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To evaluate the incremental risk of congenital heart disease on mortality after non-cardiac surgery in children.We reviewed the clinical information network database of the University Hospital Consortium for the period January 1, 1993, through December 31, 1996, and identified 191 261 patients <18 years old having 1 or more noncardiovascular surgical procedures. Of these patients, 6.5% had a diagnosis of congenital heart disease. Patients having ambulatory surgery are excluded from this database. Short-term (1-, 2-, and 3-day) and 30-day mortality were compared, as well as mortality for neonates, infants (31 days to 1 year), and older children (1-17 years); mortality for the 100 most common surgical procedures, mortality for 10 relatively minor surgical procedures, and mortality in subgroups of patients with minor versus severe cardiac diagnoses.Short-term and 30-day mortality was increased in the patients with congenital heart disease patients (30-day mortality odds ratio 3.5; 95% confidence limit, 3.2-3.9). Mortality was also increased in patients with congenital heart disease in the 2 youngest age groups, for the 100 most common operations, and for 10 relatively minor operations. Children with more severe heart disease diagnoses had higher mortality than did children carrying less serious cardiac diagnoses.A diagnosis of congenital heart disease adds significant incremental risk of mortality in children requiring inpatient noncardiovascular surgery. This outcome difference is present for both minor and major surgical procedures, and regardless of whether mortality is measured at 1, 3, or 30 days. The incremental risk is greatest in neonates and infants where the presence of congenital heart disease is associated with a 2-fold increase in mortality from noncardiac surgery.