HE RISK OF SUDDEN DEATH IN PA-tients with hypertrophic cardiomyopathy (HCM) has been known for almost 50 years. [1][2][3][4][5][6] Indeed, this disease is the most common cause of sudden cardiac death in young people, 1-6 including trained athletes. 7 However, only in the last few years has the implantable cardioverterdefibrillator (ICD) been systematically used as a potentially life-saving For editorial comment see p 452.
Hypertrophic cardiomyopathy (HCM) is an important genetic heart muscle disease for which prevalence in the general population has not been completely resolved. For the past 20 years, most data have supported the occurrence of HCM at about 1 in 500. However, the authors have interrogated a number of relevant advances in cardiovascular medicine, including widespread fee-for-service genetic testing, population genetic studies, and contemporary diagnostic imaging, as well as a greater index of suspicion and recognition for both the clinically expressed disease and the gene-positive-phenotype-negative subset (at risk for developing the disease). Accounting for the potential impact of these initiatives on disease occurrence, the authors have revisited the prevalence of HCM in the general population. They suggest that HCM is more common than previously estimated, which may enhance its recognition in the practicing cardiovascular community, allowing more timely diagnosis and the implementation of appropriate treatment options for many patients.
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