Tricuspid valve surgery in Ebstein's anomaly patients achieved 96% survival at 10 years and 78% at 30 years with no survival difference between reconstruction and replacement, but postoperative complete atrioventricular block increased death or transplant risk by 3.5-fold.
Observational (n=398)
Yes
Does surgical intervention, specifically cone reconstruction and optimal timing, improve long-term morbidity and mortality in patients with Ebstein's anomaly compared to non-surgical management or older surgical techniques?
In patients with Ebstein's anomaly, surgical treatment provides excellent long-term survival, with cone reconstruction offering significantly lower risks of complete AV block and tricuspid regurgitation compared to older techniques.
Effect estimate: Survival at 10 years 96% (reconstruction) vs 100% (replacement), 20 years 93% vs 91%, 30 years 78% vs 91%, no significant difference (p > 0.9); CAVB increased risk of death/HTx (HR ~3.5; p=0.049) (95% CI 10yr survival 95% CI 93–100% (reconstruction), 100% (replacement); 20yr 88–99% vs 75–100%; 30yr 63–97% vs 75–100%)
p-value: p=0.025 for effect of CAVB on death/HTx; p>0.9 for survival difference between reconstruction and replacement; p=0.026 for interaction of age and CAVB increasing risk
Abstract Objectives To 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. Methods For 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. Results Non-surgical patients ( n = 194/49% of 398 patients) had less tricuspid valve regurgitation ( p 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. Conclusions This 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. Graphical Abstract
Neumann et al. (2026) conducted an observational in Patients with Ebstein’s anomaly registered in the German National Register for Congenital Heart Defects including both surgically and non-surgically managed patients (n=398). Tricuspid valve surgery including reconstruction (monocusp or cone reconstruction) and replacement vs. Non-surgical management or different surgical techniques (reconstruction vs replacement, cone vs monocusp reconstruction) was evaluated on Composite endpoint of all-cause death or heart transplant after first tricuspid valve surgery (Survival at 10 years 96% (reconstruction) vs 100% (replacement), 20 years 93% vs 91%, 30 years 78% vs 91%, no significant difference (p > 0.9); CAVB increased risk of death/HTx (HR ~3.5; p=0.049), 95% CI 10yr survival 95% CI 93–100% (reconstruction), 100% (replacement); 20yr 88–99% vs 75–100%; 30yr 63–97% vs 75–100%, p=p=0.025 for effect of CAVB on death/HTx; p>0.9 for survival difference between reconstruction and replacement; p=0.026 for interaction of age and CAVB increasing risk). Tricuspid valve surgery in Ebstein's anomaly patients achieved 96% survival at 10 years and 78% at 30 years with no survival difference between reconstruction and replacement, but postoperative complete atrioventricular block increased death or transplant risk by 3.5-fold.