Surgical repair of anatomically corrected malposition of the great arteries

Anatomically corrected malposition of the great arteries is a rare malformation in which the aorta and pulmonary artery arise from their appropriate ventricles but in an abnormal spatial relationship. This report describes 2 patients with anatomically corrected malposition who underwent closure of a ventricular septal defect and placement of a right ventricle-pulmonary artery conduit. A review of the literature indicates that surgical results have been good (92% survival) in those patients with situs solitus and atrioventricular concordance [S,D,L]. However, when there is atrioventricular discordance, that is, [S,L,D] or [I,D,L], hypoplastic right heart structures, or both conditions, the outcome after palliative procedures has been poor (29% survival). The results of surgical treatment should improve as this entity is recognized earlier and prompt surgical treatment is undertaken.