An unusual case of congenitally corrected transposition of the great arteries associated with noncompaction-like remodeling of the morphological right ventricle.

Congenitally corrected transposition of the great arteries is a rare disease characterized by atrio-ventricular and ventriculo-arterial discordance (double discordance) and is associated with other cardiac anomalies in the majority of cases. The important associated anomalies include ventricular septal defect, abnormalities of left atrioventricular valve, subpulmonary stenosis, and conduction abnormalities. However, the noncompaction-like remodeling of the subaortic, morphologic right ventricle is not a commonly reported association. We, report a case of congenitally corrected transposition of the great arteries in a 40-year-old male patient, who had noncompaction-like remodeling of the morphologic right ventricle with severe ventricular dysfunction. He also had, left-sided Ebstein's anomaly, severe left atrioventricular valve regurgitation and a 2:1 atrioventricular conduction block.