Outcomes of Treatment Pathways in 240 Patients with Congenitally Corrected Transposition of Great Arteries

Abstract Objective Congenitally corrected transposition of the great arteries (ccTGA) encompasses a diverse morphologic cohort, for which multiple treatment pathways exist. Understanding surgical outcomes among various pathways and their determinants are challenged by limited sample size, follow-up, and heterogeneity. We sought to investigate these questions with a large cohort of ccTGA patients presenting at different ages and representing the full therapeutic spectrum. Methods Retrospective review of 240 patients diagnosed with ccTGA from Cleveland Clinic coupled with prospective cross-sectional follow-up. Forty-six patients whose definitive procedure was completed elsewhere were excluded. Time-related survival was described among treatment pathways using actuarial, time-varying covariate, and competing risks analyses. Temporal trends in longitudinal valve and ventricular function were assessed using non-linear mixed-effects models. Results Median follow-up was 10 years. Seventy-nine patients underwent anatomic repair, 45 physiologic repair, 24 Fontan, 6 primary transplant. Forty patients managed expectantly had excellent long-term survival when considered from time of presentation, but benefited from ‘failures’ captured following transition to physiologic repair or transplant. Morphologic right ventricular (mRV) dysfunction after physiologic repair increased from 68% to 85% after 5 years, whereas morphologic left ventricular (mLV) function was stable in anatomic repair, especially with early surgery. Transplant-free survival at 15 years for anatomic and physiologic repair was 80% and 71%, respectively. Conclusions Early anatomic repair may be preferable to physiologic repair for select ccTGA patients. Late attrition after physiologic repair, however, represents ‘failure’ of expectant management and progressive tricuspid valve and mRV dysfunction compared to anatomic repair, where mLV function is relatively preserved.