ArticleClinical research in cardiology : official journal of the German Cardiac Society2026
Risk factors for morbidity and mortality in Ebstein's anomaly: a registry-based study of 398 patients.
Article in Clinical research in cardiology : official journal of the German Cardiac Society, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
objectivesTo investigate the long-term outcomes and risk factors for morbidity and mortality in patients with Ebstein's anomaly, including the effects of type and timing of valve surgery.
methodsFor this retrospective, record-based study, all patients with Ebstein's anomaly enrolled in the German National Register for Congenital Heart Defects up to June 2021 were eligible for inclusion.
resultsNon-surgical patients (n = 194/49% of 398 patients) had less tricuspid valve regurgitation (p < 0.001) and heart failure symptoms (p < 0.001) than surgical patients (n = 204/51%). Postoperative survival at 10, 20, and 30 years was 97%, 93%, and 80%. Eighty-one (40%) patients underwent multiple surgeries. Re-operation rates were lowest in patients with first valve surgery during adolescence (p = 0.0076). Postoperative NYHA class > I was more frequent with surgery delayed to older age (p < 0.001). Initial corrective surgery was complicated by complete atrioventricular block (CAVB) in 17 (9%) of patients. CAVB was more likely with older age (p = 0.001), and tricuspid valve replacement compared to reconstruction (p = 0.029). CAVB was associated with all-cause death (p < 0.001). Cone reconstruction reduced the risk of CAVB (p = 0.008) and tricuspid valve regurgitation (p < 0.001) compared to monocusp reconstruction.
conclusionsThis registry-based study of Ebstein's anomaly corroborates good surgical long-term results, while re-operation rates remain high. Patients operated before adolescence were at the highest risk of re-operation, while older age at the time of the first surgery increased the risk of CAVB. The cone reconstruction was associated with improved tricuspid valve function and a lower risk of CAVB compared to monocusp reconstructions. Choosing an optimal time window for surgery and use of the cone reconstruction may therefore further improve outcomes.
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