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Establishment of clinical guidelines for rare cardiac disease complicated by advanced heart failure

Research Project

Project/Area Number 17K09546
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Cardiovascular medicine
Research InstitutionNational Cardiovascular Center Research Institute

Principal Investigator

Seguchi Osamu  国立研究開発法人国立循環器病研究センター, 病院, 医長 (60570869)

Project Period (FY) 2017-04-01 – 2021-03-31
Project Status Completed (Fiscal Year 2020)
Budget Amount *help
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2019: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2018: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2017: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Keywords希少心筋疾患 / 2次性心筋症 / 心臓移植 / 人工心臓 / 機械的循環補助 / 重症心不全 / 補助人工心臓 / 筋ジストロフィー / 劇症型心筋炎 / 循環器・高血圧 / 移植・再生医療 / 臨床
Outline of Final Research Achievements

This study tried to establish the clinical guidelines for patients with rare cardiac disease complicated by advanced heart failure. During the study period, database of patients with rare cardiac disease receiving heart transplantation or mechanical circulatory support therapies in National Cerebral and cardiovascular Center, have been developed and is updating. Furthermore, the principal investigator has published case reports and case-series studies of patients with various rare cardiac diseases to share the tips of diagnosis and clinical course of those rare cardiac diseased complicated by advanced heart failure.

Academic Significance and Societal Importance of the Research Achievements

本研究で取り扱う疾患の中に筋ジストロフィーがある。本疾患は骨格筋の筋力が低下する遺伝性疾患であるが、心筋症を併発することが知られている。本研究期間中に主任研究者は心臓移植治療を受けた筋ジストロフィー患者9例の運動耐容能の改善について学術論文にまとめ、詳細に報告している。このように心臓移植後の筋ジストロフィー症例の筋力改善についての報告は過去に例がなく、筋ジストロフィー関連心筋症の重症心不全症例に対して心臓移植治療を前向きに考慮する一つの大きな根拠となると考えられ、社会的意義は大きい。

Report

(5 results)
  • 2020 Annual Research Report   Final Research Report ( PDF )
  • 2019 Research-status Report
  • 2018 Research-status Report
  • 2017 Research-status Report
  • Research Products

    (7 results)

All 2021 2020 2019 2018

All Journal Article (3 results) (of which Peer Reviewed: 3 results,  Open Access: 2 results) Presentation (4 results) (of which Int'l Joint Research: 3 results,  Invited: 3 results)

  • [Journal Article] Mechanical Circulatory Support Combined With Immunosuppression for the Treatment of Giant Cell Myocarditis ― A Single-Center Experience in Japan ―2020

    • Author(s)
      Nakajima-Doi S, Mochizuki H, Iwasaki K, Kuroda K, Watanabe T, Tadokoro N, Okada A, Asaumi Y, Kanzaki H, Fukushima S, Seguchi O, Yanase M, Izumi C, Fujita T, Kobayashi J, Fukushima N.
    • Journal Title

      Circulation Journal

      Volume: 84 Issue: 5 Pages: 815-819

    • DOI

      10.1253/circj.CJ-19-0847

    • NAID

      130007835088

    • ISSN
      1346-9843, 1347-4820
    • Year and Date
      2020-04-24
    • Related Report
      2020 Annual Research Report 2019 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Heart Transplantation Ameliorates Ambulation Capacity in Patients With Muscular Dystrophy ― An Analysis of 9 Cases ―2019

    • Author(s)
      Seguchi O, Kuroda K, Fujita T, Kumai Y, Nakajima S, Watanabe T, Yanase M, Matsumoto Y, Fukushima S, Kimura K, Fukushima N.
    • Journal Title

      Circulation Journal

      Volume: 83 Issue: 3 Pages: 684-686

    • DOI

      10.1253/circj.CJ-18-1060

    • NAID

      130007603745

    • ISSN
      1346-9843, 1347-4820
    • Year and Date
      2019-02-25
    • Related Report
      2019 Research-status Report 2018 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Left ventricular assist device implantation in an adult male with Danon disease.2019

    • Author(s)
      Kuroda K, Seguchi O, Matama H, Kimura Y, Iwasaki K, Toda K, Kikuchi N, Kumai Y, Nakajima S, Matsumoto Y, Watanabe T, Yanase M, Fukushima S, Tomoyuki F, Kobayashi J, Fukushima N.
    • Journal Title

      J Cardiol Cases.

      Volume: 3 Issue: 3 Pages: 106-109

    • DOI

      10.1016/j.jccase.2019.06.002

    • Related Report
      2019 Research-status Report
    • Peer Reviewed
  • [Presentation] Hypertrophic Cardiomyopathy with Left Ventricular Systolic Dysfunction: Comparison with Dilated Cardiomyopathy, and Clinical Outcome after Left Ventricular Assist Device Implantation2021

    • Author(s)
      Nobuichiro Yagi
    • Organizer
      ISHLT 2021 Annual meeting
    • Related Report
      2020 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Cardiac Transplantation in Patients with Muscular Dystrophy2019

    • Author(s)
      瀬口 理
    • Organizer
      日本神経学会
    • Related Report
      2019 Research-status Report
    • Int'l Joint Research / Invited
  • [Presentation] Cardiac Transplantation for Patients with Muscular Dystrophy2019

    • Author(s)
      Osamu Seguchi
    • Organizer
      60th Annual Meeting of The Japanese Society of Neurology
    • Related Report
      2018 Research-status Report
    • Int'l Joint Research / Invited
  • [Presentation] 神経・筋疾患合併心不全に対する植込型補助人工心臓2018

    • Author(s)
      瀬口理
    • Organizer
      第66回日本心臓病学会
    • Related Report
      2017 Research-status Report
    • Invited

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Published: 2017-04-28   Modified: 2022-01-27  

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