Impact of Conotruncal Anomalies on Outcomes Following Total Cavopulmonary Connection

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All articles of this category (opens in new window) Background: The impact of conotruncal anomalies (CTAs) on long-term outcomes remains poorly described in the Fontan cohort of total cavopulmonary connection (TCPC). This study aimed to compare the outcomes of TCPC with and without CTAs in our large cohort. Methods: We reviewed all patients undergoing TCPC between 1994 and 2023, and patients with CTAs were identified. CTAs included transposition of the great arteries (TGA), double outlet right ventricle (DORV), tetralogy of Fallot (TOF), interrupted aortic arch type B (IAA), and truncus arteriosus communis (TAC). The impact of CTAs on survival, Failing Fontan, and other morbidities was evaluated. Results: Among 650 patients after TCPC, 291 (44.8%) patients had CTAs, including 257 TGAs, 101 DORVs, 3 TOFs, 3 IAAs, and 2TAC. The indication for the single-ventricle pathway was mainly unbalanced ventricles. CTAs were more frequently associated in patients with double inlet left ventricle (76.8 vs. 23.2%, p < 0.01) and single ventricle (80.5 vs. 19.5%, p < 0.01), and less frequently associated in patients with tricuspid atresia (25.7 vs. 74.3%, p < 0.01), compared to those without. Age at TCPC (median 2.5 vs. 2.2 years, p < 0.01), weight at TCPC (12.5 vs. 11.5 kg, p < 0.01), and association of heterotaxy (13.7 vs. 2.5%, p < 0.01), and previous pacemaker implantation (4.1 vs. 0.8%, p < 0.01) were higher in patients with CTAs, compared to those without CTAs. Pre-TCPC pulmonary artery pressure was similar between the groups (median 9 vs. 9 mmHg, p = 0.347). Pre-TCPC aortopulmonary collaterals (APCs) were less frequently observed in patients with CTAs than those without (27.4 vs. 38.9%, p < 0.01). Transplant-free survival was similar between the groups (92.3 vs. 95.7% at 10 years, p = 0.22), and freedom from Fontan failure was also similar between the groups (86.2 vs. 87.9%, p = 0.71). Sub-group analysis in patients with DILV, TA, unbalanced atrioventricular septal defect, and single ventricle, freedom from late tachyarrhythmia was lower in patients with CTAs compared to those without (79.7 vs. 95.6%, p = 0.05). Conclusion: CTAs were associated with 45% of the patients undergoing TCPC. Patients with CTAs were more frequently associated with heterotaxy syndrome, pre-TCPC pacemaker implantation, and less frequently with APCs at TCPC. Patients with CTAs did not have a demonstrable difference in long-term survival and freedom from Fontan failure, but showed a tendency to have late tachyarrhythmia more frequently. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

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