Sudden death related cardiomyopathies - Hypertrophic cardiomyopathy

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Abstract

Hypertrophic cardiomyopathy (HCM) is a form of inherited cardiomyopathy. Most individuals with HCM experience minimal symptoms throughout their lifetime. However, those with HCM are at risk of ventricular arrhythmias and sudden cardiac death (SCD), the most feared complication of HCM. Implantable cardioverter defibrillator (ICD) implantation has played a large role in transforming this disease from one with an ominous prognosis to one with mortality rates that are on par with the general public. Since the early 2000s, balance between SCD prevention and unnecessary ICD placement has been sought, this is reflected in the evolution of SCD risk stratification models for patients with HCM. This review discusses key concepts pertaining to HCM, with emphasis on prevention of SCD, and summarizes and compares the recommendations for ICD implantation in current guidelines.

Section snippets

Natural history and risk of sudden death

The natural history and clinical manifestation of HCM can be highly variable, even within members of the same family, carrying the same genetic mutation – ranging from completely asymptomatic to heart failure and/or sudden death.16 Most patients remain asymptomatic or minimally symptomatic throughout their lives. The most common complications of HCM leading to morbidity and mortality are AF (20–25%), heart failure (HF; 22%), end-stage HF (3%), and SCD (1%).12,17 Symptoms of HCM include dyspnea,

Prevention of SCD and other management considerations

SCD is the most devastating complication of HCM and can be the initial presentation of HCM. Indications for ICD implantation, to prevent SCD, have evolved over the last 2 decades.17,27., 28., 29.

Shown in Table 1 is a summary of current recommendations for ICD implantation from four current guidelines: 2017 AHA/ACC/HRS guidelines on ventricular arrhythmias (VA) and prevention of SCD, 2014 ESC HCM guidelines, 2011 ACCF/AHA HCM guidelines, and 2008 ACCF/AHA/HRS cardiac device guidelines. New

Conclusions and future perspective

HCM is the most common and well-studied inherited cardiomyopathy. In the last two decades, remarkable progress has been made in disease management and prevention of SCD. The role of medical management, namely Mavacamten, is being studied in current trials. Risk stratification for SCD continues to evolve, current data suggest that CMR may have value, but its definitive role in risk stratification for SCD is yet to be determined. Recent data have shown that patients diagnosed before the age of

Conflict of interest statement

None of the authors have any conflicts of interests with regard to this publication.

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      It is the most common genetic disorder affecting the heart and accounts for a sizable fraction of morbidity and mortality in young people, particularly athletes.20 The only widely accepted therapeutic options for HCM involve localized destruction of heart muscle (by alcohol ablation or surgical myectomy) to relieve outflow obstruction,21 and/or the use of implantable cardioverter-defibrillators to prevent arrhythmic death.22 Both modalities are invasive, associated with considerable risks and side effects, and variably effective.

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