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The Paradigm of Sudden Death Prevention in Hypertrophic Cardiomyopathy
Martin S Maron1, Ethan J Rowin1, Barry J Maron1
1Lahey Hospital and Medical Center, HCM Center, Burlington, Massachusetts.
Insights
Hypertrophic cardiomyopathy (HCM) is now treatable, with implantable cardioverter-defibrillators significantly reducing sudden cardiac death (SCD) risk. Risk stratification aids in recommending prophylactic defibrillator implants for eligible patients.
Area of Science:
- Cardiology
- Genetics
- Preventive Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a common inherited cardiac disease.
- Historically, HCM had a poor prognosis and was a leading cause of sudden cardiac death (SCD) in young individuals.
- Recent advancements have transformed HCM into a treatable condition with improved management strategies.
Purpose of the Study:
- To highlight the shift in understanding HCM from an untreatable to a treatable disease.
- To emphasize the impact of implantable cardioverter-defibrillators (ICDs) on managing SCD risk in HCM.
- To discuss current risk stratification methods for prophylactic ICD implantation.
Main Methods:
- Review of clinical practice and therapeutic advances in HCM management.
- Analysis of the role of implantable cardioverter-defibrillators in preventing SCD.
- Evaluation of guideline-based risk stratification algorithms, including those incorporating cardiac magnetic resonance imaging (CMR).
- Comparison of traditional risk marker strategies with newer mathematical risk scores for ICD decision-making.
Main Results:
- ICDs have enabled primary prevention of SCD in HCM by terminating ventricular tachyarrhythmias, reducing annual mortality by over 10-fold to 0.5%.
- Guideline-based risk stratification, incorporating clinical factors and CMR findings, aids in recommending prophylactic ICDs.
- The ACC/AHA risk marker strategy shows 95% sensitivity for identifying patients needing appropriate ICD therapy, though often years after implantation.
- The ESC mathematical SCD risk score has lower sensitivity (33%) but may reduce device overtreatment.
Conclusions:
- HCM is now a treatable condition with significantly improved outcomes due to advances like ICDs.
- Effective risk stratification is crucial for guiding prophylactic ICD implantation in HCM patients at high risk for SCD.
- Shared decision-making, incorporating physician judgment and patient factors, is essential for optimal management of HCM and SCD prevention.
Abstract:
Hypertrophic cardiomyopathy (HCM) is a relatively common and, often, inherited cardiac disease, once regarded as largely untreatable with ominous prognosis and, perhaps, most visibly as a common cause of sudden cardiac death (SCD) in the young. However, HCM is now more accurately considered a treatable disease with management options that significantly alter its clinical course. This is particularly true for SCD because the penetration of implantable cardioverter-defibrillators into HCM practice enables primary prevention device therapy that reliably terminates potentially lethal ventricular tachyarrhythmias (3% to 4%/year). This therapeutic advance is largely responsible for >10-fold decrease in the overall disease-related mortality to 0.5%/year, independent of patient age. A guideline-based clinical risk stratification algorithm has evolved, which included variables identifiable with cardiac magnetic resonance: ≥1 risk markers judged major within the clinical profile of an individual patient, associated with a measure of physician judgment and shared decision-making, can be sufficient to consider the recommendation of a prophylactic defibrillator implant. Implantable cardioverter-defibrillator decisions using the American College of Cardiology and the American Heart Association traditional major risk marker strategy are associated with a 95% sensitivity for identifying those patients who subsequently experience appropriate therapy, albeit often 5 to 10+ years after implant but without heart failure deterioration or death after a device intervention. A mathematical SCD risk score proposed by European Society of Cardiology is associated with a relatively low sensitivity (33%) for predicting and preventing SCD events but with potential for less device overtreatment.
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