{
  "abstract": "Background The Japanese Hypertrophic Cardiomyopathy Registry Study was designed to provide comprehensive, real-world insights into the clinical characteristics and management of hypertrophic cardiomyopathy (HCM) in Japan.Methods This multicentre, prospective study enrolled consecutive patients with HCM from 24 referral hospitals across Japan starting in 2016. The baseline characteristics of 1485 patients enrolled by December 2019 are presented in this analysis.Results The median ages at registration and diagnosis were 69 and 60 years, respectively, with men accounting for 54% of the cohort. Familial HCM was confirmed in 18% of cases. Of the cohort, 36% had hypertrophic obstructive cardiomyopathy (HOCM), while 8% had mid-ventricular obstruction, 14% had apical HCM and 4% were in the end-stage phase. Atrial fibrillation was observed in 27% of patients, though the majority were asymptomatic or had mild symptoms at registration. Adverse outcomes included prior sustained ventricular tachycardia or fibrillation (6%), heart failure requiring hospitalisation (11%) and embolic events (5%). Defibrillator implantation was performed in 11% of patients. Differences in the defibrillator indications for primary prevention in the current three guidelines and status of defibrillator deployment at registration were clarified: the percentages of class IIa recommendation in the whole cohort and of patients with defibrillator implantation in class IIa were 22% and 19% in the Japanese guidelines, 4% and 39% in the European guidelines and 28% and 22% in the American guidelines, respectively. Beta blockers were prescribed to 90% of patients with HOCM, while 51% received cibenzoline. Septal reduction therapies were performed in 22% of patients with HOCM, with 6% undergoing surgical myectomy.Conclusions As the first large-scale, prospective HCM registry in Japan, this study provides valuable baseline data on the clinical characteristics and management of HCM. These findings will help address gaps between current practice and guideline recommendations, improving the care of patients with HCM.",
  "authors": [
    {
      "affiliations": [
        "Department of Cardiology and Geriatrics, Kochi University, Kochi, Japan"
      ],
      "name": "Toru Kubo"
    },
    {
      "affiliations": [
        "Department of Cardiology and Geriatrics, Kochi University, Kochi, Japan"
      ],
      "name": "Kenta Sugiura"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Nippon Medical School Hospital, Bunkyo-ku, Japan"
      ],
      "name": "Yukichi Tokita"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Nippon Medical School Hospital, Bunkyo-ku, Japan"
      ],
      "name": "Hitoshi Takano"
    },
    {
      "affiliations": [
        "Sakakibara Heart Institute, Fuchu, Japan"
      ],
      "name": "Itaru Takamisawa"
    },
    {
      "affiliations": [
        "Sakakibara Heart Institute, Fuchu, Japan"
      ],
      "name": "Morimasa Takayama"
    },
    {
      "affiliations": [
        "Cardiomyopathy Institute, Chikamori Hospital, Kochi, Japan"
      ],
      "name": "Yoshinori L Doi"
    },
    {
      "affiliations": [
        "Department of Cardiology, Tokyo Women’s Medical University, Tokyo, Japan"
      ],
      "name": "Yuichiro Minami"
    },
    {
      "affiliations": [
        "Department of Cardiology, Tokyo Women’s Medical University, Tokyo, Japan"
      ],
      "name": "Shota Shirotani"
    },
    {
      "affiliations": [
        "Showa University Fujigaoka Hospital, Yokohama, Japan"
      ],
      "name": "Mio Ebato"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Nippon Medical School Hospital, Bunkyo-ku, Japan"
      ],
      "name": "Miki Tsujiuchi"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Kitasato University School of Medicine, Kanagawa, Japan"
      ],
      "name": "Takeru Nabeta"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Niigata University School of Medical and Dental Sciences, Niigata, Japan"
      ],
      "name": "Takayuki Inomata"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Kyoto University Graduate School of Medicine, Kyoto, Japan"
      ],
      "name": "Takao Kato"
    },
    {
      "affiliations": [
        "Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Mie, Japan"
      ],
      "name": "Ryuji Okamoto"
    },
    {
      "affiliations": [
        "Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Mie, Japan"
      ],
      "name": "Kaoru Dohi"
    },
    {
