American Heart Association, Circulation, Suppl_1(144), 2021
DOI: 10.1161/circ.144.suppl_1.12574
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Introduction: Neonates with symptomatic tetralogy of Fallot (sTOF) require early intervention with staged repair (SR) or primary repair (PR). As sTOF is commonly associated with genetic syndromes, we examined outcomes of SR vs. PR for syndromic sTOF neonates. Hypothesis: We hypothesized that syndromic neonates with sTOF who underwent SR will have lower early mortality compared to PR. Methods: Syndromic neonates with sTOF who underwent initial intervention (initial palliation followed by later SR, or PR) from 2005-2017 were reviewed. The primary outcome was death. Secondary outcomes included complications, and hospital and intensive care unit (ICU) lengths of stay (LOS). Unadjusted and propensity score adjusted outcomes were compared. Results: The 176 syndromic sTOF neonates consisted mostly of DiGeorge (35%), trisomy 21 (16%), and VACTERL (10%). There were 110 and 66 neonates in the SR and PR groups, respectively. DiGeorge syndrome, pulmonary atresia, prematurity, and pre-procedural ventilation were more common in the SR group. The adjusted overall hazard of death was similar between groups, but early mortality favored SR (Figure). Procedural complications were lower in the SR group at the index procedure, but SR is associated with longer cumulative hospital and ICU LOS (Table). Conclusions: In syndromic neonates with sTOF, there was an early survival benefit to SR compared to PR, although there was no overall survival difference. The cumulative cost of the SR strategy was longer hospital and ICU LOS. These findings do not suggest a single superior management strategy in the syndromic sTOF population.