Survival Following Single-Stage Repair of Truncus Arteriosus and Associated Defects.

BACKGROUND: The goal of this study is to describe in-hospital and long-term mortality following single-stage repair of truncus arteriosus communis (TAC) and explore factors associated with these outcomes.

METHODS: This is a cohort study of consecutive patients undergoing single-stage TAC repair between 1982 and 2011 reported to the Pediatric Cardiac Care Consortium registry. In-hospital mortality was obtained for the entire cohort from registry records. Long-term mortality was obtained for patients with available identifiers via matching with the National Death Index through 2020. Kaplan-Meier survival estimates were created for up to 30 years post-discharge. Cox regression models estimated hazard ratios for the associations with potential risk factors.

RESULTS: 647 patients (51% male) underwent single-stage TAC repair at a median age of 18 days. 53% had type I TAC, 13% had interrupted aortic arch and 10% underwent concomitant truncal valve surgery. Of these, 486 (75%) survived to hospital discharge. Post-discharge, 215 patients had identifiers for tracking long-term outcomes; 30-year survival was 78%. Concomitant truncal valve surgery at index procedure was associated with increased in-hospital and 30-year mortality. Concomitant interrupted aortic arch repair was not associated with increased risk for in-hospital or 30-year mortality.

CONCLUSIONS: Concomitant truncal valve surgery but not interrupted aortic arch was associated with higher in-hospital and long-term mortality. Careful consideration of the need and timing for truncal valve intervention may improve TAC outcomes.