Introduction: Truncus arteriosus (TA) is a rare congenital cardiac anomaly requiring early surgical intervention, typically through either a single-stage neonatal repair {primary correction (PC)} or staged repair (SR), involving initial palliation followed by definitive correction. The optimal approach remains debatable. Methods A systematic search of multiple databases identified studies comparing SR and PC in TA. Pooled odds ratios (ORs), mean differences (MDs), and 95% confidence intervals (CIs) were calculated using RevMan v8.13.0. Time-to-event data for freedom from reoperation were reconstructed from published Kaplan–Meier (KM) curves, and pooled hazard ratios (HRs) with 95% CI were estimated using a Cox frailty model. Results: Five studies comprising 534 pediatric patients were included, with 274 (51.3%) in the SR cohort. SR was associated with a significantly lower incidence of unplanned surgical reinterventions following complete repair at the end of follow-up [OR 0.28; 95% CI 0.14-0.55; P = .02], while mortality at follow-up did not differ significantly between the approaches [OR 0.59; 95% CI 0.10-3.34; P = .32]. Reconstructed KM curve demonstrated greater freedom from reoperation in the SR group over 12 years [HR 0.39; 95% CI 0.17-0.89; P = .025]. Early perioperative outcomes, including cardiopulmonary bypass and aortic cross-clamp times, delayed sternal closure, intubation duration, early mortality, intensive care unit, and hospital stay, were similar between groups. Conclusion SR demonstrated fewer late unplanned reoperations over 12 years with comparable survival and perioperative outcomes to PC. However, these findings are based on low-certainty evidence from limited retrospective studies, warranting cautious interpretation.
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