[2 families with dilatative cardiomyopathy]

Abstract

In two brothers each of two families congestive cardiomyopathy was diagnosed. Left ventricular biopsy showed individually differing degrees of hypertrophy of the myocardial cells together with endocardial fibrosis and circumscribed interstitial fibrosis. In family I clinical symptoms appeared at the beginning of the 4th decade, conduction disturbances being predominant. One brother died after rapid progression of the disease within one year. The other has been treated as an outpatient for 9 years; he received a pacemaker implant half a year ago. In family II initial symptoms appeared in the middle of the 6th decade, progressive heart dilation and insufficiency being the predominant characteristics. In the course of 7 years one brother has developed cardiac decompensation whereas the other brother's condition has remained nearly unchanged for 4 years. The degree of myocardial changes in left ventricular biopsies correlated with the clinical course.