Long-Term Outcomes After Interrupted Aortic Arch Repair

BACKGROUND Interrupted aortic arch (IAA) is associated with left ventricular outflow tract obstruction (LVOTO) and DiGeorge syndrome. High-risk infantile surgery is required to address IAA, with limited data available on long-term outcomes. We used the Pediatric Cardiac Care Consortium, a multicenter US-based registry for pediatric cardiac interventions, to assess long-term outcomes after IAA repair by patient characteristics and surgical approach. METHODS This is a retrospective cohort study of patients undergoing IAA repair between 1982 and 2003. Kaplan-Meier plots and Cox proportional hazards regression were used to examine associations with post-discharge deaths tracked by matching with the US National Death Index. RESULTS Among 390 patients meeting inclusion criteria, 309 (79.2%) survived to discharge. Over a median follow-up of 23.6 years, 30-year survival reached 80.7% among patients surviving hospital discharge after initial IAA repair. Adjusted analysis revealed higher risk of death for type B vs. A (aHR 3.32, 95%CI: 1.48-7.44), staged repair (aHR 2.50; 95%CI: 1.14-5.50) and LVOTO interventions during initial hospitalization (aHR 4.12, 95%CI: 1.83-9.27), but not for LVOTO without need for interventions or presence of DiGeorge syndrome. There was a trend towards improved in-hospital and long-term survival over time during the study period. CONCLUSIONS Staged repair, type B IAA and need for LVOTO intervention during initial hospitalization for repair are associated with high risk of death out to 30 years. Survival outcomes are improving, but further efforts need to minimize staged approach and risks associated with LVOTO relief procedures.