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[Classification of cardiomyopathies according to the WHO/ISFC Task Force--more questions than answers?]
Insights
The 1995 WHO/ISFC cardiomyopathy classification includes new forms like arrhythmogenic right ventricular cardiomyopathy and inflammatory cardiomyopathy. It clarifies terms for ischemic, hypertensive, and valvular cardiomyopathies, aiding heart muscle disease diagnosis.
Area of Science:
- Cardiology
- Pathology
Context:
- The 1995 World Health Organization/International Society and Federation of Cardiology (WHO/ISFC) classification of cardiomyopathies defines heart muscle diseases based on cardiac function disturbances.
- It categorizes cardiomyopathies into five primary forms: dilated (DCM), hypertrophic (HCM), restrictive (RCM), arrhythmogenic right ventricular (ARVCM), and non-classified (NKCM), based on hemodynamic criteria.
Purpose:
- To outline the updated WHO/ISFC classification of cardiomyopathies established in 1995.
- To introduce new entities and clarify existing definitions within cardiomyopathy classification.
Summary:
- The 1995 classification introduced arrhythmogenic right ventricular cardiomyopathy (ARVCM) as a distinct entity.
- It refined terms such as ischemic cardiomyopathy (focusing on non-infarcted myocardium remodeling), hypertensive cardiomyopathy (left ventricular hypertrophy in hypertension), and valvular cardiomyopathy (cardiomegaly beyond valvular dysfunction).
- Inflammatory cardiomyopathy was defined as myocarditis with cardiac dysfunction, linked to infectious or autoimmune factors. Recent consensus clarified criteria for inflammatory dilated cardiomyopathy (DCMi) and diagnostic standards for endomyocardial biopsies.
Impact:
- Provides a standardized framework for classifying heart muscle diseases, improving diagnostic accuracy and communication among clinicians and researchers.
- Facilitates further research into the specific etiologies and pathogenetic mechanisms of different cardiomyopathy subtypes, particularly inflammatory and arrhythmogenic forms.
- Establishes criteria for diagnosing inflammatory cardiomyopathies and guides molecular and virological diagnostic approaches in endomyocardial biopsies.
Abstract:
The most recent WHO/ISFC classification of cardiomyopathies (1995) describes as cardiomyopathies all heart muscle diseases, which demonstrate a disturbance of cardiac function. It distinguishes primarily according to hemodynamic criteria the following 5 forms: 1. dilated (DCM), 2. hypertrophic (HCM), 3. restrictive (RCM) from 4. arrhythmogenic right ventricular (ARVCM) and assembles in 5. non-classified cardiomyopathies (NKCM) the non-classifiable forms. When compared to the 18-year-old former classification several points have been altered: 1. ARVCM has been introduced as a new entity. 2. The new term ischemic cardiomyopathy has been reserved for the remodeling process of the non-infarcted myocardium and does not mean hemodynamic alterations of an infarcted area (aneurysm), of stunned or hibernating myocardium. Hypertensive cardiomyopathy corresponds to left ventricular hypertrophy in hypertensive patients, valvular cardiomyopathy identifies cardiomegaly, which cannot sufficiently be explained by the valvular dysfunction (stenoses or insufficiency) alone. For the first time the term inflammatory cardiomyopathy has been used and defined as acute or chronic myocarditis associated with cardiac dysfunction, for which etiological and pathogenetic factors, e.g. viral or microbial infection or autoimmune processes have been made responsible. Two ISFC task forces have just recently clarified in consensus conferences the immunohistopathological criteria for chronic myocarditis or dilated cardiomyopathy with inflammation (DCMi: > 14 lymphocytes or macrophages/mm3) and set standards for molecular and virological diagnoses in endomyocardial biopsies.
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