Valvular heart disease in infants and children differs significantly from that seen in adults in etiology, operative indications, valve involvement, operative treatment, and results. One hundred and thirty children with isolated valvular lesions underwent operative correction at the Columbus (Ohio) Children's Hospital from 1960 to 1970. Over 90% had congenital rather than acquired heart disease. The pulmonary valve was most frequently involved with 70 patients undergoing valvulotomy and usually infundibulectomy for severe stenosis (gradient range, 60 to 160 mm Hg). Forty infants and children had congenital aortic stenosis and required valvulotomy with or without valvuloplasty (mean gradient, 92 mm Hg). Mitral valvuloplasty was carried out in six children for insufficiency, while one child underwent a closed commissurotomy for congenital stenosis. Prosthetic valve replacement was necessary in 13 children. The overall operative mortality was 7.7% (ten deaths).