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Validation of the 2018 Japanese Circulation Society (JCS)/Japanese Heart Failure Society (JHFS) Guidelines for

Masashi Amano1, Hiroaki Kitaoka2, Yusuke Yoshikawa3,4

  • 1Department of Heart Failure and Transplantation, National Cerebral and Cardiovascular Center.

Circulation Journal : Official Journal of the Japanese Circulation Society
|December 10, 2025
PubMed

Insights

The 2018 Japanese Circulation Society (JCS)/Japanese Heart Failure Society (JHFS) guidelines effectively predict sudden cardiac death (SCD) risk in hypertrophic cardiomyopathy (HCM) patients. These guidelines show good discriminatory performance, especially for those recommended for defibrillators.

Area of Science:

  • Cardiology
  • Clinical Risk Stratification
  • Hypertrophic Cardiomyopathy Research

Background:

  • Sudden cardiac death (SCD) poses a significant threat to patients with hypertrophic cardiomyopathy (HCM).
  • Validation of existing SCD prevention guidelines is crucial in diverse patient populations.
  • The Japanese Circulation Society (JCS)/Japanese Heart Failure Society (JHFS) guidelines require assessment in Japanese HCM cohorts.

Purpose of the Study:

  • To validate the 2018 JCS/JHFS guidelines for SCD risk stratification in a large cohort of Japanese HCM patients.
  • To evaluate the discriminatory performance of the JCS/JHFS guidelines in predicting SCD events.

Main Methods:

  • A subanalysis of the REVEAL-HCM registry included 3,547 Japanese patients with HCM.
  • The study assessed the composite endpoint of SCD or equivalent events over a median follow-up of 5.8 years.
  • The HCM Risk-SCD score was used to categorize patient risk according to the JCS/JHFS guidelines.

Main Results:

  • During follow-up, 247 (7.0%) patients experienced SCD events.
  • A significant difference in 5-year cumulative SCD incidence was observed across guideline classes (2a, 2b, and 3).
  • The 2018 JCS/JHFS guidelines demonstrated an area under the curve of 0.75 for predicting SCD events at 5 years.

Conclusions:

  • The 2018 JCS/JHFS guidelines exhibit strong discriminatory performance for SCD risk stratification in Japanese HCM patients.
  • The guidelines are particularly effective in identifying high-risk individuals, including those with Class 2a recommendations for implantable cardioverter-defibrillators.
Abstract

Keywords:
GuidelineHypertrophic cardiomyopathyImplantable cardioverter defibrillatorSudden cardiac death

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