Abstract
We experienced a family predisposed to familial cardiomyopathy in which four of five siblings died from cardiological factors, and we performed an extensive survey of linear and collateral consanguineous relatives of this family. Screening tests disclosed that 43.9% of the persons concerned had cardiological abnormalities, and 19.5% were diagnosed as hypertrophic cardiomyopathy. The main pathological findings in these cases were hypertrophy, various degenerative changes and disarray of myocardial cells as well as diffuse fine fibrosis of the interstitium. These findings were not much different from those in nonfamilial indiopathic hypertrophic cardiomyopathy (ICM). These pathological changes were correlated with electrocardiographic abnormalities such as ST-T changes, an LVH pattern, low voltage, and abnormal Q waves. In addition, some cases were complicated with coronary atherosclerosis. Since the number of aged people is anticipated to increase in future, the incidence of this complication will grow accordingly, which will no doubt make it more and more difficult to establish a diagnosis of ICM. Under such circumstances, a wide range of screening covering as many blood relatives members as possible and a long-term follow-up will become essential for the discovery of potential patients as well as from a prophylactic point of view. The onset and progress of familial cardiomyopathy is considered dependent not only upon the inheritance of genetic factors but also upon the mode of living.
