Definition and classification of the cardiomyopathies
- Leslie T Cooper, Jr, MD
Leslie T Cooper, Jr, MD
- Professor of Medicine
- Mayo Clinic College of Medicine and Science
Cardiomyopathies are diseases of heart muscle . A contemporary definition for cardiomyopathy is a myocardial disorder in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease sufficient to explain the observed myocardial abnormality. 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 "Idiopathic restrictive cardiomyopathy" and "Arrhythmogenic right ventricular cardiomyopathy: Anatomy, histology, and clinical manifestations" and "Hypertrophic cardiomyopathy: Clinical manifestations, diagnosis, and evaluation".)
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:
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- 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
- - Endomyocardial fibrosis
- Arrhythmogenic right ventricular cardiomyopathy
- Unclassified cardiomyopathies
- - Left ventricular noncompaction
- - Stress-induced cardiomyopathy
- - Cirrhotic cardiomyopathy
- - Endocardial fibroelastosis
- SUMMARY AND RECOMMENDATIONS