Corrected transposition in situs inversus. Biventricular repair of associated cardiac anomalies.

Corrected transposition of the great arteries (C-TGA) in situs inversus [I,D,D] is a rare variant of C-TGA (5-8% of the cases). Few anatomic and surgical data on this anomaly have been published. Between 1974 and 1990, 13 such patients (mean age, 12.7 +/- 8.6 yr) underwent repair of associated cardiac anomalies, including ventricular septal defect (VSD) and pulmonary outflow tract obstruction (POTO) in all 13 patients and atrial septal defect (ASD) in 11. Systemic venous anomalies were present in four. Nine patients also had dextrocardia. Patches to close the VSD were placed on the right side of the ventricular septum (through a right [systemic] ventriculotomy) in two patients and on the left side of the ventricular septum in the other 11 via a left ventriculotomy in one or a morphological right (left-sided) atriotomy in 10. The POTO was relieved directly in five patients and bypassed by a left ventricle-pulmonary artery conduit in the other eight. There was one early death due to cerebral hemorrhage. Two patients required a pacemaker for permanent heart block. Other rhythm disturbances included transient heart block in three patients and late atrial fibrillation in one. All the survivors are asymptomatic as late as 15.5 years after the repair. One patient had conduit revision 10.5 years after the initial operation, two had residual POTO of greater than 40 mm Hg, and none had residual VSD. In conclusion, patients with [I,D,D] C-TGA present with a relatively uniform subset of anatomic lesions, including predominantly ASD, VSD, and POTO.(ABSTRACT TRUNCATED AT 250 WORDS)