Although the short-term results of atrial level repair of transposition of the great arteries (TGA) are satisfactory, longer follow-up has disclosed a significant incidence of systemic right ventricular dysfunction and rhythm disturbances. The arterial switch operation (ASO) may represent a major improvement by restoring the left ventricle as the systemic ventricle and avoiding extensive atrial surgery. We have prospectively evaluated 49 consecutive survivors of ASO for TGA with intact ventricular septum (IVS) by clinical examination, echocardiography, cardiac catheterization, ambulatory electrocardiographic monitoring, and invasive electrophysiologic studies. The mean length of follow-up has been 29 +/- 14 (SD) months after surgery. All children are currently asymptomatic and on no medications. Severe supravalvular pulmonary stenosis (greater than 60 mm Hg) was present in five children, all of whom have undergone reoperation. No patient has severe supravalvular aortic obstruction. Mild degrees of supravalvular pulmonary or aortic obstruction have not progressed. Seven children (14%) have trivial or mild aortic regurgitation. Two children have proximal occlusion of the left anterior descending coronary artery with adequate retrograde collateral perfusion. One child had an electrocardiographic pattern of inferior myocardial infarction without evidence of ventricular dysfunction. Systemic (left) ventricular function is normal as measured by end-diastolic pressure (mean 7 +/- 6 mm Hg), ejection fraction (mean 68 +/- 6%), end-diastolic volume (mean 101 +/- 22% of predicted normal), and cardiac index (mean 4.7 +/- 1.3 liters/min/m2). Only one patient has sinus node dysfunction. There have been no late deaths. These early results are encouraging. We conclude that the arterial switch operation is currently the procedure of choice for neonates with TGA and IVS.