Validation of Guideline Recommendation on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy (JACC Heart Failure June 2025)-Article: Validation of Guideline Recommendation on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy

Article: Validation of Guideline Recommendation on Sudden Cardiac Death Prevention in Hypertrophic Cardiomyopathy

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This study focuses on evaluating guideline recommendations for preventing sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM) using a large-scale Japanese registry. The study involved 3,611 patients and compared the effectiveness of the 2024 American College of Cardiology/American Heart Association (ACC/AHA) guidelines and the 2023 European Society of Cardiology (ESC) guidelines in stratifying risk for SCD. Results indicated that both sets of guidelines have favorable overall performance, but they could not adequately differentiate between certain risk categories (Classes 2a and 2b).<br /><br />Notable findings include that patients with end-stage HCM have significantly worse prognosis compared to other HCM phenotypes, with a 5-year incidence of SCD events as high as 18.5% for those with reduced ejection fractions of 50% or less. By contrast, non-obstructive and midventricular HCM showed 5-year incidence rates of 4.7% and 6.9%, respectively. <br /><br />Classifications under the ACC/AHA guidelines seemed more effective at distinguishing between different SCD risk levels than the ESC guidelines, although neither guideline adequately differentiated risk between some intermediate categories. This was evident as there were reversed event rates in some classifications using the ESC guidelines.<br /><br />The study suggests the need for revisiting risk stratification methods in these guidelines due to significant ethnic and phenotypic differences noted in the Japanese cohort, which were not as apparent in Western populations. Given the substantial proportion of missing data, especially for ambulatory ECG and cardiac magnetic resonance imaging, the generalizability of these results is cautioned. <br /><br />Authors highlight potential areas of improvement in SCD risk stratification, giving preference to cohort-specific considerations, and call for an original model adjusted for ethnic variations and HCM phenotypes, especially for non-Western patients.

Keywords

sudden cardiac death

hypertrophic cardiomyopathy

ACC/AHA guidelines

ESC guidelines

risk stratification

Japanese registry

end-stage HCM

ethnic differences

non-obstructive HCM

phenotypic variations