      "affiliations": [
        "Department of Cardiology, Tokyo Medical University, Shinjuku-ku, Japan"
      ],
      "name": "Yasuyoshi Takei"
    },
    {
      "affiliations": [
        "Department of Cardiology, Tokyo Medical University, Shinjuku-ku, Japan"
      ],
      "name": "Taishiro Chikamori"
    },
    {
      "affiliations": [
        "Department of Cardiology, Fujita Health University Bantane Hospital, Nagoya, Japan"
      ],
      "name": "Eiichi Watanabe"
    },
    {
      "affiliations": [
        "Department of Cardiology, Hokkaido Cardiovascular Hospital, Hokkaido, Japan"
      ],
      "name": "Azusa Furugen"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Surgery, Sapporo Shiroishi Memorial Hospital, Hokkaido, Japan"
      ],
      "name": "Hirosato Doi"
    },
    {
      "affiliations": [
        "Division of Cardiology, Internal Medicine III, Hamamatsu University School of Medicine, Shizuoka, Japan"
      ],
      "name": "Keitaro Akita"
    },
    {
      "affiliations": [
        "Division of Cardiology, Internal Medicine III, Hamamatsu University School of Medicine, Shizuoka, Japan"
      ],
      "name": "Yuichiro Maekawa"
    },
    {
      "affiliations": [
        "Uwajima City Hospital, Uwajima, Ehime, Japan"
      ],
      "name": "Akiyoshi Ogimoto"
    },
    {
      "affiliations": [
        "Cardiology, Sendai Kousei Hospital, Miyagi, Japan"
      ],
      "name": "Norio Tada"
    },
    {
      "affiliations": [
        "Institute of Health Science Innovation for Medical Care, Hokkaido University Hospital, Hokkaido, Japan"
      ],
      "name": "Takashi Yokota"
    },
    {
      "affiliations": [
        "Department of Cardiology, Pulmonology, Hypertension and Nephrology, Ehime University Graduate School of Medicine, Ehime, Japan"
      ],
      "name": "Shuntaro Ikeda"
    },
    {
      "affiliations": [
        "Department of Cardiology, Pulmonology, Hypertension and Nephrology, Ehime University Graduate School of Medicine, Ehime, Japan"
      ],
      "name": "Osamu Yamaguchi"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Osaka Metropolitan University Graduate School of Medicine, Osaka, Japan"
      ],
      "name": "Yasuhiro Izumiya"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Osaka Metropolitan University Graduate School of Medicine, Osaka, Japan"
      ],
      "name": "Atsushi Shibata"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan"
      ],
      "name": "Seiji Takashio"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan"
      ],
      "name": "Kenichi Tsujita"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Tokyo Medical and Dental University, Tokyo, Japan"
      ],
      "name": "Yasuhiro Maejima"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan"
      ],
      "name": "Noboru Fujino"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan"
      ],
      "name": "Akihiro Nomura"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine and Hypertension, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan"
      ],
      "name": "Yuichi Akasaki"
    },
    {
      "affiliations": [
        "Department of Cardiovascular Medicine and Hypertension, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan"
      ],
      "name": "Koji Higuchi"
    },
    {
      "affiliations": [
        "Department of Cardiology, Osaka Medical and Pharmaceutical University, Osaka, Japan"
      ],
      "name": "Shuichi Fujita"
    },
    {
      "affiliations": [
        "Department of Cardiology, Osaka Medical and Pharmaceutical University, Osaka, Japan"
      ],
      "name": "Masaaki Hoshiga"
    },
    {
      "affiliations": [
        "Department of Cardiology, Keio University School of Medicine, Tokyo, Japan"
      ],
      "name": "Yasuyuki Shiraishi"
    },
    {
      "affiliations": [
        "Department of Cardiology, Keio University School of Medicine, Tokyo, Japan"
      ],
      "name": "Masaki Ieda"
    },
    {
      "affiliations": [
        "Department of Cardiology and Geriatrics, Kochi University, Kochi, Japan"
      ],
      "name": "Yuya Miyamoto"
    },
    {
      "affiliations": [
        "Department of Cardiology and Geriatrics, Kochi University, Kochi, Japan"
      ],
      "name": "Hiroaki Kitaoka"
    }
  ],
  "title": "Contemporary clinical characteristics and management patterns in hypertrophic cardiomyopathy: insights from baseline enrolment data in a nationwide prospective Japanese registry",
  "uid": "c90d7c8b-aea3-57cd-b82e-00e9001e78e6"
}