Arterial repair for transposition of the great arteries and large ventricular septal defect in early infancy.

Arterial repair for transposition of the great arteries and large ventricular septal defect (VSD) may be superior to atrial correction, but the risk of either approach in early infancy has been high. The results of early correction were therefore assessed in 12 children with transposition of the great arteries and a large VSD who underwent arterial repair. Patients ranged in age from 3 to 90 days (median age, 19 days) and in weight from 2.5 to 3.7 kg. The VSD was infundibular in eight, inlet in three, and muscular in one. Coronary artery anomalies were present in five patients, including one patient with a single left coronary artery. There was one early death (8%) in the only patient with a pulmonary artery band. There were no late deaths. The 11 survivors have been followed up from 2 to 59 months (mean follow-up period, 20 months) and remain free of cardiac symptoms. Catheterization (n = 5) and Doppler echocardiography in all patients show no significant left ventricular outflow obstruction, aortic insufficiency, or residual VSD. Catheterization documented normal pulmonary artery pressure and unobstructed coronary arteries. Only one patient had significant pulmonary stenosis and underwent successful reoperation. These data indicate that arterial repair and VSD closure can be successfully performed in early infancy with low mortality and morbidity. Repair in this age group is advocated before changes of pulmonary vascular disease occur.