Review of surgical experience in 188 infants and young children with Ebstein anomaly

OBJECTIVES: This study sought to review our single-institutional surgical experience in paediatric Ebstein anomaly (EA). METHODS: We retrospectively reviewed the paediatric patients with EA undergoing operation between 2004 and 2020. The time-to-event analysis was studied using Kaplan–Meier estimates. Cox regression model was used to identify risk factors for recurrent moderate–severe or greater tricuspid regurgitation (TR). RESULTS: A total of 188 patients at a median age of 3.0 [interquartile range (IQR), 1.6–5.6] years were included, among whom 108 (57.4%) underwent cone reconstruction (CR). Bidirectional cavopulmonary shunt was required in 53 patients (28.2%). There were no in-hospital deaths. The median follow-up time was 5.6 (IQR, 2.9–8.9) years. Twenty-three (12.2%) developed recurrent moderate–severe or greater TR, among whom 9 required reoperation and 1 had late death. There was a lower incidence of recurrent TR (P = 0.006) and reoperation for TR (P = 0.037) in the CR group compared with the non-CR group. There was no difference in the incidence of recurrent TR (P = 0.61), reoperation (P = 0.9) and death (P = 0.48) among patients aged <1, 1–4 and 4–18 years. CONCLUSIONS: Acceptable outcomes can be anticipated in paediatric EA undergoing CR in terms of freedom from TR of > moderate degree at a mid-term follow-up.