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Assessment of morphology/function of cardiac and skeletal muscle in lung disease-associated pulmonary hypertension

Research Project

Project/Area Number 18K08129
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Review Section Basic Section 53030:Respiratory medicine-related
Research InstitutionHokkaido University

Principal Investigator

Tsujino Ichizo  北海道大学, 医学研究院, 特任教授 (00344507)

Co-Investigator(Kenkyū-buntansha) 今野 哲  北海道大学, 医学研究院, 教授 (20399835)
大平 洋  北海道大学, 大学病院, 助教 (20528301)
真鍋 徳子  自治医科大学, 医学部, 教授 (70463742)
Project Period (FY) 2018-04-01 – 2021-03-31
Project Status Completed (Fiscal Year 2020)
Budget Amount *help
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2020: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000)
Fiscal Year 2019: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2018: ¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
Keywords肺高血圧症 / 右心不全 / 間質性肺疾患 / 慢性閉塞性肺疾患 / 病理 / 肺動脈 / 肺静脈 / 心臓MRI / 右室機能 / 右室・肺動脈カップリング / 肺疾患 / 血管 / 右心機能 / 心臓MRI / 右心 / 心筋 / 骨格筋
Outline of Final Research Achievements

In lung disease-associated pulmonary hypertension (Lung-PH), right ventricular (RV) contractility was increased whereas RV-pulmonary arterial coupling was impaired. Alternatively, RV stiffness was increased, potentially causing RV diastolic dysfunction in these patients.
The most common cause of death of Lung-PH patients was PH-related death (56%), followed by PH death (23%) and PH-unrelated death (21%). These results were different from those for pulmonary arterial hypertension (PAH) where PH itself was the most common cause of death (61%), and for group 4 PH where malignant disease at least partly contributed to the death by 60%. These findings were useful to further improve the management and outcome of patients with each PH type.
Lastly, in the pathological evaluations of the autopsied lungs of interstitial lung disease (ILD), arteriopathy at the site of active fibrosis was more prominent in those with PH than those without it.

Academic Significance and Societal Importance of the Research Achievements

肺高血圧症の5病型の一つである「肺疾患に伴う肺高血圧症」は、他の病型の肺高血圧症と比較して、診断後の余命が短く、疾患の正しい理解や治療法の改善が求められていた。今回の研究では、①肺疾患合併肺高血圧症の発症には肺の線維化が進行中の場所における肺動脈の病変の有無が重要であること、②肺動脈圧上昇の結果右心室の機能異常が起こっていること(右室・肺動脈カップリング障害と右室の硬化)、さらに③死因は肺高血圧症そのものよりも肺疾患のことの方が多いことを明らかにした。これらは「肺疾患に伴う肺高血圧症」に対する治療方法と診断後の患者さんを今後どのようにして改善していくかを考える上で有用な情報である。

Report

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

    (28 results)

All 2021 2020 2019 2018

All Journal Article (15 results) (of which Peer Reviewed: 15 results,  Open Access: 6 results) Presentation (13 results) (of which Int'l Joint Research: 3 results)

  • [Journal Article] Multi-Institutional Prospective Cohort Study of Patients With Pulmonary Hypertension Associated With Respiratory Diseases2021

    • Author(s)
      Tanabe N、Kumamaru H、Tamura Y、Taniguchi Hi、Emoto N、Yamada Y、Nishiyama O、Tsujino I、Kuraishi H、Nishimura Y、Kimura H、Inoue Y、Morio Y、Nakatsumi Y、Satoh T、Hanaoka M、Kusaka K、Sumitani M、Handa T、Sakao S、Kimura T、Kondoh Y、Nakayama K、Tanaka K、Ohira H、Nishimura M、Miyata H、Tatsumi K、for the JRPHS Group
    • Journal Title

      Circulation Journal

      Volume: 85 Issue: 4 Pages: 333-342

    • DOI

      10.1253/circj.CJ-20-0939

    • NAID

      130008003051

    • ISSN
      1346-9843, 1347-4820
    • Year and Date
      2021-03-25
    • Related Report
      2020 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Right ventricular pressure-volume loop produced with simultaneous application of three‐dimensional echocardiography and high‐fidelity micromanometry in a patient with pulmonary arterial hypertension2021

