Background
Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart defect

(CHD). Contemporary data regarding the outcome in adults with CCTGA is scarce. The aim of this study

was to describe a contemporary group of adults with CCTGA and evaluate predictors of outcome.

This retrospective, single centre study included all patients with CCTGA and an age over 16

years, who were treated at our tertiary centre during the time period 2006-2018. Time point of

inclusion was the first appointment during the study period, if the patient was already 16 years of age or

older or the first appointment after the 16th birthday, if the pat. reached this age threshold during the

study period. Clinical data, including survival status, morbidities, hospitalisations, need of operations or

interventions, as well as need for pacemaker, were retrieved from hospital records. The primary

endpoint was death from any cause.

Altogether, 100 patients were included, 96 with a systemic right ventricle, 3 with anatomical repair und 1

palliated with aorto-pulmonary shunt. Mean age at baseline was 32.3 ± 15.8 years, while median follow

up was 6.5 years (IQR 2.6-12.8). There were 11 deaths, 10 due to cardiac causes, 1 due to suicide.

Three patients underwent heart transplantation, two of them died. During the follow-up, 67 patients were

hospitalized, 52 due to heart failure. On univariate Cox analysis, age at baseline (hazard ratio (HR):

1.043, CI 95%: 1.002-1.085, p=0.039), tricuspid regurgitation (HR: 2.652, CI 95%: 1.548-4.542,

p<0.001), systemic ventricular function (HR: 3.486, CI 95%: 1.658-7.327, p=0.001), NYHA class (HR:

4.566, CI 95%: 2.194-9.504, p<0.001), and presence of sinus rhythm (HR: 0.252, CI 95%: 0.073-0.872,

p=0.030) were predictors of the primary endpoint. On multivariable analysis, only tricuspid regurgitation

(HR: 2.324 , CI 95%: 1.128-4.789, p=0.022), and NYHA class (HR: 3.052, CI 95%: 1.459-6.382,

p=0.003) remained as independent predictors.

Adults with CCTGA are burdened with significant morbidity and mortality. Predictors for death

are the severity of tricuspid regurgitation and functional class.