Kubo Toru, Baba Yuichi, Ochi Yuri, Takahashi Asa, Hirota Takayoshi, Yamasaki Naohito, Hamashige Naohisa, Yamamoto Katsuhito, Kondo Fumiaki, Bando Kanji, Yamada Eisuke, Furuno Takashi, Yabe Toshikazu, Doi Yoshinori L, Kitaoka Hiroaki
Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University Nankoku Japan.
Department of Cardiology, Chikamori Hospital Kochi Japan.
Circ Rep. 2020 Jul 2;2(8):433-439. doi: 10.1253/circrep.CR-20-0056.
Sudden cardiac death (SCD) is a most devastating complication of hypertrophic cardiomyopathy (HCM). The aim of this study was to clarify the clinical features of HCM in patients who experienced SCD-relevant events in an aged Japanese community. In 2004, we established a cardiomyopathy registration network in Kochi Prefecture, and herein report on 293 patients with HCM who are followed as part of the registry. The mean (±SD) age at registration and diagnosis was 63±14 and 56±16 years, respectively. SCD-relevant events occurred in 19 patients during a mean follow-up period of 6.1±3.2 years (incidence rate 1.0%/year): sudden death in 9 patients, successful recovery from cardiopulmonary arrest in 4 patients, and appropriate implantable cardioverter-defibrillator discharge in 6 patients. At registration, 13 patients were in the dilated phase of HCM (D-HCM). During the follow-up period, HCM developed to D-HCM in 21 patients; thus, 34 patients in total had D-HCM. Multivariate analysis revealed that D-HCM at registration or during follow-up and detection of non-sustained ventricular tachycardia (NSVT) during follow-up were significant predictors of SCD-relevant events. In this HCM population in an aged Japanese community, the annual rate of SCD-relevant events was 1.0%. HCM developed to D-HCM in a considerable number of patients, and D-HCM and NSVT were shown to be independently associated with an increased risk of SCD-relevant events.
心脏性猝死（SCD）是肥厚型心肌病（HCM）最具毁灭性的并发症。本研究的目的是阐明在日本老年社区中经历过与SCD相关事件的HCM患者的临床特征。2004年，我们在高知县建立了心肌病登记网络，本文报告了作为该登记系统一部分进行随访的293例HCM患者。登记和诊断时的平均（±标准差）年龄分别为63±14岁和56±16岁。在平均6.1±3.2年的随访期内，19例患者发生了与SCD相关的事件（发病率为1.0%/年）：9例患者猝死，4例患者心肺复苏成功，6例患者植入式心律转复除颤器适当放电。登记时，13例患者处于HCM的扩张期（D-HCM）。在随访期间，21例患者的HCM发展为D-HCM；因此，共有34例患者患有D-HCM。多变量分析显示，登记时或随访期间的D-HCM以及随访期间检测到非持续性室性心动过速（NSVT）是与SCD相关事件的重要预测因素。在这个日本老年社区的HCM人群中，与SCD相关事件的年发生率为1.0%。相当数量的患者HCM发展为D-HCM，并且D-HCM和NSVT被证明与SCD相关事件风险增加独立相关。
