Risk stratification for sudden cardiac death (SCD) is essential in the management of hypertrophic cardiomyopathy (HC). The 2014 European Society of Cardiology SCD risk prediction model (Risk-SCD) is a novel risk scoring system; however, whether it can be applied to Japanese HC and its usefulness among various HC phenotypes remain unclear. The aim of this study was to validate the Risk-SCD model in Japanese HC, and to evaluate its usefulness among various HC phenotypes. We studied 370 consecutive Japanese HC patients evaluated for primary SCD prevention at our tertiary referral center. The Risk-SCD model was validated in 289 HC patients with ejection fraction (EF) ≥50% (including left ventricular outflow tract obstruction [LVOTO], mid ventricular obstruction [MVO], apical hypertrophy, and nonobstructive phenotypes), and 81 end-stage HC patients (EF <50%). The end point of the study was SCD or an equivalent event (appropriate implantable cardioverter defibrillator therapy or successful resuscitation after cardiac arrest). Thirty-one SCD events were observed during a median follow-up of 5.2 (interquartile range 3.5 to 6.9) years. The Risk-SCD model showed improved risk prediction in HC with EF ≥50% compared with the previous 2011 American College of Cardiology Foundation/American Heart Association and 2003 American College of Cardiology/European Society of Cardiology guideline approaches (number needed to treat = 3.8 at Risk-SCD >6%) regardless of phenotypes; LVOTO, MVO, apical, and nonobstructive, but misclassified SCD risk in end-stage HC. In the current external validation of the Risk-SCD model in Japanese HC, the model improved SCD prediction compared with previous approaches, and was also shown to be useful in LVOTO, MVO, apical, and nonobstructive phenotypes.
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