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Published on: June 14, 2016
Hypertrophic Cardiomyopathy: New Concepts and Therapies
Barry J Maron1, Ethan J Rowin1, Martin S Maron1
1Division of Cardiology, Hypertrophic Cardiomyopathy Institute, Tufts Medical Center, Boston, Massachusetts 02111;
Insights
Hypertrophic cardiomyopathy (HCM) is now a treatable condition with effective management options. Advanced strategies significantly reduce HCM-related morbidity and mortality, improving patient outcomes globally.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a common, inherited heart muscle disorder with global distribution.
- Historically, HCM management offered limited options for altering its natural progression.
Purpose of the Study:
- To review the current landscape of effective management strategies for hypertrophic cardiomyopathy.
- To highlight advancements in treating HCM across all age groups.
Main Methods:
- Review of recent clinical advancements and treatment guidelines for HCM.
- Analysis of risk stratification algorithms and therapeutic interventions.
Main Results:
- Effective management options now exist, transforming HCM into a treatable condition.
- Available strategies include risk stratification for defibrillators, surgical myectomy, anticoagulation, and heart transplantation.
- These interventions have led to reduced HCM-related morbidity and mortality.
Conclusions:
- Modern therapeutic strategies have significantly improved the prognosis for patients with hypertrophic cardiomyopathy.
- HCM is now a manageable condition with reduced annual mortality rates approaching 0.5%.
Abstract:
Hypertrophic cardiomyopathy (HCM), a relatively common, globally distributed, and often inherited myocardial disorder, transformed over the last several years into a treatable condition with the emergence of effective management options that alter natural history at all ages. Now available are a matured risk stratification algorithm selecting patients for prophylactic implantable defibrillators that prevent arrhythmic sudden death; low-risk, high-benefit surgical myectomy to reverse progressive heart failure symptoms due to left ventricular outflow obstruction; anticoagulation prophylaxis to prevent atrial fibrillation-mediated embolic stroke; and heart transplant for refractory end-stage disease in the absence of obstruction. Those strategies have resulted in reduction of HCM-related morbidity and reduction of mortality to 0.5% per year.
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