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Objective: Ebstein's anomaly is a rare congenital heart malformation for which surgical and medical management are still controversial. The cone repair has transformed surgical outcomes in many of these patients. We aimed to present our results on the outcomes of patients with Ebstein's anomaly who underwent a cone repair or tricuspid valve replacement., Methods: A total of 85 patients who underwent a cone repair (mean age, 16.5 years) or tricuspid valve replacement (mean age, 40.8 years) between 2006 and 2021 were included. Univariate, multivariate, and Kaplan-Meier analyses were used to evaluate operative and long-term outcomes., Results: Residual/recurrent greater than mild-to-moderate tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement (36% vs 5%; P  = .010). However, at last follow-up, the risk of greater than mild-to-moderate tricuspid regurgitation was not different between groups (35% in the cone group vs 37% in the tricuspid valve replacement group; P  = .786). The tricuspid valve replacement group had a higher risk of tricuspid valve reoperation (37% vs 9%; P  = .005) and tricuspid stenosis (21% vs 0%; P  = .002) compared with the cone repair group. Kaplan-Meier freedom from reintervention was 97%, 91%, and 91% at 2, 4, and 6 years after cone repair, respectively, and 84%, 74%, and 68% at 2, 4, and 6 years after tricuspid valve replacement, respectively ( P  = .0191). At last follow-up, right ventricular function was significantly worse from baseline in the tricuspid valve replacement group ( P  = .0294). There were no statistical differences between age-stratified cohorts or surgeon volume in the cone repair group., Conclusions: The cone procedure offers excellent results, with stable tricuspid valve function and low reintervention and death rates at last follow-up. The rate of greater than mild-to-moderate residual tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement, but this did not expose the patient to a higher risk of reoperation or death at last follow-up. Tricuspid valve replacement was associated with a significantly higher risk of tricuspid valve reoperation and tricuspid valve stenosis, and worse right ventricular function at last follow-up., (© 2023 The Author(s).)

Objectives: Tricuspid valve (TV) surgery remains understudied and little data exist describing the surgical indications, outcomes, and prognostic factors for pediatric patients with non-Ebstein 2-ventricle congenital TV lesions. This study aims to describe early and late outcomes of pediatric patients with non-Ebstein congenital TV lesions undergoing isolated TV procedures at a single institution., Methods: All patients who underwent TV surgery for non-Ebstein congenital TV disease between 2006 and 2018 were included. Patients who had missing preoperative data, patients with single-ventricle physiology, congenitally corrected transposition of the great arteries, and patients undergoing TV intervention as part of repair of an atrioventricular canal defect were excluded. The primary end point was the occurrence of TV reintervention or TV regurgitation (TR) ≥ moderate., Results: A total of 85 patients were included. The tricuspid lesion was isolated TR in 80 (94.1%), isolated tricuspid stenosis in 3 (3.5%) and mixed disease in 2 (2.4%) patients. Median age at surgery was 33 years (interquartile range, 12-53 years). TV repair and TV replacement were performed in 66 (77.6%) and 19 (22.4%) patients, respectively. One (1.2%) patient underwent TV reoperation during the same admission. There was no in-hospital mortality. Median follow-up was 3.3 years (interquartile range, 0.1-4.7 years). The overall cumulative incidence of TV reintervention or TR deemed moderate or greater at 1, 3, and 5 years was 3% ± 2%, 11% ± 4%, and 20% ± 8%. In multivariable analysis, age younger than 12 years ( P  = .04) and mitral valve regurgitation deemed moderate or greater ( P  = .01) were independent risk factors for TV reintervention or recurrent TR deemed to be moderate or greater at last follow-up., Conclusions: TV surgery in patients with non-Ebstein congenital TV disease can be performed with good outcomes. TV reintervention or TR deemed moderate or greater occurred in 20% of patients on midterm follow-up. Patients younger than age 12 years are at higher risk for recurrent TR or TV reintervention, whereas preoperative MR deemed moderate or greater increases this risk, especially in patients older than age 12 years. There was no difference in outcomes between TV replacement and repair., (© 2022 The Author(s).)