Evolving concern: Late outcomes after repair of transposition of the great arteries.

Abstract

To evaluate survival and reoperation after repair of transposition of the great arteries (TGA) by linking to administrative databases. Demographic, diagnostic, and surgical data were retrieved from a surgical database for children born between 1990 and 2019 who underwent TGA repair at a single institution and were linked to administrative databases. TGA repair was performed in 838 patients, among whom 791 (94%) had an arterial switch operation (ASO), 25 a Rastelli, 10 Mustard, and 9 repaired by aortic translocation. Follow-up was complete to December 2023 in 828 (98.8%) patients. In-hospital death was 3.8%. Survival probability at 30 years was 92%, not different between ASO versus other repairs. Freedom from cardiac reoperation was 82% at 30 years postrepair. Reoperation rates among patients who underwent ASO were greatest within the first 2 years after the initial repair (cumulative incidence function increase 0.85%/year), followed by 18 years of lower risk (0.25%/year), and then a late increase >20 years (0.48%/year). Coronary reoperations occurred early (median 3.3 months). Single coronary pattern from sinus 2 occurred in 56 patients with an in-hospital mortality of 10.7%. Contemporary operative risk was <2% for 93% of coronary artery patterns but persistently 7 times greater for 2LRCx and intramural pattern. Reoperation for neoaortic valve and root problems occurred late (17.7 years). Patient survival was excellent 3 decades after TGA repair. We found disconcerting evidence of a late increase in neoaortic valve reoperations >17-years post-ASO attributable to enlarging neoaortic roots and/or neoaortic valve insufficiency. Future improvement after ASO hinges on better strategies for the subset of <7% with high-risk coronary artery patterns and avoiding neoaortic root distortion.