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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Hypertrophic cardiomyopathy in the elderly
Toru Kubo1, Hiroaki Kitaoka, Makoto Okawa
1Department of Medicine and Geriatrics, Kochi Medical School, Kochi, Japan.
Insights
Hypertrophic cardiomyopathy (HCM) often affects elderly patients, not just the young. This study details HCM
Area of Science:
- Cardiology
- Genetics
- Geriatrics
Background:
- Hypertrophic cardiomyopathy (HCM) is a common genetic heart disorder with varied presentations.
- While often associated with young individuals, HCM increasingly affects elderly populations.
- Limited research exists on the specific clinical features of HCM in older adults.
Purpose of the Study:
- To describe the clinical characteristics of hypertrophic cardiomyopathy in a community-based elderly cohort.
- To investigate the prevalence and features of HCM in an aged population in Japan.
Main Methods:
- Established a cardiomyopathy registration study in Kochi Prefecture, a region with a high elderly population.
- Analyzed clinical, morphological, and functional data from a community-based HCM cohort.
- Focused on patients aged 60 years and above, who comprised 70% of the cohort.
Main Results:
- The majority of the registered HCM patients were elderly (70% aged >=60).
- Late-onset HCM is often linked to genetic mutations, particularly in the cardiac myosin-binding protein C gene.
- Elderly HCM patients experienced significant heart failure and embolic events, strongly associated with atrial fibrillation.
Conclusions:
- Hypertrophic cardiomyopathy is prevalent in the elderly, often with genetic underpinnings.
- Heart failure and embolic events are critical complications in middle-aged and elderly HCM patients.
- Longitudinal management is crucial for preventing adverse clinical outcomes in HCM.
Abstract:
Hypertrophic cardiomyopathy (HCM) is a relatively common genetic cardiac disorder with heterogeneous morphological, functional and clinical features. Although the risk of sudden death and incapacitating symptoms in young patients has been focused upon, the disease has been found with increasing frequency in elderly patients. However, there have been few studies on clinical features of HCM in the elderly. We established a cardiomyopathy registration study in Kochi Prefecture, which is one of the most aged communities in Japan, to provide detailed descriptions of the clinical features of HCM in a community-based patient cohort. The unselected regional HCM population consisted largely of elderly patients (70% of the study cohort being >or=60 years of age at registration), although HCM has been regarded largely as a disease of the young. Cardiac hypertrophy that becomes clinically apparent late in life can be a genetic disorder, and mutations in the cardiac myosin-binding protein C gene are the most common cause of late-onset or elderly HCM. In the morphological features, sarcomere gene defects seem to have a predilection for a crescent-shaped left ventricular cavity with reversed septal curvature even in elderly patients, although an ovoid left ventricular shape was frequently seen in elderly patients in previous clinical studies on morphological characteristics of HCM. In middle-aged or elderly patients with HCM, heart failure and embolic events, which were strongly associated with atrial fibrillation, were very important. It is important to manage HCM patients from the standpoint of longitudinal evolution in order to prevent those clinical complications.
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