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Right ventricular kinematic model and pahological findings in pulmonary arterial hypertension

Research Project

Project/Area Number 19K08279
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Review Section Basic Section 52050:Embryonic medicine and pediatrics-related
Research InstitutionThe University of Tokushima

Principal Investigator

HAYABUCHI Yasunobu  徳島大学, 病院, 特任教授 (20403686)

Project Period (FY) 2019-04-01 – 2022-03-31
Project Status Completed (Fiscal Year 2021)
Budget Amount *help
¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000)
Fiscal Year 2021: ¥260,000 (Direct Cost: ¥200,000、Indirect Cost: ¥60,000)
Fiscal Year 2020: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2019: ¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
Keywords右室 / 肺高血圧 / 運動力学 / 心筋病理
Outline of Research at the Start

小児の肺動脈性肺高血圧症は非常に難治な疾患であり、右室機能はその生命予後に大きく関わるが右心不全に対する治療方法は未だ確立されていない。右室収縮不全の発症前段階には拡張不全が認められるが、その病態と進行の詳細も解明されていない。
本研究では、右室圧・心筋壁の運動力学と心筋病理・機能を統合することで、右室拡張障害の早期診断と経時的変化の診断および治療効果判定への応用・開発を目的とした。

Outline of Final Research Achievements

Right ventricular pressure measurements were recorded using a pressure wire, and right ventricular pressure drop waveforms from isovolumic diastole to early diastole were fitted to the equation of motion of decaying oscillations to evaluate relaxation and ventricular stiffness. The consistency with myocardial histopathological findings and molecular biological characteristics was examined. Indices of stiffness and relaxation of right ventricular myocardium applied to the equation of motion for damped oscillations showed findings indicative of pathological findings. The severity of histopathological abnormalities in the right ventricular myocardium could be estimated from the right ventricular pressure waveform. Our results suggest that a detailed prognostic evaluation of right ventricular function is possible by integrating kinomechanics and myocardial pathology/function.

Academic Significance and Societal Importance of the Research Achievements

本研究は、右室圧・心筋壁の運動力学と心筋病理・機能を統合することで、右室拡張障害の早期診断と経時的変化の早期診断と経時的変化の診断および治療効果判定への応用・開発を目的とした。右室の等容性拡張期から拡張期における右室圧降下波形を減衰振動の運動方程式にあてはめ、弛緩(relaxation)、心室の硬さ(stiffness)を評価した。次に、心筋組織病理学的所見・分子生物学的特徴との合致性を検討した。まず、右室圧 P(t)を減衰振動の運動方程式に適用した右室心筋のstiffnessやrelaxationの指標は病理学的所見や分子生物学的検討との合致性や関連性が示唆された。

Report

(4 results)
  • 2021 Annual Research Report   Final Research Report ( PDF )
  • 2020 Research-status Report
  • 2019 Research-status Report
  • Research Products

    (35 results)

All 2022 2021 2020 2019

All Journal Article (14 results) (of which Peer Reviewed: 14 results,  Open Access: 11 results) Presentation (21 results) (of which Invited: 3 results)

  • [Journal Article] Duplications in the G3 domain or switch II region in HRAS identified in patients with Costello syndrome2022

    • Author(s)
      Nagai K, Niihori T, Okamoto N, Kondo A, Suga K, Ohhira T, Hayabuchi Y, Homma Y, Nakagawa R, Ifuku T, Abe T, Mizuguchi T, Matsumoto N, Aoki Y.
    • Journal Title

      Hum Mutat

      Volume: 43 Issue: 1 Pages: 3-15

    • DOI

      10.1002/humu.24287

    • Related Report
      2021 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Functionally confirmed compound heterozygous ADAM17 missense loss-of-function variants cause neonatal inflammatory skin and bowel disease 12021

    • Author(s)
      Imoto I, Saito M, Suga K, Kohmoto T, Otsu M, Horiuchi K, Nakayama H, Higashiyama S, Sugimoto M, Sasaki A, Homma Y, Shono M, Nakagawa R, Hayabuchi Y, Tange S, Kagami S, Masuda K
    • Journal Title

      Scientific reports

      Volume: 11 Issue: 1 Pages: 9552-9552

    • DOI

      10.1038/s41598-021-89063-0

    • NAID

      120007188413

    • Related Report
      2021 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Three-dimensional imaging of pulmonary arterial vasa vasorum using optical coherence tomography in patients after bidirectional Glenn and Fontan procedures2021

    • Author(s)
      Hayabuchi Y, Homma Y, Kagami S.
    • Journal Title

      Eur Heart J Cardiovasc Imaging

      Volume: 22 Issue: 8 Pages: 941-949

    • DOI

      10.1093/ehjci/jeaa098

    • Related Report
      2021 Annual Research Report 2020 Research-status Report
    • Peer Reviewed
  • [Journal Article] Pregnancy and Delivery in Patients With Repaired Congenital Heart Disease ― A Retrospective Japanese Multicenter Study ―2020

