Definition and classification of the cardiomyopathies
- Leslie T Cooper, Jr, MD
Leslie T Cooper, Jr, MD
- Professor of Medicine
- Mayo Clinic College of Medicine
Cardiomyopathies are diseases of heart muscle . Cardiomyopathies include a variety of myocardial disorders that manifest with various structural and functional phenotypes and are frequently genetic. Although some have defined cardiomyopathy to include myocardial disease caused by known cardiovascular causes (such as hypertension, ischemic heart disease, or valvular disease), current major society definitions of cardiomyopathy exclude heart disease secondary to such cardiovascular disorders.
Definitions and classification systems for cardiomyopathies are described here. The individual disorders and the evaluation of the patient with heart failure or cardiomyopathy are discussed separately. (See "Determining the etiology and severity of heart failure or cardiomyopathy" and "Causes of dilated cardiomyopathy" and "Overview of hypertrophic cardiomyopathy management including treatment of special problems" and "Idiopathic restrictive cardiomyopathy" and "Clinical manifestations and diagnosis of arrhythmogenic right ventricular cardiomyopathy".)
MAJOR SOCIETY CRITERIA
In 1980, the World Health Organization (WHO) defined cardiomyopathies as "heart muscle diseases of unknown cause" to distinguish cardiomyopathy from cardiac dysfunction due to known cardiovascular entities such as hypertension, ischemic heart disease, or valvular disease . In clinical practice, however, the term "cardiomyopathy" has also been applied to diseases of known cardiovascular cause (eg, “ischemic cardiomyopathy” and “hypertensive cardiomyopathy”).
As a result, the 1995 WHO/International Society and Federation of Cardiology (ISFC) Task Force on the Definition and Classification of the Cardiomyopathies expanded the classification to include all diseases affecting heart muscle and to take into consideration etiology as well as the dominant pathophysiology . In this 1995 classification, the cardiomyopathies were defined as "diseases of the myocardium associated with cardiac dysfunction." They were classified according to anatomy and physiology into the following types, each of which has multiple different causes:
●Dilated cardiomyopathy (DCM)
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- MAJOR SOCIETY CRITERIA
- ECHOCARDIOGRAPHIC EVALUATION
- Systolic dysfunction
- Diastolic dysfunction
- ANATOMIC AND PHYSIOLOGIC CLASSIFICATION
- Dilated cardiomyopathy
- Hypertrophic cardiomyopathy
- - Athlete's heart
- - Other causes of hypertrophy
- - Not HCM
- Restrictive cardiomyopathy
- - Endocardial fibroelastosis
- Arrhythmogenic right ventricular cardiomyopathy
- Unclassified cardiomyopathies
- - Left ventricular noncompaction
- - Stress-induced cardiomyopathy
- - Cirrhotic cardiomyopathy
- SUMMARY AND RECOMMENDATIONS