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Long-Term Outcomes Following One-and-a-Half and Biventricular Repair for Ebstein Anomaly in Older Children and
Cassandra Nicheal Moses1, Putri Binti Yubbu1, Mohd Azhari Yakub2
1Department of Pediatrics, Faculty of Medicine and Health Science, University Putra Malaysia, Serdang, Selangor Darul Ehsan 43400, Malaysia.
Insights
One-and-a-half ventricular repair (1.5VR) offers long-term survival comparable to biventricular repair (BVR) for Ebstein anomaly (EA) in older children and adults. Preoperative low oxygen saturation and advanced NYHA class predict adverse outcomes, guiding surgical strategy.
Area of Science:
- Cardiology
- Congenital Heart Disease Surgery
- Pediatric Cardiac Surgery
Background:
- Ebstein anomaly (EA) is a rare congenital heart defect affecting the tricuspid valve.
- Surgical repair options include one-and-a-half ventricular repair (1.5VR) and biventricular repair (BVR).
- Long-term outcomes and predictors of adverse events in older children and adults with EA are not fully elucidated.
Purpose of the Study:
- To evaluate long-term survival and freedom from reoperation after 1.5VR and BVR in older children and adults with EA.
- To identify predictors of postoperative right ventricular (RV) dysfunction and mortality in these patients.
Main Methods:
- Retrospective review of 126 Ebstein anomaly patients who underwent surgery between 2005 and 2023.
- Kaplan-Meier method for survival and reoperation rates.
- Logistic and Cox regression analyses to identify predictors of RV dysfunction and mortality.
Main Results:
- 1.5VR (n=28) and BVR (n=98) showed comparable 10-year survival (87% vs 93.5%) and freedom from reoperation (94.5% vs 78%).
- Lower preoperative oxygen saturation predicted RV dysfunction.
- NYHA class IV, prior ablation, and cardiothoracic ratio >70% predicted mortality.
Conclusions:
- 1.5VR provides comparable long-term survival to BVR in older children and adults with EA, with a trend towards fewer reoperations.
- Careful preoperative assessment is crucial, considering predictors like low oxygen saturation, advanced NYHA class, prior ablation, and cardiomegaly to guide surgical strategy.
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.
