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Published on: March 22, 2017
Towards a New Classification of Cardiomyopathies
1Institute of Cardiovascular Science, University College London, Rayne Institute, 5 University St., London, WC1E 6JF, UK. perry.elliott@ucl.ac.uk.
Insights
Current cardiomyopathy classification systems are limited. Future disease notation needs to incorporate new etiological knowledge and phenotypes for better clinical and scientific understanding of cardiomyopathies.
Area of Science:
- Cardiology
- Genetics
- Medical Informatics
Background:
- Cardiomyopathies represent a heterogeneous group of myocardial diseases.
- Current disease notation systems for cardiomyopathies face challenges in integrating evolving etiological insights.
Purpose of the Study:
- To review the clinical approach to cardiomyopathy disease notation.
- To identify limitations in current systems, particularly concerning etiological factors.
Main Methods:
- Review of existing cardiomyopathy classification systems.
- Case example: arrhythmogenic cardiomyopathy to illustrate limitations.
Main Results:
- Existing classification systems for cardiomyopathies are inadequate for diverse user needs (scientists, clinicians, patients).
- The concept of arrhythmogenic cardiomyopathy highlights the need for more comprehensive notation.
- Classical subtypes remain useful but require modification.
Conclusions:
- Current cardiomyopathy notation requires significant updates to reflect new knowledge on phenotypes and aetiologies.
- A unified classification system is needed to accommodate basic scientists, clinicians, and patients.
- Future notation must integrate genetic and etiological data for improved understanding and management.
Purpose Of Review:
The aim of this paper is to briefly summarise the clinical approach to disease notation for cardiomyopathies and to highlight its limitations with respect to the integration of new knowledge about aetiology.
Recent Findings:
The paper uses the recently advocated concept of arrhythmogenic cardiomyopathy as an example of the limitations of current classification systems. At present, there is no single classification system that meets the needs of all potential users, whether they are basic scientists, clinicians, patients or families. The classical cardiomyopathy subtypes still have utility, but future disease notation needs to be modified to take into account the new and more complete phenotypes and aetiologies.
Related Concept Videos
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Heart Failure IV: Classification and Diagnostic Evaluation
Cardiomyopathy IV: Restrictive Cardiomyopathy
Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy V: Interprofessional Care

