Evolving Concern: Late Outcomes after Repair of Transposition of the Great Arteries

J Thorac Cardiovasc Surg. 2024 Nov 29:S0022-5223(24)01113-9. doi: 10.1016/j.jtcvs.2024.11.032. Online ahead of print.

Abstract

Objective: Evaluate survival and re-operation after repair of transposition of great arteries (TGA) by linking to administrative databases.

Methods: Demographic, diagnostic and surgical data were retrieved from a surgical database for children born between 1990-2019 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 is complete to 12/2023 in 828 (98.8%) patients. In-hospital death was 3.8%. Survival probability at 30 years is 92%, not different between ASO versus other repairs. Freedom from cardiac re-operation is 82% at 30 years post-repair. Re-operation rates among ASO patients are highest within the first 2 years after the initial repair (CIF increase 0.85%/yr.), followed by 18 years of lower risk (0.25%/yr.), and then a late increase >20 years (0.48%/yr.). Coronary re-operations 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 is <2% for 93% of coronary artery patterns but is persistently 7x higher for 2LRCx and intramural pattern. Re-operation for neo-aortic valve and root problems occurred late (17.7 years).

Conclusions: Patient survival is excellent 3 decades after TGA repair. We found disconcerting evidence of a late increase in neo-aortic valve re-operations >17-years post-ASO due to enlarging neo-aortic roots and/or neo-aortic valve insufficiency. Future improvement following ASO hinges on better strategies for the subset of <7% with high-risk coronary artery patterns and avoiding neo-aortic root distortion.