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Heart 89:977-978 doi:10.1136/heart.89.9.977
  • Editorial

Contemporary considerations for risk stratification, sudden death and prevention in hypertrophic cardiomyopathy

  1. B J Maron
  1. Correspondence to:
    Barry J Maron, MD, Minneapolis Heart Institute Foundation, 920 E 28th Street, Suite 60, Minneapolis, MN 55407, USA;
    hcm.maron{at}mhif.org

    For patients with hypertrophic cardiomyopathy, the likelihood of receiving primary prevention for sudden death with an ICD may depend in part on the country of residence

    Sudden death has been the most devastating, unpredictable and recognisable complication of hypertrophic cardiomyopathy (HCM), since the modern description of the disease by Teare over 40 years ago.1 HCM is now recognised as the most common cause of sudden cardiac death in young people, including trained athletes,2,3 although only a minority of all HCM patients are susceptible to the risk of sudden death (perhaps 10–15%).4 Nevertheless, the identification of this patient subset has been a priority, and the subject of a considerable body of literature.3–10 Because of the power and availability of the implantable cardioverter-defibrillator (ICD) for both primary and secondary prevention of sudden death, and its recent application to HCM,9 it now more important than ever to identify those patients within this broad disease spectrum who are at high risk for a catastrophic event.

    RISK STRATIFICATION PROFILES

    Defining reliable clinical markers for sudden death, and isolating the minority of HCM patients who are at high risk, has been an imposing task. This has been due largely to the relatively low prevalence of HCM within cardiology practice and its striking heterogeneity in clinical expression and outcome.3,10 Indeed, there is not yet a single test which is generally accepted as capable of assessing the risk level in all HCM patients with precision.

    Because HCM patients who are at risk for sudden death are often asymptomatic, risk factors assessed non-invasively have been most practical in clinical terms. The highest risk profiles in HCM are presently identified by one …