Revisiting risk stratification in hypertrophic cardiomyopathy after the recent guidelines – Late gadolinium enhancement remains tough to beat

INTRODUCTION AND OBJECTIVES: Recommendations for implantable cardioverter-defibrillator (ICD) implantation to prevent sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) have evolved over time, with late gadolinium enhancement (LGE) emerging as an important additional risk marker. Our study aimed to evaluate changes in the accuracy and discriminative performance of international guidelines for ICD recommendations and to determine whether LGE can improve this risk stratification. METHODS: We conducted a multicenter retrospective observational study of HCM patients who underwent cardiac magnetic resonance (CMR) imaging for diagnosis or risk assessment. ICD eligibility was determined based on the ESC guidelines (2014, 2022, 2023) and the American College of Cardiology (ACC) guidelines (2011, 2020, 2024). Our primary endpoint was a composite of sudden cardiac death (SCD), appropriate ICD discharge, or sustained ventricular tachycardia (VT). RESULTS: A total of 531 patients (median age 49 years; 57% male) were included. During a median follow-up of 49 months, twenty-eight events occurred (15 SCDs, six appropriate ICD discharges, seven sustained VTs). Over time, both European and American guidelines demonstrated improved sensitivity, positive predictive value, and discriminatory ability, while maintaining a high negative predictive value. The agreement between the recent ESC and ACC guidelines was moderate (κ≈0.6, p