Complex transposition with interrupted right aortic arch and partial Di George syndrome: successful palliation with combined medical and surgical therapy.

Abstract

A five-day-old infant with transposition of the great arteries, ventricular septal defect, and an interrupted right aortic arch underwent successful balloon septostomy, pulmonary artery banding, and aortic arch repair. The infant also had abnormal facies with severe refractory hypocalcemia and depressed T-lymphocyte number and function believed to represent a partial Di George syndrome. The hypocalcemia resolved following treatment with a vitamin-D analogue, T-cell number increased, and T-cell function improved, but both remained subnormal.