Transposition of the great arteries. Results of treatment with early palliation and late intracardiac repair.

A longitudinal study was made of 62 patients with dextro-transposition of the great arteries. The patients were managed with balloon atrial septostomy (BAS), palliative surgery if any operation was needed in the first year of life, and Mustard intra-atrial baffle repair after one year of age. Of the 47 patients with intact ventricular septum (IVS) treated in this manner, 41 (87 percent) were living at three months of age, 36 (77 percent) at one year, and 31 (66 percent) at two years of age. Survival rates were lower at each age for patients with associated ventricular septal defect (VSD). In relation to therapeutic interventions in the 62 patients, there were six medical deaths within one week of BAS, three deaths during palliative operations, and eight deaths associated with 31 Mustard procedures. Three patients with IVS and five with VSD developed pulmonary vascular obstructive disease, and two patients suffered strokes. In view of the relatively low mortality rate after the neonatal period and our good results with palliative surgery, we continue to defer the Mustard procedure to the second year of life. This is in contrast to the policy of some centers which now advocate elective intracardiac repair in the first year of life.