Isolated congenital mitral stenosis.

ISOLATED congenital mitral stenosis is an extremely rare condition. Only 8 cases have been reported in the literature, whereas 37 cases are recorded in association with other cardiac anomalies, such as patent ductus arteriosus, aortic stenosis, coarctation of the aorta, and aortic valve anomaly.' This paper describes 2 female infants, 4 months and 3 months old, respectively, with isolated congenital mitral stenosis. The diagnosis was proved in one at autopsy; the second case was diagnosed clinically. The latter is alive and moderately well 16 months after mitral valvotomy. The first case of isolated congenital mitral stenosis was recorded by Summons in 1906.2 In 1953, Bower and associates3 reported the first clinically proved case of isolated congenital mitral stenosis. The lesion was suspected after cardiac catheterization and confirmed by angiocardiography and operation. The over-all prognosis is extremely poor; 4 cases died in the first year of life, 3 in the second, and 1 in the third year of life. Since the advent of surgery for mitral stenosis, 3 patients with congenital mitral stenosis have been operated upon, 2 with isolated congenital mitral stenosis and 1 with a large patent ductus arteriosus. The first 2 patients both died postoperatively, one 30 hours3 and the other 6 weeks later.4 The third patient was alive 7 months after the operation.