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dilated cardiomyopathy (DCM)

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In dilated cardiomyopathy, weakening of heart muscle results in progressive dilatation of all heart chambers.

The term cardiomyopathy was originally used to describe heart muscle disease of unknown cause but in many cases it is now possible to identify a specific cause and, whenever possible, it is preferable to use specific terminology, such as "alcoholic heart disease" or "familial dilated cardiomyopathy".

Presentation may be with features of heart failure from impaired systolic function of the left and/or right ventricles.

Summary features (1,2):

  • prevalence 1:300 - 1:2750
  • inheritance - most AD; rarer X-linked or AR
  • genetics - large number of mutations and, currently relatively low yield of genetic testing
  • presentation - often asymptomatic.First presentation can be fatal ventricular arrhythmia or extra-cardiac associated phenotypes (e.g.muscular dystrophies)

Reference:

  • British Heart Foundation (1999). Factfile 1/99, Dilated Cardiomyopathy.
  • British Heart Foundation (2009).Factfile 5/09, Inherited cardiovascular disease.

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