Anatomically corrected malposition of the great arteries--case reports and a review

Anatomically corrected malposition of the great arteries (ACM) is an extremely uncommon cardiac disease in which the great arteries arise from their appropriate ventricles despite abnormal spatial interrelations. Two patients with this malformation underwent successful surgical intervention for their associated anomalies. One was of situs solitus, d-loop and l-malposition and the other of situs inversus, l-loop and d-malposition of the great arteries. Fifty-three cases with accurate anatomical description were collected from the literature, and anatomical characteristics and associated anomalies were investigated. From the standpoint of anatomy and hemodynamics, we considered that ACM should be classified into atrioventricular concordant ACM, atrioventricular discordant ACM and ACM with situs ambiguus. Heart position and conus were variable. Dextrocardia and mesocardia were frequent. Bilateral conus was characteristic in concordant ACM, and subpulmonary conus was prominent in discordant ACM. The hypoplasia of the right heart and juxtaposition of the atrial appendages were in high incidence in cases of ACM. Surgical intervention was also discussed.