Surgical management of congenital pulmonary valve dysplasia.

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.