Discordant Atrioventricular Connections 1: Congenitally Corrected Transposition

We first demonstrate the key features of congenitally corrected transposition, namely the discordant connections across both the atrioventricular and ventriculo-arterial junctions. The two discordant connections cancel each other out, so that potentially the circulations are congenitally corrected. In the heart shown in this videoclip, the septal structures are intact, so that indeed the circulations are congenitally corrected. Most usually, however, there are additional associated lesions that serve to uncorrect the circulatory patterns. The most common associated lesion is a malformation of the morphologically tricuspid valve, found in nine-tenths of autopsied specimens. An example of the usual lesion malforming the morphologically tricuspid valve is shown in the heart used for this demonstration, namely Ebstein's malformation, albeit that in this particular heart, the degree of apical displacement of the septal leaflet is minimal.

*Since filming, Children’s Memorial Hospital has been renamed Ann & Robert H. Lurie Children’s Hospital of Chicago and has been relocated to its new home in downtown Chicago.