Critical aortic stenosis has been a challenging congenital heart defect in the neonate commonly due to severe circulatory failure and multiple organ dysfunction. Since January, 1982, 20 neonates with a mean age of 5.6 +/- 1.6 days and weight of 3.25 +/- 0.1 kg underwent aortic commissurotomy. Early surgical intervention, cardiopulmonary bypass with hypothermia at 30 degrees C, careful assessment of the aortic leaflets, commissures and sinuses, and extensive commissurotomy short of causing aortic regurgitation, were essential principles of the operation. There were three operative deaths (15.0%) and three late deaths. One-year and 7-year survival rates are 74 +/- 10% and 69 +/- 11%. There were five reoperations for recurrent stenosis and two of these are late deaths. At 7 years 80 +/- 11% of patients remain free of a reoperation. Growth curves of survivors have been excellent with only two patients below the 5th percentile for both height and weight; 80% of the patients are totally asymptomatic. Despite substantial improvements in the treatments of most heart defects in neonates in the past decade, critical aortic stenosis still carries a malignant behavior with significant early mortality and the need for reoperations. Close follow-up of the patients is essential due to recurrence of the stenosis and progressive left ventricular hypertrophy, even when patients are totally asymptomatic.