Abstract

[first paragraph of article]Sudden cardiac death (SCD) is a devastating and often unpredictable complication of hypertrophic cardiomyopathy (HCM) that may occur as the initial disease presentation, frequently in asymptomatic or mildly symptomatic young people. Until 2000, only small series of patients examining predictors of SCD had been published, with a selection bias towards severe disease. Subsequently, larger series that are more representative of the HCM spectrum have shown that the annual SCD rate is less than 1%, and that there are subgroups of patients with a clearly higher risk.

Highlights

  • Sudden cardiac death (SCD) is a devastating and often unpredictable complication of hypertrophic cardiomyopathy (HCM) that may occur as the initial disease presentation, frequently in asymptomatic or mildly symptomatic young people

  • Implantable cardioverter defibrillators (ICD) implantation in secondary prevention is rarely a clinical challenge[10], but identifying patients within this heterogeneous disorder who are at high risk of SCD when prior to a first event is a challenge

  • Left atrial diameter has been associated with SCD in a single study with a Hazard ratio (HR) 1.03; 95% confidence interval (CI) [1.00–1.05], p = 0.0419

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Summary

INTRODUCTION

Sudden cardiac death (SCD) is a devastating and often unpredictable complication of hypertrophic cardiomyopathy (HCM) that may occur as the initial disease presentation, frequently in asymptomatic or mildly symptomatic young people. Larger series that are more representative of the HCM spectrum have shown that the annual SCD rate is less than 1%1,2, and that there are subgroups of patients with a clearly higher risk. Life-threatening ventricular tachyarrhythmias can be triggered by a number of environmental factors (e.g., intense physical exertion) or features intrinsic to the disease process, including left ventricular outflow obstruction, systemic arterial hypotension and supraventricular tachyarrhythmias. RISK STRATIFICATION AND PREVENTION OF SCD Implantable cardioverter defibrillators (ICD) are effective in terminating life-threatening ventricular arrhythmias in HCM, whereas pharmacologic therapy has not been demonstrated to provide protection from SCD. ICD implantation in secondary prevention is rarely a clinical challenge[10], but identifying patients within this heterogeneous disorder who are at high risk of SCD when prior to a first event is a challenge.

Female gender
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RISK FACTORS AND MODIFIERS
Findings
FINAL CONSIDERATIONS
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