Towards a New Classification of Cardiomyopathies

Figures

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  Fig. 1. The MOGES nosology system for classifying cardiomyopathies. (M) Morphofunctional phenotype. This description is designed to contain additional information. These red flags are placed in parentheses after the notation of morphofunctional phenotype. (O) Organ/system involvement. This described involvement of other organs. (E) Aetiologic annotation provides information on specific disease genes and mutation, as well as a description of nongenetic causes of disease. (G) May denote a genetic disease, supporting family monitoring strategies. (S) Stage. This provides a simple description of functional limitation using existing scores such as the New York Heart Association class. Key: ACC, American College of Cardiology; AHA, American Heart Association; ARVC/D, arrhythmogenic right ventricular cardiomyopathy/dysplasia; DCM, dilated cardiomyopathy; ECG, electrocardiogram; ECHO, echocardiogram; HCM, hypertrophic cardiomyopathy; LVNC, left ventricular noncompaction; NYHA, New York Heart Association; RCM, restrictive cardiomyopathy. (Used with permission of Elsevier from: J Am Coll Cardiol. 2014;64:304–318. https://doi.org/10.1016/j.jacc.2014.05.027, permission conveyed through Copyright…

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  Fig. 2. Multiparametric approach to clinical notation. This figure shows how a systematic multiparametric approach to clinical phenotyping linked with targeted diagnostics including genetic testing can be used to create highly specific phenotypes that facilitate personalised treatment plans. In this worked example, the diagnosis transforms from a simplistic categorisation to a complex genetic disorder characterised by myocardial scar and a propensity to ventricular arrhythmia. The current approach to disease nomenclature based on a single descriptor (in this case arrhythmogenic LV cardiomyopathy or even more simply arrhythmogenic cardiomyopathy) is subjective and fails to convey anything about aetiology or treatment. Key: h=hour, VE=ventricular ectopics, DSP=desmoplakin