Surgical Repair of Anatomically Corrected Malposition of the Great Arteries

Anatomically corrected malposition of the great arteries ia 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.