OBJECTIVES: Primary repair is currently preferred for truncus arteriosus, however, staged repair may be useful when the lesion is complex. This study aimed to compare the mortality and reoperation rates of primary versus staged repair. METHODS: Nineteen patients undergoing primary repair and 30 undergoing staged repair between 1991 and 2021 were reviewed. The main indications for staged repair were moderate or greater truncal valve insufficiency, arch obstruction, and low weight (<
2.5 kg). RESULTS: The staged group included more patients with moderate or greater truncal valve insufficiency (primary vs staged, 11 vs 53%, P = 0.001) and more small patients (median, 3.4 kg vs 2.8 kg, P = 0.002). In the staged group, 26 patients (87%) achieved definitive repair with a median weight of 6.3 kg. The 15 year survival rate after initial surgery was 90% in the primary group and 90% in the staged group (P = 0.906). In patients with moderate or greater truncal valve insufficiency or arch obstruction, the survival rate was higher in the staged group than in the primary group (P = 0.024). There was no difference in the cumulative incidence of reoperation on the right ventricular outflow tract (72 vs 72% at 15 years, P = 0.448) or pulmonary artery branches (20 vs 38% at 15 years, P = 0.179). CONCLUSIONS: Truncus arteriosus can now be treated with low mortality risk. In patients with complex lesions, the use of a staged approach may yield similar results as in patients without complex lesions.