Congenitally Corrected Transposition of the Great Arteries in Adults-A Contemporary Single Center Experience

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart defect (CHD). Contemporary data regarding its outcome in adults are scarce. Methods: Retrospective, single-center study of all ccTGA patients over the age of 16 years treated at our center during the time period 2006-2018. Only patients with a biventricular circulation were included. The primary endpoint was all-cause mortality. Results: Altogether, 96 patients (mean age 32.8 +/- 16.0 years, female 50%) with ccTGA and a systemic right ventricle (SRV) were included in the study. An additional CHD was present in 81 patients (84.4%); most common were a ventricular septal defect (VSD) and a left ventricular outflow tract obstruction. Out of the whole cohort, 45 (46.9%) had already undergone cardiac surgery at baseline. During a median follow-up of 6.5 (IQR 2.8-12.7) years, the primary endpoint occurred in 10 patients (10.8%). Cause of death was cardiac in nine patients and suicide in one. Hospitalizations due to heart failure occurred in 48 patients (51.6%). Upon univariate Cox analysis, an NYHA class >= III, severe tricuspid regurgitation, severe SRV systolic impairment, as well as a reduced left ventricular systolic function were predictors of the primary endpoint. Upon multivariable analysis, only NYHA class >= III (HR: 18.66, CI 95%: 3.01-115.80, p = 0.0017) and a reduced left ventricular systolic function (HR: 7.36, CI 95%: 1.18-45.99, p = 0.038) remained as independent predictors. Conclusions: Adults with ccTGA and an SRV are burdened with significant morbidity and mortality. Predictors for mortality are NYHA class and subpulmonary left ventricular function.