Article Text

Download PDFPDF
Original research
Epidemiology of cardiomyopathies and incident heart failure in a population-based cohort study
  1. Jack RW Brownrigg1,
  2. Vincenzo Leo1,
  3. Joel Rose2,
  4. Eric Low3,
  5. Sarah Richards3,
  6. Gerry Carr-White4,
  7. Perry M Elliott5
  1. 1 Rare Diseases, Pfizer Ltd, Tadworth, UK
  2. 2 Cardiomyopathy UK, Chesham, UK
  3. 3 Amyloidosis Research Consortium, Edinburgh, UK
  4. 4 Department of Cardiology, Guy's and St. Thomas' Foundation Trust, London, UK
  5. 5 Institute of Cardiovascular Science, University College London, London, UK
  1. Correspondence to Dr Perry M Elliott, Institute of Cardiovascular Science, University College London, London, UK; perry.elliott{at}


Aims The population prevalence of cardiomyopathies and the natural history of symptomatic heart failure (HF) and arrhythmia across cardiomyopathy phenotypes is poorly understood. Study aims were to estimate the population-diagnosed prevalence of cardiomyopathies and describe the temporal relationship between a diagnosis of cardiomyopathy with HF and arrhythmia.

Methods People with cardiomyopathy (n=4116) were identified from linked electronic health records (~9 million individuals; 2000–2018) and categorised into hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy (RCM) and cardiac amyloidosis (CA). Cardiomyopathy point prevalence, rates of symptomatic HF and arrhythmia and timing relative to a diagnosis of cardiomyopathy were determined.

Results In 2018, DCM was the most common cardiomyopathy. DCM and HCM were twice as common among men, with the reverse trend for ARVC. Between 2010 and 2018, prevalence increased for ARVC by 180% and HCM by 9%. At diagnosis, more patients with CA (66%), DCM (56%) and RCM (62%) had pre-existing HF compared with ARVC (29%) and HCM (27%). Among those free of HF at diagnosis of cardiomyopathy, annualised HF incidence was greatest in CA and DCM. Diagnoses of all cardiomyopathies clustered around the time of HF onset.

Conclusions The recorded prevalence of all cardiomyopathies increased over the past decade. Recognition of CA is generally preceded by HF, whereas individuals with ARVC or HCM more often developed HF after their cardiomyopathy diagnosis suggesting a more indolent course or better asymptomatic recognition. The clustering of HF and cardiomyopathy diagnoses suggests opportunities for presymptomatic or earlier diagnosis.

  • cardiomyopathies
  • heart failure
  • epidemiology

Data availability statement

No data are available.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Data availability statement

No data are available.

View Full Text


  • Contributors JRWB is the guarantor of the study, had full access to the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Concept and design: all authors. Acquisition, analysis or interpretation of data: all authors. Drafting of the manuscript: JRWB, PME. Critical revision of manuscript for important intellectual content: all authors. Statistical analysis: JRWB. Administrative, technical or material support: JRWB. Supervision: PME.

  • Funding This study was sponsored by Pfizer. Analytical and editorial support were provided by Michael Hurst and Thomas Mason of Health Economics & Outcomes Research Ltd and was funded by Pfizer.

  • Competing interests JRWB, SR, EL, GC-W and PME received compensation from Pfizer for their services as a member of the study Steering Committee. PME and GC-W have also received educational and/or research grants from Pfizer. PME has consultancy agreements from Alnylam, Sanofi, Genzyme and MyoKardia. JRWB at the time of this analysis was a full-time employee of Pfizer and holds stock and/or stock options. VL was a paid contractor to Pfizer in connection with the development of this manuscript.

  • Patient and public involvement statement Representatives from two patient organisations (Cardiomyopathy UK, Amyloidosis Research Consortium) were members of the study steering committee and participated in developing the design of the study, interpretation of the results and drafting the manuscript.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.