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

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.