Related Experiment Video
Updated: Jun 30, 2025
Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Long term mortality in patients with hypertrophic cardiomyopathy - A Danish nationwide study
Mads-Holger Bang Jacobsen1, Jeppe Kofoed Petersen1, Daniel Modin2
1Dep. of Cardiology, Copenhagen University Hospital, Rigshospitalet, Blegdamsvej 9, 2100 Copenhagen, Denmark.
Insights
Hypertrophic cardiomyopathy (HCM) significantly increases long-term mortality risk. This nationwide study found HCM patients have a 10-year mortality probability of 36.4%, compared to 19.4% in the general population.
Area of Science:
- Cardiology
- Public Health
- Epidemiology
Background:
- Hypertrophic cardiomyopathy (HCM) is linked to increased risks of arrhythmia, stroke, heart failure, and sudden cardiac death.
- Existing mortality data for HCM often comes from selected patient groups, limiting generalizability.
Purpose of the Study:
- To investigate the long-term mortality rate in a nationwide cohort of patients diagnosed with hypertrophic cardiomyopathy (HCM).
- To compare the mortality rates of HCM patients with a matched cohort from the general Danish population.
Main Methods:
- A nationwide cohort of first-time HCM diagnoses in Denmark (2007-2018) was identified using registries.
- Patients were matched 1:3 to controls based on age, sex, comorbidities, and diagnosis date, with a subset requiring two outpatient visits for specificity.
- Mortality was analyzed using Kaplan-Meier estimators and multivariable Cox regression.
Main Results:
- 3126 patients with first-time HCM diagnoses were identified; 1197 met criteria for the main analysis.
- The 10-year all-cause mortality probability was 36.4% for HCM patients versus 19.4% for controls.
- HCM diagnosis was independently associated with a 48% increased mortality rate (HR 1.48, p=0.001) after adjusting for comorbidities and medications.
Conclusions:
- Hypertrophic cardiomyopathy (HCM) is associated with a significant increase in long-term mortality.
- This nationwide study confirms a higher mortality risk for HCM patients compared to the general population.
Background:
Patients with hypertrophic cardiomyopathy (HCM) are generally regarded as having increased risk of arrhythmia, stroke, heart failure, and sudden cardiac death, but reported mortality rates vary considerably and originate from selected populations.
Study Objective:
We aimed to investigate the long-term mortality rate in a nationwide cohort of patients with HCM compared to a matched cohort from the general Danish population.
Methods:
All patients with a first-time HCM diagnosis in Denmark between January 1, 2007 and December 31, 2018 were identified through nationwide registries. In the main analysis, two visits in an outpatient clinic were required in order to increase specificity. Patients were matched to controls from the background population in a 1:3 ratio based on age, sex, selected comorbidities and date of HCM. Mortalities were compared using Kaplan Meier estimator and multivariable Cox regression models.
Results:
We identified 3126 patients with a first-time diagnosis of HCM. 1197 patients had at least two visits in the outpatient clinic (43 % female, median age 63.1 [25th-75th percentile 52.1-72.1] years). All-cause mortality was significantly higher in HCM patients than in matched controls: 10-year probabilities of death were 36.4 % (95 % CI 30.2-43.5 %) for HCM patients and 19.4 % (95 % CI 16.8-22.5 %) for controls. After adjusting for additional comorbidities and medications, a diagnosis with HCM was associated with an increased mortality rate (HR 1.48 (95 % CI 1.18-1.84, p = 0.001)).
Conclusion:
Compared to matched controls from the background population, presence of HCM was associated with a significant increase in mortality rate.
