Between January 1967 and December 1988, 34 patients ranging in age from 3 to 18 years (mean, 14 +/- 3.6 years) underwent replacement of the aortic valve or root with their own pulmonary valve. The indication for operation was left ventricular outflow obstruction in 16 patients (47%), aortic regurgitation in 14 (41%), mixed aortic valve disease in 3 (9%), and failure of a previously implanted aortic homograft in 1(3%), There were four early deaths, all before 1971, giving a hosptial mortality of 11.8% (70% confidence interval, 6% to 20%). Surviving patients have been followed up a cumulative total of 214 patient-years, the longest period of observation being 16 years 8 months. Late mortality was 13.3% (70% confidence interval, 7% to 23%), and 4 other patients required removal of the pulmonary autograft for endocarditis. Actuarial rates at 16 years were 74% +/- 11% for freedom from reoperation on the left ventricular outflow tract, 80% +/- 10% for freedom from reoperation on the right ventricular outflow tract, and 77% +/- 10% for late survival. There was no instance of primary structural degeneration in the pulmonary autograft, and all surviving patients were in New York Heart Association functional class I without medication. This experience demonstrates that the pulmonary autograft can achieve good early and mediumterm results in young patients. Should growth potential be realized, it might constitute the ideal biological valve for the left ventricular outflow in children.