Amano Masashi, Kitaoka Hiroaki, Yoshikawa Yusuke, Sakata Yasushi, Dohi Kaoru, Tokita Yukichi, Kato Takao, Matsushima Shouji, Kitai Takeshi, Okada Atsushi, Furukawa Yutaka, Tamura Toshihiro, Hayashida Akihiro, Abe Haruhiko, Ando Kenji, Yuda Satoshi, Inoko Moriaki, Kadota Kazushige, Abe Yukio, Iwakura Katsuomi, Kitamura Tetsuya, Masuda Jun, Ohara Takahiro, Omura Takashi, Tanigawa Takashi, Nakamura Kenji, Nishimura Kunihiro, Izumi Chisato
Department of Heart Failure and Transplantation, National Cerebral and Cardiovascular Center, Suita, Japan.
Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University, Kochi, Japan.
JACC Heart Fail. 2025 Jun;13(6):1014-1026. doi: 10.1016/j.jchf.2024.12.006. Epub 2025 Mar 12.
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.
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.
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.
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).
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.
为预防肥厚型心肌病（HCM）患者的心源性猝死（SCD），HCM风险-SCD计算器和指南建议被用于辅助植入式心脏复律除颤器植入的决策制定。
本研究旨在通过HCM的表型评估临床特征和SCD的发生情况，并从日本大规模多中心注册研究中验证现行指南的性能。
这是一项回顾性、多中心、观察性纵向队列研究，纳入了3611例连续的HCM患者。主要终点是SCD或等效事件的复合终点。
射血分数<50%定义的终末期HCM患者的SCD事件5年累积发生率显著较高（18.5%），其次是室中隔梗阻和非梗阻性HCM（6.9%和4.7%）。根据这两个指南，每个推荐类别的SCD事件5年累积发生率如下：根据2024年美国心脏病学会（ACC）/美国心脏协会（AHA）指南，1类、2a类、2b类和3类分别为23.8%、7.2%、5.7%和2.3%；根据2023年欧洲心脏病学会（ESC）指南，分别为23.8%、2.9%、9.3%和2.6%。根据2024年ACC/AHA指南，2a类和2b类之间的5年风险分层不佳（P = 0.101），而根据2023年ESC指南，事件发生率甚至出现逆转（P = 0.545）。
在HCM表型中，终末期HCM患者的预后明显更差。2024年ACC/AHA和2023年ESC指南对HCM患者的SCD风险进行了良好分层；与2023年ESC指南相比，2024年ACC/AHA指南在2a类和2b类之间对SCD风险的分层似乎更好。
