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Increasing evidence that risk scores underperform in predicting sudden death in hypertrophic cardiomyopathy
  1. Martin Maron,
  2. Ethan Rowin,
  3. Barry J Maron
  1. Hypertrophic Cardiomyopathy Center Division of Cardiology, Tufts Medical Center, Boston, Massachusetts, USA
  1. Correspondence to Dr Martin Maron, Tufts Medical Center, Boston, MA 02116, USA; mmaron{at}tuftsmedicalcenter.org

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Hypertrophic cardiomyopathy (HCM) remains the most common cause of sudden cardiac death (SCD) in young people, underscoring the need for accurate selection of patients for primary prevention implantable cardioverter-defibrillator (ICD) therapy.1 Since 2014, the European Society of Cardiology (ESC) has promoted a quantitative risk score, composed of seven disease-related features that can be imputed on a smart phone (ESC Risk Score App) to predict sudden death events over 5 years.2 The ESC risk score ascribes management recommendations for primary prevention ICDs based on whether a patient falls into one of three risk categories: low (<4%, ICD not indicated), intermediate (4%–6%, ICD could be considered) or high (≥6%, ICD should be considered). Of note, the ESC risk score excludes children and adolescent patients with HCM, a subgroup traditionally considered at particularly high risk for SCD. Prior investigations of this risk score have included only a small number of Asian patients with HCM2 3 and therefore the primary aim of the present Heart study by Choi et al,4 was to determine the efficacy of the HCM Risk-SCD calculator in a cohort of Korean patients with HCM.

In this observational cohort study, 730 Korean patients with HCM were followed for 4288 person-years for the primary endpoint of SCD events. Over the follow-up, 11 patients died suddenly and six experienced appropriate ICD shocks for ventricular tachyarrythmias, including seven patients with low ESC risk score, three with intermediate scores and six with high-risk scores. Of particular importance was the observation that …

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