Abstract

Background: The optimal strategy to treat symptomatic young infants with tetralogy of Fallot (TOF) is unclear. Objective: We performed a balanced comparison of staged repair (SR) vs. primary repair (PR) at two institutions that have exclusively adopted each strategy. Methods: Between 1993 and 2021, 143 consecutive infants under 4 months of age underwent systemic-to-pulmonary shunt at the Royal Children’s Hospital, Melbourne, Australia. These were compared with 122 consecutive infants in the same age group who underwent PR between 2004 and 2018 at the Children’s National Hospital, Washington, D.C. The primary outcome was mortality. Secondary outcomes were component and cumulative hospital stay, durations of perioperative support, hospital complications, and early and late reinterventions. Propensity-score matching was performed for risk adjustment. Mean follow-up was 9.3 years. Results: In the unmatched comparison, the SR cohort was younger, more commonly on prostaglandin infusion and mechanical ventilation, more likely to require urgent procedures, and had lower weight and smaller pulmonary annulus size. Hospital mortality after initial procedure (SR: 2.8% [4/143] vs. PR: 2.5% [3/122]; p = 0.86) and 10-year survival (SR: 95% [95% confidence interval (CI): 90 - 98%] vs. PR: 90% [95% CI: 81 - 95%]; p = 0.65) were similar. Hazard of reinterventions favored the PR group during early (<6 months) follow-up (hazard ratio: 0.22; 95% CI: 0.11 - 0.48; p < 0.001) but was similar at late follow-up. Most secondary outcomes cumulatively favored the PR group. Among 57 propensity-score matched pairs, the SR group had superior survival at 10 years (98% [95% CI: 88 - 99.8%] vs. 85% [95% CI: 69 - 93%]; p = 0.02). Conclusions: In this two-institutional, propensity-score matched comparison of SR vs. PR in symptomatic young infants with TOF, the SR strategy was associated with superior long-term survival and equivalent late reinterventions, at a cost of increased early reinterventions.

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