Anatomically corrected malposition of the great arteries: diagnostic difficulties and surgical repair of associated lesions.

Four patients, aged 5 to 14 years, had repair of abnormalities associated with anatomically "corrected malposition," a condition associated with mitral/aortic discontinuity with atrioventricular and ventriculoarterial concordance, and with the aorta lying anterior and to the left of the pulmonary artery. All 4 patients had ventricular septal defect; in addition, 1 patient had coarctation, 2 patients had pulmonary infundibular stenosis, 1 patient had acquired pulmonary valve atresia, 1 patient had acquired fixed subaortic stenosis and 2 patients had left juxtaposition of the atrial appendages. The position of the great arteries suggest corrected transposition, but the true diagnosis is made from finding atrioventricular and ventriculoarterial concordance with wide mitral/aortic separation on the cross-sectional echocardiogram. The electrocardiogram shows normal ventricular activation. Three of the 4 patients had anticlockwise rotation of the heart, which displaced the apex. This made echocardiography difficult and caused a problem in visualizing the right-sided valved conduit and closing the VSD in conventional ways. One patient died at reoperation for an obstructed conduit. Mitral regurgitation developed after resection of subaortic stenosis in 1 patient. No arrhythmias occurred in the 3 surviving patients, followed for 1 to 5 years, but the risk of subaortic stenosis remains.