    • Author(s)
      Nakaya Toshitaka、Tsujino Ichizo、Nakamura Junichi、Chiba Yasuyuki、Iwano Hiroyuki
    • Journal Title

      Echocardiography

      Volume: NA Issue: 5 Pages: 805-807

    • DOI

      10.1111/echo.15032

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Right ventriculo-pulmonary arterial uncoupling and poor outcomes in pulmonary arterial hypertension2020

    • Author(s)
      Nakaya Toshitaka、Ohira Hiroshi、Sato Takahiro、Watanabe Taku、Nishimura Masaharu、Oyama-Manabe Noriko、Kato Masaru、Ito Yoichi M.、Tsujino Ichizo
    • Journal Title

      Pulmonary Circulation

      Volume: 10 Issue: 3 Pages: 1-11

    • DOI

      10.1177/2045894020957223

    • Related Report
      2020 Annual Research Report
    • Peer Reviewed
  • [Journal Article] A histopathological report of a 16-year-old male with peripheral pulmonary artery stenosis and Moyamoya disease with a homozygous RNF213 mutation2020

    • Author(s)
      Takahashi Kei、Nakamura Junichi、Sakiyama Shinya、Nakaya Toshitaka、Sato Takahiro、Watanabe Taku、Ohira Hiroshi、Makita Keishi、Tomaru Utano、Ishizu Akihiro、Tsujino Ichizo
    • Journal Title

      Respiratory Medicine Case Reports

      Volume: 29 Pages: 100977-100977

    • DOI

      10.1016/j.rmcr.2019.100977

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Guidelines for the Treatment of Pulmonary Hypertension (JCS 2017/JPCPHS 2017)2019

    • Author(s)
      Fukuda Keiichi、Kasai Takatoshi、et al、on behalf of the Japanese Circulation Society and the Japanese Pulmonary Circulation and Pulmonary Hypertension Society Joint Working Group
    • Journal Title

      Circulation Journal

      Volume: 83 Issue: 4 Pages: 842-945

    • DOI

      10.1253/circj.CJ-66-0158

    • NAID

      130007620555

    • ISSN
      1346-9843, 1347-4820
    • Year and Date
      2019-03-25
    • Related Report
      2019 Research-status Report 2018 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Chinese herbal medicine Qing-Dai-induced pulmonary arterial hypertension in a patient with ulcerative colitis: A case report and experimental investigation2019

    • Author(s)
      Sato Kazuki、Ohira Hiroshi、Horinouchi Takahiro、Nakaya Toshitaka、Mazaki Yuichi、Sugimoto Ayako、Watanabe Taku、Tsujino Ichizo、Nishimura Masaharu
    • Journal Title

      Respiratory Medicine Case Reports

      Volume: 26 Pages: 265-269

    • DOI

      10.1016/j.rmcr.2019.02.007

    • NAID

      120006626587

    • Related Report
      2019 Research-status Report 2018 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Reduced diffusing capacity for carbon monoxide predicts borderline pulmonary arterial pressure in patients with systemic sclerosis2019

    • Author(s)
      Ninagawa K, Kato M, Nakamura H, Tsujino I, et al (a total of 11 authors)
    • Journal Title

      Rheumatol Int.

      Volume: 39 Issue: 11 Pages: 1883-1887

    • DOI

      10.1007/s00296-019-04370-0

    • NAID

      120006941690

    • Related Report
      2019 Research-status Report
    • Peer Reviewed
  • [Journal Article] Prognostic value of phase analysis on gated single photon emission computed tomography in patients with cardiac sarcoidosis2019

    • Author(s)
      Koyanagawa Kazuhiro、Naya Masanao、Aikawa Tadao、Manabe Osamu、Kuzume Masato、Ohira Hiroshi、Tsujino Ichizo、Tamaki Nagara、Anzai Toshihisa
    • Journal Title

      Journal of Nuclear Cardiology

      Volume: 印刷中 Issue: 1 Pages: 128-136

    • DOI

      10.1007/s12350-019-01660-9

    • NAID

      120007189933

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] Cardiac sarcoidosis classification with deep convolutional neural network-based features using polar maps2019

    • Author(s)
      Togo Ren、Hirata Kenji、Manabe Osamu、Ohira Hiroshi、Tsujino Ichizo、Magota Keiichi、Ogawa Takahiro、Haseyama Miki、Shiga Tohru
    • Journal Title

