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Estimation of response to medical therapy in patients with atypical pulmonary arterial hypertension

Research Project

Project/Area Number 20K08465
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Review Section Basic Section 53020:Cardiology-related
Research InstitutionGunma University

Principal Investigator

Takama Noriaki  群馬大学, 医学部附属病院, 助教 (80638982)

Co-Investigator(Kenkyū-buntansha) 倉林 正彦  群馬大学, その他部局等, 名誉教授 (00215047)
Project Period (FY) 2020-04-01 – 2023-03-31
Project Status Completed (Fiscal Year 2022)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2022: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2021: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2020: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Keywords肺動脈性肺高血圧症 / 薬物治療 / 肺高血圧症 / 非典型的肺動脈性肺高血圧症 / 脂肪酸代謝関連因子
Outline of Research at the Start

本研究は非典型的肺動脈性肺高血圧症の病態を肺血管床や心臓における酸化ストレスや慢性炎症により惹起されるミトコンドリア機能異常症と捉え、分子レベルで収縮能が保持されている心不全(HFpEF)との相違を検討する。
これまでにHFpEF患者の血中に同定したミトコンドリア機能異常を反映すると推測できる脂肪酸代謝関連因子(FABP1, FABP4, βヒドロキシ酪酸(bOHB)、FGF21, 遊離脂肪酸)がtypical~atypicalのスペクトラムをもつ肺高血圧症患者の病態や治療反応性を予測するかを解析するものである。

Outline of Final Research Achievements

It was reported that high-dose epoprostenol treatment could improve not only hemodynamic improvements but also long-term prognosis for patients with severe pulmonary arterial hypertension. We estimated the relationships between response to medical therapy and their prognoses in patients with pulmonary arterial hypertension initiating epoprostenol treatment. We concluded that bleeding event in acute period is major poor prognosis factor for patients with severe pulmonary arterial hypertension initiating epoprostenol treatment. After acute period, one-year prognosis in patients with severe pulmonary arterial hypertension initiating epoprostenol treatment can show equal good prognosis compared with patients with oral combination therapy. On the other hand, high-dose epoprostenol therapy shows higher rate of endocrine diseases requiring treatment including thyroid, pituitary and pancreas disease.

Academic Significance and Societal Importance of the Research Achievements

今回の研究では、肺動脈性肺高血圧症症例において初期に適切な診断を行い、その症例の持つ重症度を評価することで治療介入後適切に状態ならびに液性因子の値も改善することが判明した。一方、本邦で重症例に対して実施されることがあるエポプロステノール高容量持続点滴治療に関しては、初期に出血事象で予後不良となることも確認できた。また治療介入後の経過を追ったことによりその後、重症例の約1/3の症例にて内分泌疾患(甲状腺、下垂体疾患、膵疾患など)を発症し治療介入が必要なことも判明した。

Report

(4 results)
  • 2022 Annual Research Report   Final Research Report ( PDF )
  • 2021 Research-status Report
  • 2020 Research-status Report
  • Research Products

    (5 results)

All 2023 2021 2020

All Journal Article (1 results) (of which Int'l Joint Research: 1 results,  Peer Reviewed: 1 results,  Open Access: 1 results) Presentation (4 results) (of which Int'l Joint Research: 2 results)

  • [Journal Article] Selexipag for the treatment of chronic thromboembolic pulmonary hypertension.2021

    • Author(s)
      Ogo T, Shimokawahara H, Kinoshita H, Sakao S, Abe K, Matoba S, Motoki H, Takama N, Ako J, Ikeda Y, Joho S, Maki H, Saeki T, Sugano T, Tsujino I, Yoshioka K, Shiota N, Tanaka S, Yamamoto C, Tanabe N, Tatsumi K; Study Group.
    • Journal Title

      Eur Respir J.

      Volume: 25 Issue: 1 Pages: 2101694-2101694

    • DOI

      10.1183/13993003.01694-2021

    • Related Report
      2021 Research-status Report
    • Peer Reviewed / Open Access / Int'l Joint Research
  • [Presentation] Bleeding event in acute period is major poor prognosis factor for patients with pulmonary arterial hypertension initiating epoprostenol treatment2023

    • Author(s)
      児玉智華
    • Organizer
      ACC 2023
    • Related Report
      2022 Annual Research Report
    • Int'l Joint Research
  • [Presentation] Relationships between endocrine diseases and high-dose epoprostenol therapy for patients with pulmonary arterial hypertension2023

    • Author(s)
      児玉智華
    • Organizer
      ACC 2023
    • Related Report
      2022 Annual Research Report
    • Int'l Joint Research
  • [Presentation] 肺動脈性肺高血圧症治療の最前線2021

    • Author(s)
      高間典明
    • Organizer
      肺高血圧セミナー
    • Related Report
      2020 Research-status Report
  • [Presentation] Ⅰ群PAHにおける呼吸器疾患合併症例~呼吸器内科より紹介となった肺高血圧症例の検討~2020

    • Author(s)
      高間典明
    • Organizer
      碓氷峠 PH Seminar<Case study meeting for patients>
    • Related Report
      2020 Research-status Report

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Published: 2020-04-28   Modified: 2024-01-30  

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