title: Surgical management of congenital pulmonary valve dysplasia.
abstract: Pulmonary valvular stenosis secondary to congenital valve dysplasia differs markedly from the classic variety of pulmonary stenosis. The reported mortality of patients treated by standard commissurotomy is 38 to 66%. The clinical features and operative management of 14 patients with dysplastic pulmonary valves are reviewed. Three groups of patients were studied. Group 1 consisted of 5 patients treated by commissurotomy. Group 2 comprised 3 patients treated by partial excision of the valve. In neither group were there operative deaths, but 5 of the 8 patients developed recurrent stenosis; 3 required reoperation. In 1975, because of the high incidence of recurrent stenosis, total valvectomy was begun. Ten patients (Group 3) have undergone valvectomy with 1 death. Nine patients were doing well at 3 to 15 months of follow-up. Based on the reported mortality and present findings, total excision of the valve is recommended for relief of stenosis in pulmonary valve dysplasia.
mesh_terms: D000293:Adolescent; D006332:Cardiomegaly / Q000000981:diagnostic imaging / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D006344:Heart Septal Defects, Atrial / Q000000981:diagnostic imaging / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D011664:Pulmonary Valve / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D011666:Pulmonary Valve Stenosis / Q000000981:diagnostic imaging / Q000601:surgery; D011859:Radiography