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The clinical course of 38 patients with congenital mitral stenosis (MS) is reviewed. Associated cardiac defects were present in 28 patients, including tetralogy of Fallot in five. In all but one of the eight patients with supravalvar mitral ring (SVR), there were concomitant abnormalities of the mitral valve. Delay in the diagnosis of MS was common. Serial cardiac catheterizations and pulmonary pathologic examination indicated that pulmonary vascular obstructive disease develops during childhood. Mitral valve surgery was performed in 19 of 38 patients: valvotomy alone in eight, excision of SVR in five (two ofwhom also had valvotomy) and mitral valve replacemtnt in seven. Additional non-mitral cardiac surgery was performed in 18 patients. Overall surgical mortality was 49%; mortality for surgery on the mitral valve was 26%. Only patients having mitral valve replacement or with isolated SVR which was then resected became asymptomatic and had normal hemodynamics on postoperative catheterization.

SUMMARYSix patients with origin of one pulmonary artery from the ascending aorta are presented. Two are alive: a 21-year-old female, unoperated, with bilateral pulmonary vascular obstructive disease (PVO) and a 4-yearold girl who underwent successful correction at age 5 months. In addition, the data are reviewed from previous reports of 44 patients with this anomaly. Among the total of 50 patients, 42 had congestive heart failure in infancy. The right pulmonary artery arose anomalously in 44. A patent ductus arteriosus was present in 38 cases. At cardiac catheterization, pressure in both pulmonary arteries was generally at systemic level and the diagnosis was confirmed by aortography. Histologically, no significant PVO was observed in the majority of 18 infants who died, while significant changes, mainly in the "systemic" lung, were observed in 6 of 10 older children.Twenty-two patients have been operated on with 13 survivors. Anastomosis of the aberrant pulmonary artery to the main pulmonary artery directly or using a graft has been successful in 12 of 18 children. Corrective surgery should be performed as early as possible to prevent PVO.