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Published on: August 8, 2022
Clinical course of hypertrophic cardiomyopathy with survival to advanced age
Barry J Maron1, Susan A Casey, Robert G Hauser
1Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, 920 E. 28th Street, Suite 60, Minneapolis, MN 55407, USA. hcm.maron@mhif.org
Insights
Hypertrophic cardiomyopathy (HCM) is often well-tolerated in older adults, allowing for normal life expectancy. Many patients experience mild symptoms, with diagnosis and symptom onset often delayed until later in life.
Area of Science:
- Cardiology
- Geriatrics
- Genetics
Background:
- Adverse outcomes of hypertrophic cardiomyopathy (HCM) are typically emphasized in younger and middle-aged individuals.
- The clinical course and long-term prognosis of HCM in elderly populations remain less understood.
Purpose of the Study:
- To elucidate the clinical profile and long-term outcomes of elderly patients diagnosed with hypertrophic cardiomyopathy (HCM).
Main Methods:
- A community-based cohort of 312 patients with hypertrophic cardiomyopathy (HCM) was assessed to avoid tertiary center referral bias.
- Long-term outcomes, including life expectancy, symptom severity, and survival probabilities, were analyzed.
Main Results:
- 23% of patients achieved a normal life expectancy (>=75 years), with 14% living to >=80 years.
- Most elderly HCM patients (64%) experienced mild symptoms and few clinical consequences, with diagnosis and symptom onset delayed to ages 74 and 70, respectively.
- Survival probabilities for patients >=50 years at diagnosis did not significantly differ from the general population; elderly women with HCM showed less wall thickness and more frequent basal outflow obstruction.
Conclusions:
- Hypertrophic cardiomyopathy (HCM) is frequently well-tolerated in older individuals, compatible with normal life expectancy.
- The disease can remain clinically dormant for extended periods, with diagnosis and symptoms often appearing late in life.
- These findings offer reassurance to patients, suggesting a potentially less unfavorable and unpredictable course for HCM than previously assumed.
Objectives:
This study was designed to clarify and resolve the clinical profile of older patients with hypertrophic cardiomyopathy (HCM).
Background:
Adverse consequences of HCM such as sudden death and incapacitating symptoms have been emphasized for the young and middle-aged.
Methods:
Long-term outcome of HCM was assessed in a community-based cohort not subject to tertiary center referral bias.
Results:
Of 312 patients, 73 (23%) achieved normal life expectancy (> or =75 years; range to 96); 44 (14%) were > or =80 years old. Most patients > or =75 years (47; 64%) experienced no or only mild limiting symptoms and lived virtually their entire lives with few HCM-related clinical consequences; 26 patients (36%) experienced severe progressive symptoms. In elderly patients with HCM, diagnosis and symptom onset were considerably delayed to 74 +/- 8 and 70 +/- 11 years, respectively. For patients > or =50 years at diagnosis, the probability of survival for 5, 10, and 15 years was 85 +/- 3%, 74 +/- 4%, and 57 +/- 6%, respectively, and did not significantly differ from a matched general population (p = 0.20). Patients > or =75 years were predominantly women, and had less marked wall thickness and more frequently showed basal outflow obstruction > or =30 mm Hg (compared with those <75 years; p < 0.01 and 0.001, respectively).
Conclusions:
Hypertrophic cardiomyopathy is frequently well tolerated and compatible with normal life expectancy, and may remain clinically dormant for long periods of time with symptoms and initial diagnosis deferred until late in life. These observations afford a measure of reassurance to many patients with HCM, a disease for which clinical course is often unfavorable and unpredictable.
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