Abstract
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.
Highlights
Corrected transposition of the great arteries is characterized by discordant atrio-ventricular and ventriculo-arterial connections [1]
99 patients with corrected transposition of the great arteries (ccTGA) were included in the study
An additional congenital heart defect (CHD) was present in 81 patients (84.4%); most common were a ventricular septal defect (VSD) and a left ventricular outflow tract obstruction (LVOTO)
Summary
Corrected transposition of the great arteries (ccTGA) is characterized by discordant atrio-ventricular and ventriculo-arterial connections [1]. The morphologically right ventricle supports the systemic circulation and is described as a systemic right ventricle (SRV), while the morphologically left ventricle is the subpulmonary ventricle It is a rare congenital heart defect (CHD), with an estimated prevalence of 1 per 33,000 live births, accounting for approximately only 0.05% of all CHD [2]. One of the largest studies reported so far was an international multicenter crosssectional study that included 182 patients from 19 centers [4] It reported an increasing incidence of SRV dysfunction and clinical congestive heart failure, especially in those patients with associated lesions, such as a large ventricular septal defect [4]. The aim of this study was to describe a contemporary cohort of adult ccTGA patients and to evaluate predictors of outcome.
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