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The term cardiomyopathy is used to describe a heterogeneous group of diseases that directly alter cardiac structure, impair myocardial function, or alter myocardial electrical properties. Discoveries in molecular genetics and the description of (ion) channelopathies as diseases have resulted in new definitions and classification of cardiomyopathies.1 Some primary cardiomyopathies are listed in Table 59-1. Secondary cardiomyopathies include heart muscle diseases associated with specific systemic disorders. They often present with hemodynamic findings similar to those of the idiopathic dilated or restrictive forms of cardiomyopathy. Some causes of secondary cardiomyopathies are listed in Table 59-2. As a group, the cardiomyopathies are the third most common form of cardiac disease encountered in the U.S., following coronary (ischemic) heart disease and hypertensive heart disease. Hypertrophic cardiomyopathy (HCM) is the second most common cause of sudden cardiac death in the adolescent population and the leading cause of sudden death in competitive athletes.2

Table 59-1 The Primary Cardiomyopathies
Table 59-2 Common Causes of Secondary Cardiomyopathies

An in-depth discussion of each of the primary and secondary cardiomyopathies is beyond the scope of this chapter, and one is unlikely to make a specific diagnosis in the ED. This chapter discusses selected cardiomyopathies (Table 59-3). The cardiomyopathies usually present with signs of systolic and diastolic ventricular dysfunction. The ED evaluation will generally guide the need for urgent treatment, admission, or referral. for further diagnostic evaluation, based on the severity of symptoms.

Table 59-3 Features of Selected Cardiomyopathies

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