Abstract
Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital anomaly defined by double discordance – atrioventricular and ventricular-arterial. ccTGA frequently has associated lesions, which influence long-term survival.
The management of patients with ccTGA regarding the closure of defects and pacing strategies is often very challenging. Moreover, tricuspid valve regurgitation and its impact on the systemic right ventricle requires close monitoring and potentially timely interventions. In this paper, we present and discuss the management challenges raised by a patient with situs inversus totalis, dextrocardia, ccTGA, Ebstein anomaly of the tricuspid valve, atrial septal defect, and complete atrioventricular block.