right ventricle remodeling.