Key result
Single-ventricle physiology in TAPVC repair is linked to ~3.7-fold higher mortality vs biventricular anatomy.
Why the study?
Children with single ventricle physiology and total anomalous pulmonary venous connection face high surgical risk and survival has plateaued despite advances in care.
Does single-ventricle physiology predict higher mortality, resource utilization, and pulmonary vein reintervention compared to biventricular anatomy in infants undergoing TAPVC repair?
Cohort (n=1,258)
Yes
Does single-ventricle physiology predict higher mortality, resource utilization, and pulmonary vein reintervention compared to biventricular anatomy in infants undergoing TAPVC repair?
Single-ventricle physiology in infants undergoing TAPVC repair is a major predictor of adverse outcomes, resulting in nearly 50% one-year mortality, higher reintervention rates, and a 5-fold increase in healthcare costs.
Answers are grounded in this paper and cite their sources.
SV physiology flags high-risk TAPVC infants for specialized care; hypothesis-generating for interventions to improve outcomes versus BiV anatomy.
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.
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Turner et al. (2026) conducted a cohort in Total anomalous pulmonary venous connection (TAPVC) (n=1,258). Single-ventricle physiology vs. Biventricular anatomy was evaluated on Survival at 1 year after repair (HR 3.69, p=<0.001). Single-ventricle physiology in infants undergoing TAPVC repair strongly predicted mortality (HR 3.69, P<0.001), with severely reduced 1-year survival compared to biventricular anatomy (52% vs 88%).
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