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

Abstract

A longitudinal study was made of 62 patients with dextrotransposition 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 intraatrial baffle repair after one year of age. Of the 47 patients with intact ventricular septum (IVS) treated in this manner, 41 (87%) were living at 3 mth of age, 36 (77%) at one year, and 31 (66%) at 2 yr 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 6 medical deaths within one week of BAS, three deaths during palliative operations, and 8 deaths associated with 31 Mustard procedures. Three patients with IVS and 5 with VSD developed pulmonary vascular obstructive disease, and 2 patients suffered strokes. In view of the relatively low mortality rate after the neonatal period and the good results with palliative surgery, the Mustard procedure is deferred 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.