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Risk stratification of sudden cardiac death in hypertrophic cardiomyopathy

Nature Clinical Practice Cardiovascular Medicine volume 4, pages 667–676 (2007)Cite this article

Abstract

Hypertrophic cardiomyopathy (HCM) is the most common hereditary cardiac condition and the leading cause of sudden cardiac death (SCD) in young adults. Given that SCD can be the first and most devastating clinical expression of HCM, identifying individuals at high risk is paramount. Determining an individual's risk for HCM-related SCD requires a thorough understanding of the recognized risk factors, of which there are seven established or 'major' and five 'possible'. Major risk factors can be identified by thoroughly reviewing a patient's personal medical history and noninvasive cardiovascular testing. The presence of major risk factors identify patients who are at high enough risk of SCD to warrant consideration of an implantable cardioverter-defibrillator; whereas the absence of any major risk factors provides considerable reassurance to both patient and physician. The risk of HCM-related SCD in patients with no major risk factors is, however, not zero. Possible risk factors gain importance in the presence of an isolated major risk factor. Here, we provide a contemporary review of established and possible risk factors for HCM-related SCD. We also examine microvolt T-wave alternans and cardiovascular MRI as emerging risk stratification tools that could further hone our ability to accurately identify the high-risk patient.

Key Points

  • Prior ventricular fibrillation or sustained ventricular tachycardia related to hypertrophic cardiomyopathy is associated with a high recurrence rate and patients with these risk factors should receive an implantable cardioverter-defibrillator (ICD) as secondary prevention against sudden cardiac death

  • An individual with two or more of the remaining established risk factors is at high risk of sudden death and should be offered an ICD as primary prevention; routine ICD placement is not warranted in patients without any recognized risk factors

  • Though it is reasonable to consider a primary-prevention ICD in patients with only one established risk factor, the decision has to be individualized for each patient on the basis of variables such as age, NYHA functional class, recurrence of events and presence of possible risk factors

  • Possible risk factors, though associated with an increased risk of sudden death, are less predictive than major risk factors, and the evidence does not support invasive primary-prevention measures

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Figure 1: Risk stratification for sudden cardiac death in patients with hypertrophic cardiomyopathy.

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Authors and Affiliations

  1. MA Miller is a Fellow in Cardiovascular Diseases at Mount Sinai School of Medicine, JA Gomes is Professor of Medicine and Director of the Consultative Services and Senior Consultant in Clinical Cardiac Electrophysiology at the Mount Sinai Medical Center, and V Fuster is Director of Mount Sinai Heart at the Mount Sinai School of Medicine, New York, NY, USA.,

    Marc A Miller, J Anthony Gomes & Valentin Fuster

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Correspondence to Marc A Miller.

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JA Gomes is a stockholder/Director of Medtronic.

The other authors declared no competing interests.

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Miller, M., Anthony Gomes, J. & Fuster, V. Risk stratification of sudden cardiac death in hypertrophic cardiomyopathy. Nat Rev Cardiol 4, 667–676 (2007). https://doi.org/10.1038/ncpcardio1057

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