Congenitally corrected transposition of the great arteries (ccTGA) is rare congenital heart disease (CHD) with an estimated prevalence of less than 1% among all CHDs. Long time prognosis is dependent on the systemic right ventricule function, presence of heart block and surgical correction.

In this retrospective study, all cases of ccTGA from 2 tertiary centers (one university hospital, one private pratice) were analyzed starting from 1980. Diagnosis circumstances, associated lesions, apparition of conduction blockage, surgery (anatomical versus arterial switch), systemic ventricular dysfunction, cardiac magnetic Imaging (CMR) and cardiopulmonary exercise testing (CET) were retrieved from patient records.

Eighty-two patients were included with a mean follow up time of 23.3 years (Interquartile range (IQR)=25.6 years). Survival rate was 99% CI95% [96–100] at 5 years, 97% CI95% [93–100] at 10 years and 90% CI95% [81–98] at 20 years. Patients had complete heart block after a mean time of 9.7 years (IQR 14.3 years) with a prevalence of 28% CI95% [8.4–44] at 30 years. Patients with systemic right ventricule had heart failure after a mean time of 52.7 years CI95% [42.6–100]. The ejection fraction of the systemic right ventricule based on CMR was correlated with VO2 uptake (ρ=0.49, P=0.05) at CET.

Lifetime follow-up is necessary for the diagnosis of complications of ccTGA, specifically complete heart block and systemic right ventricle dysfonction. Right heart ejection fraction with CMR is correlated and could be used as a surrogate of peak VO2 uptake at CET.