Classification and Definitions of Cardiomyopathies

Abstract

Cardiomyopathies are an important and heterogeneous group of diseases. The awareness and knowledge of these diseases in both the public and medical communities historically has been impaired by persistent confusion surrounding definitions and nomenclature. Classification schemes, of which there have been many, (Thiene et al., 2000, 2004; Richardson et al., 1996) are potentially useful in drawing relationships and distinctions between complex disease states for the purpose of promoting greater understanding; indeed, the precise language used to describe these diseases are profoundly important. Cardiomyopathies are diseases of the heart muscle, characterized by abnormality in chamber size and wall thickness, or functional contractile dysfunctions mainly systolic or diastolic dysfunction in the absence of coronary artery disease, hypertension, valvular disease, or congenital heart disease (Elliott et al., 2008). These diseases are classified as either primary or secondary. Primary cardiomyopathies consist of disorders solely or predominantly confined to the heart muscle, which have genetic, non-genetic, or acquired causes. Secondary cardiomyopathies are disorders that have myocardial damage as a result of systemic or multiorgan disease (Maron et al., 2006). Cardiomyopathies are classified traditionally according to morphological and functional criteria into four categories: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM) and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). These cardiomyopathies can be primary myocardial disorders or develop as a secondary consequence of a variety of conditions, including myocardial ischemia, inflammation, infection, increased myocardial pressure or volume load and toxic agents. The definitions of cardiomyopathies presented here are in concert with the molecular era of cardiovascular disease and have direct clinical applications and implications for cardiac diagnosis. However, the classification of cardiomyopathies presented herein is not intended to provide precise methodologies or strategies for clinical diagnosis. Rather, the classification of cardiomyopathies represents a scientific presentation that offers new perspectives to aid in understanding this complex and heterogeneous group of diseases and basic disease mechanisms.