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

Objective: To evaluate survival and reoperation after repair of transposition of the great arteries (TGA) by linking to administrative databases.

Methods: 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.

Results: 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).

Conclusions: 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.