When Validation Reveals the Limits ― The Next Step in Sudden Death Prevention for Hypertrophic Cardiomyopathy ―

years (area under the curve 0.75, 95% confidence interval 0.71-0.80)and, crucially, separated Class IIa from Class IIb (5-year cumulative incidence 6.7% vs. 4.9%, P=0.006).On the other hand, when the Class IIa threshold was used to simulate implantable cardioverter defibrillator (ICD) decisions, net reclassification improvement over contemporary American College of Cardiology (ACC)/American Heart Association (AHA) 2024 and European Society of Cardiology (ESC) 2023 frameworks was not significant. Sudden cardiac death (SCD) remains the most consequential event in hypertrophic cardiomyopathy (HCM).The 2018 Japanese Circulation Society/ Japanese Heart Failure Society (JCS/JHFS) guideline sought to organize everyday decision-making into a stepwise pathway tailored to Japanese practice and phenotypes, 1 and in this issue of the Journal, a new multicenter validation by Amano et al. tests how this framework performs in a large, real-world Japanese cohort and clarifies both its strengths and faults. 2 In 3,547 patients followed for a median of 5.8 years, SCD or an equivalent event occurred in 7.0%.The guideline achieved fair discrimination at 5

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Publication Details

Journal
Circulation Journal
Published
2025-12-11
DOI
https://doi.org/10.1253/circj.cj-25-0960
Primary Topic
Cardiomyopathy and Myosin Studies
Type
article
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article

When Validation Reveals the Limits ― The Next Step in Sudden Death Prevention for Hypertrophic Cardiomyopathy ―

Kenya Kusunose
Circulation Journal
Cardiomyopathy and Myosin Studies
article

When Validation Reveals the Limits ― The Next Step in Sudden Death Prevention for Hypertrophic Cardiomyopathy ―

Kenya Kusunose
article en

Abstract

years (area under the curve 0.75, 95% confidence interval 0.71-0.80)and, crucially, separated Class IIa from Class IIb (5-year cumulative incidence 6.7% vs. 4.9%, P=0.006).On the other hand, when the Class IIa threshold was used to simulate implantable cardioverter defibrillator (ICD) decisions, net reclassification improvement over contemporary American College of Cardiology (ACC)/American Heart Association (AHA) 2024 and European Society of Cardiology (ESC) 2023 frameworks was not significant. Sudden cardiac death (SCD) remains the most consequential event in hypertrophic cardiomyopathy (HCM).The 2018 Japanese Circulation Society/ Japanese Heart Failure Society (JCS/JHFS) guideline sought to organize everyday decision-making into a stepwise pathway tailored to Japanese practice and phenotypes, 1 and in this issue of the Journal, a new multicenter validation by Amano et al. tests how this framework performs in a large, real-world Japanese cohort and clarifies both its strengths and faults. 2 In 3,547 patients followed for a median of 5.8 years, SCD or an equivalent event occurred in 7.0%.The guideline achieved fair discrimination at 5

Circulation JournalVol. 90(2)
University of the Ryukyus (JP)
Openalex Percentile: Top 43%
Cardiomyopathy and Myosin Studies
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When Validation Reveals the Limits ― The Next Step in Sudden Death Prevention for Hypertrophic Cardiomyopathy ― — Kenya Kusunose · Circulation Journal (2025) | TGRS Research Map | TGRS