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Hypertrophic Cardiomyopathy: State of the Art
Steve R Ommen1, Rick A Nishimura1, Hartzell V Schaff2
1Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN.
Insights
Hypertrophic cardiomyopathy (HCM) management focuses on improving quality of life and longevity. Modern guidelines and new therapies, like cardiac myosin inhibitors, offer effective treatment options for patients with this genetic heart condition.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is the most prevalent genetic cardiomyopathy.
- While many patients achieve normal longevity and quality of life with guideline-directed care, some experience debilitating symptoms or life-threatening events.
- Understanding the pathophysiology is key to effective management.
Purpose of the Study:
- To outline current management strategies for hypertrophic cardiomyopathy.
- To highlight the role of comprehensive assessment in guiding therapy.
- To discuss available medical and invasive treatment options, including novel therapies.
Main Methods:
- Longitudinal assessment utilizing echocardiography, ambulatory electrocardiography, and stress testing.
- Cardiac magnetic resonance imaging for select cases.
- Review of therapeutic options including pharmacologic agents and invasive procedures.
Main Results:
- Guideline-based care can lead to normal longevity and good quality of life for many HCM patients.
- Advanced diagnostics aid in tailoring treatment plans.
- Cardiac myosin inhibitors represent a new therapeutic class targeting HCM's cellular mechanisms.
- Invasive options offer successful symptom relief for refractory cases when performed at expert centers.
Conclusions:
- Comprehensive and individualized management is crucial for patients with hypertrophic cardiomyopathy.
- New pharmacological and established invasive treatments can effectively manage symptoms and improve outcomes.
- The ultimate goal is to enable patients with HCM to lead active, healthy lifestyles.
Abstract:
Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy. Patients with HCM who are treated according to modern guidelines can reasonably be expected to have normal longevity and good quality of life. Nevertheless, effort intolerance, heart failure, arrhythmias, and sudden cardiac death can occur in some patients. Comprehensive and longitudinal assessment with echocardiography, ambulatory electrocardiography, stress testing, and in some cases cardiac magnetic resonance imaging helps guide therapy. Symptomatic patients can be treated with medications including a new class, cardiac myosin inhibitors, developed specifically to treat the cellular pathophysiologic mechanism of HCM. For patients whose symptoms do not respond to medical therapy or in whom medical therapy is not desired, invasive options to relieve symptoms, when performed at expert centers, have robust data for successful outcomes. The goal of management is to encourage and help patients with HCM lead an active, healthy lifestyle that includes regular exercise similar to activity recommendations for the general public.
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