BACKGROUND/PURPOSE Hospital mortality rate among children with hypoplastic left heart syndrome (HLHS) after cardiac repair is well documented, but comparable data after noncardiac, surgical procedures are unknown. The authors hypothesized an increasing number of noncardiac procedures were being performed on children with HLHS, less than 2 years of age, from 1988 to 1997, and that these procedures were associated with a substantial mortality rate. METHODS A retrospective review of hospital discharge data for 2,457 children less than 2 years of age with HLHS for 1988 through 1997 was performed. The authors examined the outcomes of HLHS children undergoing only noncardiac surgical procedures during their hospital stay. Differences in hospital mortality rates between 1988 through 1992 versus 1993 through 1997 were assessed using the Chi2 square statistic. RESULTS Nineteen percent of the 147 children with HLHS undergoing noncardiac, surgical procedures died (95% CI, 13% to 25%). Comparing the 2 study periods, there was no significant change in outcome among HLHS children undergoing noncardiac, surgical procedures (78% v. 83%; P >.1). There was no significant difference in the percentage of hospital discharges with noncardiac, surgical procedures performed per year. CONCLUSIONS Although children with HLHS were not undergoing an increase in the number of noncardiac surgical procedures performed annually, even minor surgical procedures were associated with considerable mortality. Outcomes after noncardiac surgery in high-risk children with congenital heart disease warrant further investigation.