      Computers in Biology and Medicine

      Volume: 104 Pages: 81-86

    • DOI

      10.1016/j.compbiomed.2018.11.008

    • NAID

      120006776823

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] Successful Application of Edoxaban in the Treatment of Venous Thromboembolism Recurrence in a Patient with Non-small Cell Lung Cancer after Tumor Shrinkage2018

    • Author(s)
      Shoji Tetsuaki、Mizugaki Hidenori、Ikezawa Yasuyuki、Furuta Megumi、Takashima Yuta、Kikuchi Hajime、Goudarzi Houman、Asahina Hajime、Kikuchi Junko、Kikuchi Eiki、Sakakibara-Konishi Jun、Shinagawa Naofumi、Tsujino Ichizo、Nishimura Masaharu
    • Journal Title

      Internal Medicine

      Volume: 57 Issue: 12 Pages: 1769-1772

    • DOI

      10.2169/internalmedicine.9741-17

    • NAID

      130007397149

    • ISSN
      0918-2918, 1349-7235
    • Year and Date
      2018-06-15
    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] Use of 18F-FDG PET/CT texture analysis to diagnose cardiac sarcoidosis2018

    • Author(s)
      Manabe O, Ohira H, Hirata K, Hayashi S, Naya M, Tsujino I, Aikawa T, Koyanagawa K, Oyama-Manabe N, Tomiyama Y, Magota K, Yoshinaga K, Tamaki N
    • Journal Title

      Eur J Nucl Med Mol Imaging

      Volume: - Issue: 6 Pages: 1240-1247

    • DOI

      10.1007/s00259-018-4195-9

    • Related Report
      2018 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Which is the proper reference tissue for measuring the change in FDG PET metabolic volume of cardiac sarcoidosis before and after steroid therapy?2018

    • Author(s)
      Furuya Sho、Manabe Osamu、Ohira Hiroshi、Hirata Kenji、Aikawa Tadao、Naya Masanao、Tsujino Ichizo、Koyanagawa Kazuhiro、Anzai Toshihisa、Oyama-Manabe Noriko、Shiga Tohru
    • Journal Title

      EJNMMI Research

      Volume: 8 Issue: 1 Pages: 94-94

    • DOI

      10.1186/s13550-018-0447-8

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension: A systematic review2018

    • Author(s)
      Tanabe Nobuhiro、Kawakami Takashi、Satoh Toru、Matsubara Hiromi、Nakanishi Norifumi、Ogino Hitoshi、Tamura Yuichi、Tsujino Ichizo、Ogawa Aiko、Sakao Seiichiro、Nishizaki Mari、Ishida Keiichi、Ichimura Yasunori、Yoshida Masahiro、Tatsumi Koichiro
    • Journal Title

      Respiratory Investigation

      Volume: 56 Issue: 4 Pages: 332-341

    • DOI

      10.1016/j.resinv.2018.03.004

    • Related Report
      2018 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Amelioration of right ventricular systolic function and stiffness in a patient with idiopathic pulmonary arterial hypertension treated with oral triple combination therapy2018

    • Author(s)
      Nakaya Toshitaka、Tsujino Ichizo、Ohira Hiroshi、Sato Takahiro、Watanabe Taku、Oyama-Manabe Noriko、Nishimura Masaharu
    • Journal Title

      Pulmonary Circulation

      Volume: 8 Issue: 2 Pages: 1-5

    • DOI

      10.1177/2045894018765350

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Journal Article] Efficient detection of pulmonary arterial hypertension using serum haptoglobin level and cardiac MRI in patients with connective tissue diseases: a pilot study2018

    • Author(s)
      Nakamura H, Kato M, Nogochi A, Ohira H, Tsujino I, Atsumi T
    • Journal Title

      Clin Exp Rheumatol

      Volume: 36 Pages: 345-346

    • Related Report
      2018 Research-status Report
    • Peer Reviewed
  • [Presentation] 間質性肺疾患合併肺高血圧症の肺血管形態に関する病理学的検討2021

    • Author(s)
      杉本 絢子、辻野 一三、中村 順一、中谷 資隆、佐藤 隆博、渡部 拓、大平 洋、鈴木 雅、今野 哲、大塚 紀幸
    • Organizer
      第6回 肺高血圧・肺循環学会
    • Related Report
      2020 Annual Research Report
  • [Presentation] 3群肺高血圧症における右室機能障害および予後との関連2020

