Do patients with hypertrophic cardiomyopathy have excess mortality compared with the general population?
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.
Mortality rates in hypertrophic cardiomyopathy remain higher than those of the general population but have improved in contemporary cohorts.
It is unclear whether hypertrophic cardiomyopathy (HCM) conveys excess mortality when compared with the general population.
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.
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.
Lorenzini M, Anastasiou Z, O’Mahony C, et al. Mortality Among Referral Patients With Hypertrophic Cardiomyopathy vs the General European Population. JAMA Cardiol. Published online November 27, 2019. doi:https://doi.org/10.1001/jamacardio.2019.4534
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