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Abstract
Tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries are a complex congenital heart defect. For years, our program has recommended early single-stage midline unifocalization at three to six months of age. However, many patients are referred beyond six months. Thus, we sought to evaluate surgical outcomes according to age at repair. We performed a retrospective review of patients who underwent unifocalization from age 3 to 12 months. These patients had not undergone prior surgery at our institution or elsewhere and were also not protocoled into early surgery. Patients were divided in the following groups: 3.0 to 4.9 (n = 61), 5.0 to 5.9 (n = 56), 6.0 to 7.9 (n = 56), and 8.0 to 11.9 (n = 47) months. Competing risk regression analyses were performed. We included 220 patients from 2001 to 2020. Baseline characteristics were not significantly different among the four groups. Overall, single-stage complete repair with bilateral unifocalization, ventricular septal defect closure, atrial septal defect closure, and right ventricular-pulmonary artery (PA) conduit placement was achieved at the index operation in 174 (79%) patients and did not differ across age groups. Early mortality was 4% (n = 9). At one year, 91% (200/220) of the entire cohort was fully septated. Comparing with group 1, group 4 was significantly less likely to undergo any PA reinterventions [hazard ratio (HR) 0.44, 95% CI 0.21-0.92, P = .028] or surgical PA reinterventions [HR 0.12, 95% CI 0.02-0.95, P = .044] following complete repair. Given the excellent outcomes across all ages, surgical timing for single-stage unifocalization should be dictated by clinical and anatomic details, with potential advantage in select clinically appropriate older infants who appear to be at a lower probability of PA reinterventions following full septation.
Published Version
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