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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 daily practice: an introduction on diagnosis, prognosis and treatment
Insights
Hypertrophic cardiomyopathy (HCM) is an inherited heart condition. This review details its diagnosis, prognosis, and diverse treatment strategies, including invasive options for severe cases.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a complex inherited cardiac disease.
- Understanding of HCM diagnosis, prognosis, and treatment has evolved significantly since its initial description in 1957.
- Recent analyses have refined insights into HCM's natural history and sudden cardiac death risk.
Approach:
- This report summarizes current diagnostic criteria for HCM.
- It reviews the clinical course and therapeutic management of HCM.
- Special attention is given to specific issues relevant to HCM.
Key Points:
- Optimal HCM therapy is individualized based on symptoms, hemodynamics, and sudden cardiac death risk factors.
- Sudden cardiac death is the most devastating aspect of HCM's natural history.
- Invasive therapies like surgery or septal ablation are options for obstructive, drug-refractory HCM.
Conclusions:
- HCM management requires a comprehensive approach considering disease progression and patient-specific factors.
- Continued research is crucial for improving outcomes in patients with hypertrophic cardiomyopathy.
- This review provides a consolidated overview for clinicians managing HCM.
Abstract:
Hypertrophic cardiomyopathy (HCM) is a complex, inherited cardiac disease that has been subject to intense investigation since it was first described in 1957. Over the past 40 years, understanding has evolved regarding the diagnosis, prognosis and treatment of HCM. Analyses of HCM populations from nonreferral centres have refined the insights into the natural history and the occurrence of sudden cardiac death, which is the most devastating component of its natural history. Therapeutic strategies are diverse and may vary during the course of the disease. Optimal therapy depends on symptoms, haemodynamic findings and the presence of risk factors for sudden cardiac death. At present, invasive therapy for patients with obstructive HCM and drug-refractory symptoms includes surgery or percutaneous transluminal septal myocardial ablation. This report summarises the diagnostic criteria, clinical course and therapeutic management of HCM. Attention is also paid to certain issues of special interest in this disease.
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