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Original Investigation
November 27, 2019

Mortality Among Referral Patients With Hypertrophic Cardiomyopathy vs the General European Population

Author Affiliations
  • 1Barts Heart Centre, Institute for Cardiovascular Science, St Bartholomew’s Hospital, University College London, London, United Kingdom
  • 2Cardiology, Department of Experimental, Diagnostic, and Specialty Medicine, Alma Mater Studiorum University of Bologna, Bologna, Italy
  • 3Department of Statistical Science, University College London, London, United Kingdom
  • 4Cardiac Department, University Hospital Virgen Arrixaca, Murcia, Spain
  • 5Cardiology Department and Research Unit, A Coruña University Hospital, Galician Health Service, A Coruña, Spain
  • 6Unit of Inherited Cardiovascular Diseases, First Department of Cardiology, University of Athens, Athens, Greece
  • 7Heart Failure and Inherited Cardiac Diseases Unit, Hospital Universitario Puerta del Hierro-Majadahonda, Madrid, Spain
  • 8Centro de Investigacion Biomedica en Red en Enfermedades Cardiovasculares, Madrid, Spain
  • 9Facultad de Ciencias de la Salud, University Francisco de Vitoria, Pozuelo de Alarcón, Madrid, Spain
  • 10Department of Cardiothoracic Sciences, Monaldi Hospital, AORN Colli, Università della Campania “Luigi Vanvitelli,” Naples, Italy
JAMA Cardiol. Published online November 27, 2019. doi:https://doi.org/10.1001/jamacardio.2019.4534
Key Points

Question  Do patients with hypertrophic cardiomyopathy have excess mortality compared with the general population?

Findings  This cohort study of 4893 patients with hypertrophic cardiomyopathy recruited between 1980 and 2013 at 7 European referral centers comparing their survival (a composite end point of all-cause mortality, heart transplant, and aborted sudden cardiac death) with that of the general population found that patients with hypertrophic cardiomyopathy had excess mortality throughout life that improved with time. Female patients had higher excess mortality than male patients throughout the age spectrum, whereas mortality among male patients older than 60 years was similar to that of the general population.

Meaning  Mortality rates in hypertrophic cardiomyopathy remain higher than those of the general population but have improved in contemporary cohorts.

Abstract

Importance  It is unclear whether hypertrophic cardiomyopathy (HCM) conveys excess mortality when compared with the general population.

Objective  To compare the survival of patients with HCM with that of the general European population.

Design, Setting, and Participants  Retrospective cohort study of 4893 consecutive adult patients with HCM presenting at 7 European referral centers between 1980 and 2013. The data were analyzed between April 2018 and August 2019.

Main Outcomes and Measures  Survival was compared using standardized mortality ratios (SMRs) calculated with data from Eurostat, stratified by study period, country, sex, and age, and using a composite end point in the HCM cohort of all-cause mortality, aborted sudden cardiac death, and heart transplant.

Results  Of 4893 patients with HCM, 3126 (63.9%) were male, and the mean (SD) age at presentation was 49.2 (16.4) years. During a median follow-up of 6.2 years (interquartile range, 3.1-9.8 years), 721 patients (14.7%) reached the composite end point. Compared with the general population, patients with HCM had excess mortality throughout the age spectrum (SMR, 2.0, 95% CI, 1.48-2.63). Excess mortality was highest among patients presenting prior to the year 2000 but persisted in the cohort presenting between 2006 and 2013 (SMR, 1.84; 95% CI, 1.55-2.18). Women had higher excess mortality than men (SMR, 2.66; 95% CI, 2.38-2.97; vs SMR, 1.68; 95% CI, 1.52-1.85; P < .001).

Conclusions and Relevance  Among patients referred to European specialty centers, HCM was associated with significant excess mortality through the life course. Although there have been improvements in survival with time, potentially reflecting improved treatments for HCM, these findings highlight the need for more research into the causes of excess mortality among patients with HCM and for better risk stratification.

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