    • Author(s)
      島 秀起、辻野一三、中谷資隆、中村順一、杉本絢子、渡部 拓、大平 洋、佐藤隆博、今野 哲
    • Organizer
      第5回 肺高血圧・肺循環学会
    • Related Report
      2020 Annual Research Report
  • [Presentation] Sequential analysis of right ventricular function and exercise capacity in pulmonary arterial hypertension2019

    • Author(s)
      T. Nakaya M.D.1, T. Sato M.D., Ph.D.1, T. Watanabe M.D., Ph.D.1, H. Ohira M.D., Ph.D.1 , N. Oyama-Manabe M.D., Ph.D.2, I. Tsujino M.D., Ph.D.1
    • Organizer
      European Respiratory Society 2019
    • Related Report
      2019 Research-status Report
    • Int'l Joint Research
  • [Presentation] PAH~“Classic” vs “Left heart/Pulmonary” phenotype2019

    • Author(s)
      辻野一三
    • Organizer
      第83回日本循環器学会総会
    • Related Report
      2018 Research-status Report
  • [Presentation] 末梢型肺動脈狭窄症の1例2019

    • Author(s)
      中村順一、辻野一三ほか
    • Organizer
      第117回日本呼吸器学会北海道支部学術集会
    • Related Report
      2018 Research-status Report
  • [Presentation] Associations between intrinsic right ventricular function and exercise tolerance/ventilatory efficiency in pulmonary arterial hypertension2018

    • Author(s)
      Nakaya T, Tsujino I, et al
    • Organizer
      European Respiratory Society, International Congress 2018
    • Related Report
      2018 Research-status Report
    • Int'l Joint Research
  • [Presentation] Risk of underdiagnoses of cardiac sarcoidosis by routine electrocardiogram and echocardiogram in patients with biopsy-proven extracardiac sarcoidosis2018

    • Author(s)
      Ohira H, Tsujino I, et al.
    • Organizer
      European Respiratory Society, International Congress 2018
    • Related Report
      2018 Research-status Report
    • Int'l Joint Research
  • [Presentation] 肺動脈性肺高血圧症の右室機能と運動耐容能・換気効率の関連性2018

    • Author(s)
      中谷 資隆, 大平 洋, 佐藤 隆博, 渡部 拓, 真鍋 徳子, 絹川 真太郎, 西村 正治, 辻野 一三
    • Organizer
      第3回日本肺高血圧・肺循環学会
    • Related Report
      2018 Research-status Report
  • [Presentation] 肺動脈性肺高血圧症におけるインスリン抵抗性の有無と臨床的意義2018

    • Author(s)
      崎山信哉、大平 洋、黒木 茜、中谷資隆、鈴木奈緒子、林下晶子、佐藤隆博、渡部 拓、西村正治、辻野一三
    • Organizer
      第3回日本肺高血圧・肺循環学会
    • Related Report
      2018 Research-status Report
  • [Presentation] 肺高血圧合併間質性肺炎の診断における肺拡散能力検査の有用性2018

    • Author(s)
      清水薫子, 辻野一三, 大平 洋, 渡部 拓, 杉本絢子, 中谷資隆, 鈴木 雅, 今野 哲, 西村正治
    • Organizer
      第3回日本肺高血圧・肺循環学会
    • Related Report
      2018 Research-status Report
  • [Presentation] 青黛関連肺動脈性肺高血圧症2018

    • Author(s)
      大平 洋, 堀之内孝広, 佐藤一紀, 中谷 資隆, 杉本 絢子, 渡部 拓, 西村 正治, 辻野一三
    • Organizer
      第3回日本肺高血圧・肺循環学会
    • Related Report
      2018 Research-status Report
  • [Presentation] 運動負荷試験~肺疾患合併PHの病態の理解と治療戦略構築における活用術~2018

    • Author(s)
      辻野一三
    • Organizer
      第3回日本肺高血圧・肺循環学会
    • Related Report
      2018 Research-status Report
  • [Presentation] 診断法とその問題点からみた第3群肺高血圧症2018

    • Author(s)
      辻野一三
    • Organizer
      第58回日本呼吸器学会
    • Related Report
      2018 Research-status Report

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Published: 2018-04-23   Modified: 2022-01-27  

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