Abstract
Two children with suprawalvular mitra stenosis are reported. The first child, in addition to his cardiac malformation, had a perculiar facies, musculoskeletal deformities, and genitourinary anomalies. The clinical features were severe retardation of growth, microcytic hypochronic anemia, loud midsystolic ejection click over the pulmonary area, and electrocardiographic evidence of right ventricular hypertrophy. The second patient had intractable congestive heart failure, progressive cardiac enlargement, and severe right ventricular hypertrophy on electrocardiogram. In both patient, the angiocardiograms were found to be diagnostic in that showed shelflike structure above the mitral valve.
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