Surgical strategies in Ebstein anomaly: 28 years' experience in a pediatric hospital

Abstract

Ebstein anomaly is a congenital defect characterized by a lack of delamination and apical displacement of the tricuspid valve, tricuspid insufficiency, right atrial enlargement, and ventricular dysfunction. To analyze the results and evolution of the different surgical strategies, data were collected from 45 patients operated on during 1990-2018. Twenty-six patients were included with a median age at initial surgery of 11.3 years (range: 13 days-18.6 years). Procedures were plastic 10 patients (38%), cone technique reconstruction 11 (42%), and replacement 5 (19%). Additional interventions were required in 7 patients: cavo-pulmonary anastomosis 4 and Cox-maze 4. Nine patients (34.6%) required reoperation due to severe tricuspid insufficiency. Six had previous plastic, two bioprothesis, and one, cone. Overall mortality was 11.5% (3)at a median of 10.1 years post-surgery (range: 5.7-10.12) associated with arrhythmias (p = 0.05), right (p = 0.008), left (p = 0.0001) ventricular dysfunction and reoperations (p = 0.03). None were previous conus. Median follow-up was 6.5 years (range: 1-29.1). Ninety-one-point-six percent were in functional class I/II and 79.2% in sinus rhythm. The results of the different classic techniques were similar, although not free of complications and reoperations. Cone reconstruction proved to be effective, with low surgical mortality, less need for reoperations, and durability in the medium term.