Analysis of the mode of transmission of right ventricular dysplasia

摘要: A formal analysis of the mode transmission right ventricular cardiomyopathy was performed in seven families with this condition. Ninety-six subjects (81 family members, 15 connected) were studied. The index cases members who had died suddenly their youth autopsy evidence massive fibrous-adipose myocardial replacement. Pedigree showed that 58 per cent affected, a male predominance (63% men vs 53% women). kindreds all normal and none both parents affected. Carrier states observed males females vertical demonstrated. Clinically, disease very variable some showing widespread involvement or without cardiomegaly, other localised abnormalities. These data are consistent congenital an autosomal dominant inheritance incomplete penetrance expression.