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Young infants with severe tetralogy of Fallot: Early primary surgery versus transcatheter palliation.

BACKGROUND: Infants with severe tetralogy of Fallot may undergo (1) early primary surgical repair (EARLY) or (2) early transcatheter palliation (CATH) before delayed surgical repair. We compared these strategies with (3) elective single-stage tetralogy of Fallot repair (IDEAL).

METHODS: From 2000 to 2012, 453 children underwent tetralogy of Fallot repair (excluding systemic-pulmonary shunts), including 383 in the IDEAL (75%), 42 in the EARLY (9%), and 28 in the CATH (6%) groups. IDEAL repair at The Hospital for Sick Children occurs after 3 months. Risk-adjusted hazard analysis compared freedom from surgical or catheter reintervention. Somatic size, branch pulmonary artery size, and right ventricle systolic pressure were modeled using 2780 echocardiogram reports via mixed-model regression.

RESULTS: CATH involved right ventricular outflow tract stent in 18 patients, right ventricular outflow tract balloon in 9 patients, and ductal-stent in 1 patient. Three patients died (1 per group). Risk-adjusted freedom from surgical reoperation was 89% ± 4%, 88% ± 5%, and 85% ± 6% for the IDEAL, EARLY, and CATH groups, respectively, at 10 years. Patients in the EARLY and CATH groups had similar reoperation rates, except for neonates (<1 month), for whom EARLY repair conferred an increased risk of reoperation. Risk-adjusted freedom from catheter reintervention was lower in the EARLY group (76%) and especially for the CATH group (53%) at 10 years versus the IDEAL group (83%). Somatic growth and progression of right ventricle systolic pressure were similar among groups at 8 years. Although those undergoing EARLY (P = .02) and CATH (P = .09) tend to have smaller branch pulmonary arteries initially, late pulmonary artery size was not significantly different among groups.

CONCLUSIONS: Early primary repair for neonates may increase surgical reoperation, whereas transcatheter palliation comes at a cost of increased catheter reintervention. However, overall outcomes between groups, in terms of survival, growth, and hemodynamic parameters, were comparable, suggesting that both strategies are a reasonable option for children with severe tetralogy of Fallot.

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