[Importance of inheritance in cardiomyopathies (author's transl)]

Abstract

Inheritance plays an important role in the cardiomyopathies (i.e. dysfunction of the cardiac muscle of unknown origin). This especially has become obvious for the entity asymmetric septal hypertrophy of autosomal dominant inheritance (ASH), a cardiomyopaty with and without obstruction. But this is not the only type of cardiomyopathy of dominant transmittance as was shown by the demonstration of a kindred, in which echocardiographically no hypertrophy of the septum could be found. For it was not possible to find a uniformity for all the affected members of the kindred, for example a congestive course of the disease. Therefore it is suggested to name the disease without any prejudice "cardiomyopathy without asymmetric septal hypertrophy of dominant inheritance" to distinguish it from the "cardiomyopathy with asymetric septal hypertrophy of dominant inheritance". There also may occur a cardiomyopathy of autosomal recessive inheritance. This form could represent a large part of the "sporadic" cardiomyopathies and should challenge to search intensively for enzyme defects as a cause of the disease.