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Observational Study
. 2025 Jun;13(6):1014-1026.
doi: 10.1016/j.jchf.2024.12.006. Epub 2025 Mar 12.

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

Masashi Amano  1 , Hiroaki Kitaoka  2 , Yusuke Yoshikawa  3 , Yasushi Sakata  4 , Kaoru Dohi  5 , Yukichi Tokita  6 , Takao Kato  7 , Shouji Matsushima  8 , Takeshi Kitai  1 , Atsushi Okada  1 , Yutaka Furukawa  9 , Toshihiro Tamura  10 , Akihiro Hayashida  11 , Haruhiko Abe  12 , Kenji Ando  13 , Satoshi Yuda  14 , Moriaki Inoko  15 , Kazushige Kadota  16 , Yukio Abe  17 , Katsuomi Iwakura  18 , Tetsuya Kitamura  19 , Jun Masuda  20 , Takahiro Ohara  21 , Takashi Omura  22 , Takashi Tanigawa  23 , Kenji Nakamura  24 , Kunihiro Nishimura  25 , Chisato Izumi  26 ; REVEAL-HCM Investigators
Collaborators, Affiliations
Free article
Observational Study

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

Masashi Amano et al. JACC Heart Fail. 2025 Jun.
Free article

Abstract

Background: To prevent sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM), the HCM Risk-SCD calculator and guideline recommendations are used to aid decision making for implantable cardioverter-defibrillator placement.

Objectives: The aim of this study was to assess the clinical profiles and occurrence of SCD by phenotypes of HCM and validate the performance of the current guidelines from a large-scale Japanese multicenter registry.

Methods: This was a retrospective, multicenter, observational, longitudinal cohort study that enrolled 3,611 consecutive patients with HCM. The primary endpoint was a composite of SCD or an equivalent event.

Results: The 5-year cumulative incidence of SCD events was markedly high in patients with end-stage HCM, defined by ejection fraction <50% (18.5%), followed by midventricular obstruction and nonobstructive HCM (6.9% and 4.7%). The 5-year cumulative incidence rates of SCD events for each recommendation class by the 2 guidelines were as follows: with the 2024 ACC (American College of Cardiology)/AHA (American Heart Association) guidelines, 23.8%, 7.2%, 5.7%, and 2.3% for Classes 1, 2a, 2b, and 3, respectively, and with the 2023 ESC (European Society of Cardiology) guidelines, 23.8%, 2.9%, 9.3%, and 2.6%, respectively. The 5-year risk was not well stratified between Classes 2a and 2b with the 2024 ACC/AHA guidelines (P = 0.101), and the event rate was even reversed with the 2023 ESC guidelines (P = 0.545).

Conclusions: Among HCM phenotypes, the prognosis of patients with end-stage HCM was markedly worse. The 2024 ACC/AHA and 2023 ESC guidelines well stratified SCD risk in patients with HCM; the 2024 ACC/AHA guidelines seemed to better stratify SCD risk between Classes 2a and 2b compared with the 2023 ESC guidelines.

Keywords: ejection fraction; end-stage hypertrophic cardiomyopathy; hypertrophic cardiomyopathy; implantable cardioverter-defibrillator; sudden cardiac death.

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Conflict of interest statement

Funding Support and Author Disclosures This work was supported by the Japan Agency for Medical Research and Development (grant JP23ek0109539). Dr Sakata has received grants or honoraria from Abbott Medical Japan, Otsuka Pharmaceutical, Nippon Boehringer Ingelheim, AstraZeneca, Novartis Pharma, and Bayer. Dr Furukawa has received personal fees from Novartis. Dr Abe has received personal fees from Boehringer Ingelheim Japan. Dr Ando has received personal fees from Abbott Medical Japan, Medtronic Japan, and Biotronik. Dr Iwakura has received speaker honoraria from AstraZeneca and Eli Lilly and Company. Dr Kitamura has received personal fees from Daiichi-Sankyo and Abbott Medical Japan. Dr Izumi has received speaker honoraria from Daiichi-Sankyo, Nippon Boehringer Ingelheim, and Novartis; and has received research funding from Pfizer, LSI Medience, PPD-SNBL, Abott Medical Japan, Bristol Myers Squibb, and Eli Lilly and Company. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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