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Cardiomyopathies and specific heart muscle diseases. Definitions, terminology, classifications and new and old
1Royal Postgraduate Medical School, Hammersmith, London, UK.
Insights
Cardiomyopathies are heart muscle diseases. This review covers hypertrophic, dilated, and restrictive types, noting causes, features, and recent genetic and viral research findings for hypertrophic and dilated cardiomyopathies.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Cardiomyopathies are heart muscle diseases of unknown cause, classified into hypertrophic, dilated, and restrictive types.
- Hypertrophic cardiomyopathy presents with ventricular hypertrophy and risk of sudden death, often familial.
- Dilated cardiomyopathy involves systolic failure and congestive heart failure, typically without familial links.
Purpose of the Study:
- To describe the major features of different cardiomyopathies.
- To highlight current and future advances in understanding these heart muscle diseases.
- To explore potential causes and genetic links in specific cardiomyopathy types.
Main Methods:
- Review of existing literature on cardiomyopathy classification and features.
- Discussion of genetic research, including gene identification for hypertrophic cardiomyopathy.
- Examination of etiological factors, such as viral RNA in dilated cardiomyopathy.
Main Results:
- Identification of a gene on chromosome 14 linked to hypertrophic cardiomyopathy.
- Detection of virus RNA particles in myocarditis and dilated cardiomyopathy cases.
- Evidence suggests some dilated cardiomyopathy cases may result from prior myocarditis.
Conclusions:
- Cardiomyopathies encompass diverse heart muscle diseases with distinct clinical and etiological profiles.
- Genetic and infectious factors are increasingly implicated in the pathogenesis of cardiomyopathies.
- Ongoing research promises further insights into diagnosis and treatment of these conditions.
Abstract:
Cardiomyopathies are defined as 'heart muscle diseases of unknown cause' and classified into hypertrophic, dilated and restrictive types, respectively. Hypertrophic cardiomyopathy is notable for massive ventricular hypertrophy without obvious cause, impaired diastolic and systolic function, a tendency for sudden death and a familial propensity. Dilated cardiomyopathy by contrast, demonstrates severe systolic failure progressing to congestive heart failure, with usually no familial tendency. Restrictive cardiomyopathy and diastolic heart disease represent syndromes with restriction to ventricular filling due to restrictive forces in the endomyocardium (and in constrictive pericarditis in the pericardium). The commonest cause of restrictive cardiomyopathy is endomyocardial fibrosis now usually known as hypereosinophilic endomyocardial disease. Specific heart muscle diseases are those conditions in which myocardial disease is due to a known cause: they usually produce systolic failure though occasionally a restrictive syndrome is evident. Amyloid heart disease occupies a place intermediate between cardiomyopathies and specific heart muscle diseases. The major features of the above conditions are described and current and future advances noted. Examples are the identification of the gene probably responsible for hypertrophic cardiomyopathy located on chromosome 14, and the identification of virus RNA particles in the myocardium in both myocarditis and in dilated cardiomyopathy, which strengthens the growing evidence suggesting that some cases of dilated cardiomyopathy may be due to previous myocarditis.