Aortic atresia with Ebstein's and Uhl's anomaly in corrected transposition of the great arteries Clinicopathologic findings.

Abstract

The common form of aortic atresia is associated with atrioventricular and ventriculoarterial concordant connections, but it has been very rarely found in "discordant connection" in the literature. In a 6-day-old male infant, clinical and postmortem pathologic features of this rare lesion, associated with Ebstein's and Uhl's anomaly in "congenitally corrected transposition" in situs solitus, are described. A possible pathogenesis is suggested for the concomitant presence of aortic atresia and poor-functioning, morphologic right ventricle.