dilated cardiomyopathy (DCM)

Last edited 11/2022 and last reviewed 11/2022

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 - genes most commonly known to cause DCM, include LMNA, MYH7, TNNT2, TTN, RBM20, BAG3 (3)
• 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.
• McDonagh TA et al., ESC Scientific Document Group, 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: Developed by the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) With the special contribution of the Heart Failure Association (HFA) of the ESC, European Heart Journal, 2021;, ehab368, https://doi.org/10.1093/eurheartj/ehab368