Single-Ventricle Physiology as a Dominant Determinant of Mortality, Resource Utilization, and Pulmonary Vein Reintervention in Total Anomalous Pulmonary Venous Connection: A Large Multicenter Study.
Durable LVAD outcomes in Japan improved substantially with HeartMate 3, and the residual failure modes are right-sided.
The third J-MACS report, 1,859 patients from 2011 to 2025 , shows 5-year survival of 84% in the post-2019 era versus 76% before, with significant gains in freedom from pump thrombosis, stroke, major bleeding, and pump exchange.
BackgroundChildren with single ventricle (SV) physiology and total anomalous pulmonary venous connection (TAPVC) face high surgical risk. Despite advances in single ventricle care, survival in SV/TAPVC patients has plateaued. We investigated multicenter outcomes, reintervention rates, and healthcare burden in this complex population.MethodsThe Pediatric Health Information System was queried for TAPVC repairs (2015-2024). Infants were stratified by single-ventricle versus biventricular (BiV) anatomy.ResultsAmong 1258 infants undergoing TAPVC repair, 10% (N = 120) had SV and 90% (N = 1138) had BiV anatomy. SV/TAPVC patients showed higher prematurity (11% [13/120] vs 8% [91/1138], P < .001), low birthweight (14% [17/120] vs 9% [102/1138], P < .001), and neurologic abnormalities (13% [16/120] vs 5% [60/1138], P < .001). Postoperatively, SV/TAPVC had increased delayed sternal closure (11% [13/120] vs 4% [45/1138], P < .001) and ECMO use (7% [8/120] vs 3% [34/1138], P < .001). Healthcare burden was substantially higher in SV/TAPVC children: 5-fold longer hospitalizations (102 vs 29 days, P < .001), 6-fold more hospital visits (6 vs 1, P < .001), and 5-fold higher costs ($1,019,390 vs $259,303, P < .001). Survival at 1 year after repair was severely reduced (SV: 52% vs BiV: 88%, P < .001). Pulmonary vein reinterventions were substantially higher (12-month cumulative incidence: 32% vs 5%, P < .001). Single ventricle physiology was a strong mortality predictor (HR: 3.69, P < .001), particularly pulmonary atresia with intact septum (HR: 6.62, P < .001) and hypoplastic left heart syndrome (HR: 4.42, P < .001).ConclusionsSingle ventricle physiology remains a powerful predictor of adverse outcome in children with TAPVC, with 1-year survival rates approaching 50%. Children with SV/TAPVC face substantially longer hospitalizations, higher reintervention rates, higher hospital visits, and overall 5-fold greater healthcare costs.
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