Primary cardiomyopathies are diseases intrinsic to the myocardium and are classified pathophysiologically into dilated, hypertrophic, restrictive, arrhythmogenic right ventricular, and unclassified.
ardiomyopathies are defined as diseases of the myocardium, which cause cardiac dysfunction with heart failure, arrhythmia, and sudden death. Cardiomyopathies represent a major cause of morbidity and mortality in both children and adults and are a frequent indication for cardiac transplantation. In 1995, the World Health Organization (WHO)/ International Society and Federation of Cardiology (ISFC) task force recommended that the cardiomyopathies be classified into two main groups: specific cardiomyopathies, and primary cardiomyopathies. In contrast, the primary cardiomyopathies are diseases intrinsic to the myocardium itself and are classified pathophysiologically. This group includes dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and unclassified cardiomyopathy.
Hughes et al. (2005) conducted a review in Inherited cardiomyopathy. Primary cardiomyopathies are diseases intrinsic to the myocardium and are classified pathophysiologically into dilated, hypertrophic, restrictive, arrhythmogenic right ventricular, and unclassified.