Cardiomyopathies: Classification, Diagnosis, and Treatment Bernhard Maisch, MD, FESC a, *, Michel Noutsias, MD b , Volker Ruppert, PhD b , Anette Richter, MD b , Sabine Pankuweit, PhD b By their historic definition, cardiomyopathies or myocardiopathies were defined as genuine heart muscle diseases. This definition excluded such causes as hypertension, congenital or valvular disease, a purely pericardial manifestation without epicardial inflammation, and also ischemic cardio- vascular disease. In the eighteenth century, the only heart muscle disease known in medicine was myocarditis, as in these ancient times even coro- nary artery sclerosis and infarction were unknown and if they occurred they were put into the basket of myocarditides. Around 1900, heart muscle diseases were mentioned as such, but it was not until 1960 that the term cardiomyopathy or myocar- diopathy was used for the first time. 1–3 Until 1980, several definitions for the term cardiomyopathies or myocardiopathies were in use until the World Health Organization/International Society and Federation of Cardiology (WHO/ISFC) Task Force defined it as “heart muscle diseases of unknown cause.” 4 The WHO/ISCF classification of 1996 expanded the term cardiomyopathies to all heart muscle diseases that lead to functional distur- bances of the heart. 5 Both classifications described 4 main phenotypes, which can be as- sessed by invasive and noninvasive imaging methods: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), restrictive cardiomyopathy (RCM), and unclassified forms. In 1996, right ventricular cardiomyopathy, previ- ously right ventricular dysplasia (ARVCM), was added (Fig. 1). These phenotypes were at that time thought to be primary cardiomyopathies, that is, heart muscle diseases of unknown cause. As secondary cardiomyopathies, heart muscle diseases of known causes, the task force included inflammatory heart muscle diseases (myocarditis, perimyocarditis), hypertensive cardiomyopathy, ischemic cardiomyopathy, and other forms of heart failure. Ischemic cardiomyopathy was defined as cardiac dysfunction of the nonischemic part of the heart muscle owing to overload following adverse This work was supported by a grant of the Bundesministerium fu ¨ r Wissenschaft und Forschung (BMBF) in the German Competence Net of Heart Failure (KNHI), by the Prof. Dr Reinfried Pohl Stiftung, by the Marburg Heart Foundation (VFDK) and the UKGM Foundation. The authors have nothing to disclose. a Division of Cardiology, Department of Internal Medicine, Faculty of Medicine, Philipps University, UKGM GmbH, Baldinger Street, 35043 Marburg, Germany b Division of Cardiology, Department of Internal Medicine, UKGM GmbH, Baldinger Street, 35043 Marburg, Germany * Corresponding author. E-mail addresses: maisch@staff.uni-marburg.de; bermaisch@aol.com KEYWORDS  Cardiomyopathies  Myocarditis  Hypertrophic cardiomyopathy  Right ventricular cardiomyopathy  Restrictive cardiomyopathy Heart Failure Clin 8 (2012) 53–78 doi:10.1016/j.hfc.2011.08.014 1551-7136/12/$ – see front matter Ó 2012 Elsevier Inc. All rights reserved. heartfailure.theclinics.com