Surgical treatment of Ebstein anomaly in pediatric patients: A 10-year single-center study.

Abstract

We report the early and long-term results of the strategies and surgical methods used in our center to treat pediatric patients who underwent surgical intervention to correct Ebstein anomaly (EA) in our center. In our study, a consecutive sample of 29 patients who underwent surgery for EA between February 2011 and February 2020 in our center were evaluated retrospectively. The 29 patients underwent a total of 40 operations. Univentricular repair was performed in 5 (17.2%), 1.5 ventricular repair in 5 (17.2%), and biventricular repair in the remaining 19 (65.5%) patients. Cone reconstruction (CR) was performed in eight (27.5%), non-Cone tricuspid valve (TV) repair technique in five (17.2%), ring annuloplasty in two (6.9%), and TV replacement in two patients (6.9%) who had undergone biventricular repair. In two patients (6.9%), only close an atrial septal defect. Two (6.9%) patients underwent a second operation for advanced tricuspid regurgitation (TR) in the early period. None of the 15 patients who underwent CR and TV plasty had moderate or advanced TR before discharge. Early mortality was seen in 1 (3.4%) patient. The mean follow-up period of the patients was 48.4±27.6 months. Three (10.7%) of the patients who were discharged after their first operation later underwent a second operation for TV regurgitation in the long term. No mortality was observed in any patient during long-term follow-up. Surgical treatment of EA is difficult, but its overall results are good. The anatomical repair rate is lower in neonatal and infant patients requiring surgery, but most of these patients underwent biventricular repair. Our long-term results demonstrated an acceptable survival rate, low mortality in the early postoperative period, and low incidence of re-intervention and morbidity.