Congenitally corrected transposition of the great arteries

Abstract

Congenitally corrected transposition of the great arteries (cc-TGA, also known as L-transposition) is a rare condition that represents approximately 0.5% of congenital heart disease. It is characterized by atrioventricular and ventriculoarterial discordance. Patients with cc-TGA are at risk for cardiac arrhythmias due to abnormalities of the conduction system. We present an autopsy case of a 49-year-old man with cc-TGA who died unexpectedly, likely as a result of a fatal arrhythmia. This case illustrates the cardiac pathology characteristic of cc-TGA and describes how to grossly identify inversion of the atrioventricular valves.