OBJECTIVES This study sought to review our single-institutionally surgical experience in paediatric Ebstein anomaly (pEA). METHODS We retrospectively reviewed the paediatric patients with Ebstein anomaly undergoing operation between 2004 and 2020. 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 (BCPS) was required in 53 patients (28.2%). There were no in-hospital death. 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 CR group compared with 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 less than 1 year old, patients with 1–4 and 4–18 years of age. CONCLUSIONS Acceptable outcomes can be anticipated in pEA undergoing CR in terms of freedom from TR of > moderate degree at a mid-term follow-up.

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Objectives. The tricuspid anterior leaflet is considered important in most repair techniques for Ebstein anomaly (EA). We aim to assess the anterior leaflet morphology using novel metrics and investigate the association of the morphology with recurrent moderately severe or greater tricuspid regurgitation (TR). Methods. Seventy-four paediatric patients with EA undergoing cone reconstruction (CR) between 2010 and 2021 were included. Anterior leaflet mobility (ALM) and anterior leaflet length (ALL) were remeasured on preoperative 2D echocardiography. The prediction accuracies of ALM and ALL-I (ALL indexed to body surface area) for recurrent TR were evaluated using receiver operating characteristic (ROC) curve analyses. Results. The median age of patients was 3.3 years (interquartile range, 1.9–7.1 years). Both ALM and ALL-I correlated with the Carpentier type and GOSH score. Nine patients (12.2%) developed recurrent TR during the one-year follow-up. By univariable logistic regression analyses, ALM (odds ratio [OR], 0.89; 95% CI [confidence interval], 0.82–0.96; p  = 0.003) and ALL-I (OR, 1.39; 95% CI, 1.08–1.78; p  = 0.011) were risk factors for recurrent TR. ROC curve analyses showed that ALM (AUC = 0.81) and ALL-I (AUC = 0.77) had better predictive performance for recurrent TR compared with the GOSH score (AUC = 0.68), the Carpentier type (AUC = 0.67), and preoperative TR severity (AUC = 0.58), and the combinations of ALM and ALL-I (AUC = 0.87) improved the predictive performance compared with ALM or ALL-I alone. Conclusions. ALM and ALL-I can help optimize evaluation in the anterior leaflet morphology and predict recurrent TR after CR in pediatric EA.