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

Background Children 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. Methods The Pediatric Health Information System was queried for TAPVC repairs (2015-2024). Infants were stratified by single-ventricle versus biventricular (BiV) anatomy. Results Among 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). Conclusions Single 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.