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Background and aim of the study: Debate continues over the optimal timing for total repair of tetralogy of Fallot. Here, we report our experience with early one-stage total repair of tetralogy of Fallot. Methods: Between January 2000 and March 2008, surgical treatment was required in 24 symptomatic patients who were diagnosed with tetralogy of Fallot at less than three months of age. Among them, total repair was performed in 13 patients (group 1) and initial palliation was done in the others (group 2). The median follow-up duration was 20 months (5 to 40 months) and 67 months (1 to 100 months) in each group. Results: There was one operative death in group 2. The duration of mechanical ventilatory support, and the lengths of intensive care unit stay and hospital stay were not statistically different between both groups. The ventricular septal defects were repaired through the right atrium in 12 patients (12/13, 92.3%) of group 1 and nine patients (9/11, 81.8%) of group 2 (p = 0.576). Transannular repair was performed in six patients (6/13, 46.2%) of group 1 and 10 patients (10/11, 90.9%) of group 2 (p = 0.036). Reintervention or reoperation was required in two patients of group 1 and three of group 2 (p = 0.630). During follow-up, the pulmonary arterial growth was more prominent in group 1. Conclusions: Early one-stage total repair of tetralogy of Fallot can be performed safely without increasing the risk for reoperation or reintervention, in the short term. The pulmonary annulus is likely to be preserved when it is repaired at an early age. Early total repair may be better for the pulmonary arterial growth than staged repair. (J Card Surg 2010;25:394-399) [ABSTRACT FROM AUTHOR]