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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
Medical management of hypertrophic cardiomyopathy
Jesse J Naghi1, Robert J Siegel
1Division of Cardiology and the Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Insights
Hypertrophic cardiomyopathy (HCM) is a genetic heart condition causing left ventricular hypertrophy. Medical treatments aim to manage symptoms and reduce the risk of sudden cardiac death (SCD) in HCM patients.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is the most prevalent inherited cardiomyopathy.
- It stems from mutations in genes encoding sarcomeric proteins, leading to left ventricular hypertrophy.
- HCM presents with diverse symptoms, from mild discomfort to life-threatening events like sudden cardiac death (SCD).
Purpose of the Study:
- To review the current medical treatment landscape for hypertrophic cardiomyopathy.
- To highlight therapeutic strategies for symptom management and risk reduction in HCM.
- To provide an overview of medications used in HCM management.
Main Methods:
- Literature review of medical treatments for HCM.
- Analysis of therapeutic approaches for symptom control and SCD risk mitigation.
- Compilation of commonly prescribed medications including beta-blockers, calcium channel blockers, amiodarone, disopyramide, and angiotensin receptor blockers.
Main Results:
- Medical therapy is the primary approach for the majority of HCM patients.
- Various medications are employed to alleviate symptoms such as dyspnea and chest pain.
- Treatment strategies focus on both symptomatic relief and preventing sudden cardiac death.
Conclusions:
- Current medical treatments for HCM focus on symptom management and reducing SCD risk.
- A range of pharmacological agents are utilized to control HCM symptoms.
- Ongoing research and clinical practice continue to refine HCM treatment protocols.
Abstract:
Hypertrophic cardiomyopathy (HCM) is the most common genetically transmitted cardiomyopathy. The underlying cause of HCM has been attributed to a number of mutations within genes encoding primarily for sarcomeric proteins, which lead to a heterogeneous phenotype of left ventricular hypertrophy in the absence of other causes (eg, hypertension, aortic stenosis, or a discrete membranous subaortic stenosis). Symptoms may range from mild to severely limiting and consist of dyspnea and chest pain with exertion or at rest, syncope, or even sudden cardiac death (SCD). The majority of patients with HCM are treated medically. The primary aim of therapy is to reduce symptoms, but it should also address the risk of SCD. Throughout the years, numerous medical treatments have been used to achieve symptom control in these patients, and include medications such as β-blockers, calcium channel blockers, amiodarone, disopyramide, and angiotensin receptor blockers. This review provides an overview of the current medical treatment of HCM.
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