Hypertrophic cardiomyopathy and sudden death: new perspectives on risk stratification and prevention with the implantable cardioverter-defibrillator.

Hypertrophic cardiomyopathy is a genetic cardiac disease with a particularly heterogeneous presentation and diverse natural history. Sudden and unexpected death has been recognized as a prominent and devastating consequence of hypertrophic cardiomyopathy since the initial description of this disease over 40 years ago. Many authors have emphasized that these catastrophic events occur not uncommonly in young asymptomatic patients with annual mortality rates reportedly as high as 4–6% in tertiary centre referral populations disproportionately comprised of high-risk patients.