ABSTRACT
This chapter describes the key features of the cardiomyopathies most likely to be encountered in everyday practice. In 1995, a classification scheme for the cardiomyopathies was proposed by the World Health Organization (WHO). A rare form of cardiomyopathy, first described in Japan, is apical ballooning or stress cardiomyopathy. In hypertrophic cardiomyopathy, the left ventricle (LV) is non-dilated; indeed, the LV cavity is usually small. The advent of molecular genetics has provided new insights into the pathophysiology of the cardiomyopathies, and in some respects the WHO classification is somewhat outdated. Arrhythmogenic right ventricular (RV) cardiomyopathy or dysplasia is a rare hereditary cardiomyopathy, primarily affecting the RV, in which there is loss of myocytes and replacement with fatty/fibrous tissue. The WHO classification also recognized an overlapping group of ‘specific’ cardiomyopathies, in which a particular case of cardiomyopathy could be attributed to a specific underlying aetiology.
