Myocardial and Pericardial Disease

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Myocardial Disease

Overall, myocardial disease can be subdivided into two types: primary and secondary myocardial disease. Whereas the primary type most commonly has a genetic cause, secondary myocardial diseases are mostly acquired but may be precipitated by a genetic background.

Primary myocardial disease

Five different groups of primary myocardial disease exist; which are defined as ‘diseases of the myocardium with impaired cardiac function’, also referred to as cardiomyopathies.

• Hypertrophic cardiomyopathy (HCM)
• Dilated cardiomyopathy (DCM)
• Restrictive cardiomyopathy (RCM)
• Arrythmic cardiomyopathy (ACM)
• Unclassified cardiomyopathy (UCM)

Hypertrophic cardiomyopathy

The modern description of hypertrophic cardiomyopathy (HCM) dates from 1958. The essential characteristics of HCM are unexplained hypertrophy of the left ventricle in the absence of causative other cardiac or systemic disorders. Distinctive features further comprise myocyte disarray, familial occurrence, and an association with sudden cardiac death (SCD).

Epidemiology

The prevalence of the HCM phenotype was found to be approximately 0.2%, or 1 in 500, in several epidemiological studies. This frequency is notably higher than its occurrence in daily clinical practice. Hence, a large amount of patients remains undiagnosed, most probably without symptoms of inferences for their prognosis.