Long-term outcomes following one-and-a-half and biventricular repair for Ebstein Anomaly in older children and adults: a single-centre experience

Objectives: To evaluate long-term survival and freedom from reoperation following one-and-a-half (1.5VR) and biventricular repair (BVR) in older children and adults with Ebstein anomaly (EA) and to identify predictors of postoperative right ventricular (RV) dysfunction and mortality. Methods: We retrospectively reviewed EA patients who underwent surgery at the National Heart Institute between 2005 and 2023. Clinical, imaging, and surgical data were analysed. Survival and reoperation rates were assessed with Kaplan-Meier method, while logistic and Cox regression were applied to identify predictors of RV dysfunction and mortality. Results: Among 126 EA patients [median age: 19 years (IQR: 11, 33.2)], 98 (77.8%) underwent BVR and 28 (22.2%) underwent 1.5VR. Compared with the BVR group, 1.5VR patients were younger [9.5 vs 15.6 years, P=.03], had lower oxygen saturation, more severe Carpentier types (C/D), and poorer RV function [FAC 25.4% vs 32.6%, P=.01; TAPSE 13.3 vs 17.5 mm, P=.006]. Overall mortality was 6.3%. Ten-year survival rates were 93.5% for BVR and 87% for 1.5VR, while freedom from reoperation rates were 78% and 94.5%, respectively (P>.05). Twelve patients (12.2%) initially intended for BVR required conversion to 1.5VR due to RV failure, with 2 (16.7%) cases of mortality. Independent predictors were: lower preoperative oxygen saturation for postoperative RV dysfunction (OR 0.92, 95% CI 0.85-0.99, P=.04); and New York Heart Association (NYHA) class IV (HR 28.9, P=.035), prior ablation (HR 6.5, P=.02), and cardiothoracic ratio >70% (HR 16.8, P=.004) for mortality. Conclusions: In older children and adults with EA, 1.5VR provided long-term survival comparable to BVR, with a trend towards fewer reoperations. Low oxygen saturation, advanced NYHA class, prior ablation, and severe cardiomegaly independently predicted adverse outcomes, highlighting the need for careful preoperative assessment to guide surgical strategy.

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