    • Author(s)
      Takatsuki Shinichi、Furutani Yoshiyuki、Inai Kei、Kobayashi Tohru、Inuzuka Ryo、Uyeda Tomomi、Kamisago Mitsuhiro、Muneuchi Jun、Kaneko Masahide、Misaki Yasushi、Ono Hiroshi、Kato Hitoshi、Shimada Eriko、Shinohara Tokuko、Waki Kenji、Suda Kenji、Hayabuchi Yasunobu、 Ohki Hirotaka 、Hiroyuki Yamagishi 、et al.
    • Journal Title

      Circulation Journal

      Volume: 84 Issue: 12 Pages: 2270-2274

    • DOI

      10.1253/circj.CJ-19-1150

    • NAID

      130007942471

    • ISSN
      1346-9843, 1347-4820
    • Year and Date
      2020-11-25
    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Next-generation sequencing for the diagnosis of patients with congenital multiple anomalies and / or intellectual disabilities2020

    • Author(s)
      Suga Kenichi、Imoto Issei、Ito Hiromichi、Naruto Takuya、Goji Aya、Osumi Keita、Tokaji Narumi、Homma Yukako、Ono Akemi、Ichihara Yuko、Shono Miki、Mori Tatsuo、Urushihara Maki、Nakagawa Ryuji、Hayabuchi Yasunobu、Kagami Shoji
    • Journal Title

      The Journal of Medical Investigation

      Volume: 67 Issue: 3.4 Pages: 246-249

    • DOI

      10.2152/jmi.67.246

    • NAID

      130007936868

    • ISSN
      1343-1420, 1349-6867
    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Successful treatment by stent implantation for systemic-to-pulmonary shunt obstruction due to a Staphylococcus aureus abscess: a case report2020

    • Author(s)
      Homma Yukako、Hayabuchi Yasunobu
    • Journal Title

      Cardiology in the Young

      Volume: 30 Issue: 10 Pages: 1538-1540

    • DOI

      10.1017/s1047951120002565

    • Related Report
      2020 Research-status Report
    • Peer Reviewed
  • [Journal Article] Molecular diagnosis of an infant with TSC2/PKD1 contiguous gene syndrome2020

    • Author(s)
      Osumi Keita、Suga Kenichi、Ono Akemi、Goji Aya、Mori Tatsuo、Kinoshita Yukiko、Sugano Mikio、Toda Yoshihiro、Urushihara Maki、Nakagawa Ryuji、Hayabuchi Yasunobu、Imoto Issei、Kagami Shoji
    • Journal Title

      Human Genome Variation

      Volume: 7 Issue: 1 Pages: 21-21

    • DOI

      10.1038/s41439-020-0108-0

    • NAID

      120007144491

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Assessment of right ventricular function by isovolumic acceleration of pulmonary and tricuspid annulus in surgically repaired tetralogy of Fallot2020

    • Author(s)
      Ono Akemi、Hayabuchi Yasunobu、Tanaka Manami、Kagami Shoji
    • Journal Title

      The Journal of Medical Investigation

      Volume: 67 Issue: 1.2 Pages: 145-150

    • DOI

      10.2152/jmi.67.145

    • NAID

      130007839830

    • ISSN
      1343-1420, 1349-6867
    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Right ventricular myocardial stiffness and relaxation components by kinematic model-based analysis2020

    • Author(s)
      Hayabuchi Yasunobu
    • Journal Title

      The Journal of Medical Investigation

      Volume: 67 Issue: 1.2 Pages: 11-20

    • DOI

      10.2152/jmi.67.11

    • NAID

      130007839855

    • ISSN
      1343-1420, 1349-6867
    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] 肺循環障害と右心不全の病態と治療 -小児心臓病のパラダイムシフト-2020

    • Author(s)
      早渕康信
    • Journal Title

      四国医学雑誌

      Volume: 76 Pages: 251-258

    • Related Report
      2020 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] A novel index equivalent to the myocardial performance index for right ventricular functional assessment in children and adolescent patients.2019

    • Author(s)
      Hayabuchi Y, Homma Y, Kagami S.
    • Journal Title

      Sci Rep

      Volume: 9 Issue: 1 Pages: 19975-19975

    • DOI

      10.1038/s41598-019-56564-y

    • NAID

      120006783217

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Right ventricular myocardial stiffness and relaxation components by kinematic model-based transtricuspid flow analysis in children and adolescents with pulmonary arterial hypertension.2019

    • Author(s)
      Hayabuchi Y, Homma Y, Kagami S.
    • Journal Title

      Ultrasound Med Biol

      Volume: 45 Issue: 8 Pages: 1999-2009

    • DOI

      10.1016/j.ultrasmedbio.2019.04.021

    • NAID

      120006844040

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Optical coherence tomography for observing development of pulmonary arterial vasa vasorum after bidirry connection in children.ectional cavopulmona2019

    • Author(s)
      Hayabuchi Y, Homma Y, Kagami S.
    • Journal Title

      PLoS One

      Volume: 14 Issue: 4 Pages: e0215146-e0215146

    • DOI

      10.1371/journal.pone.0215146

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access
  • [Journal Article] Development of acquired intrapulmonary venous anastomosis contributing to establishment of Fontan circulation.2019

    • Author(s)
      Hayabuchi Y, Homma Y, Sugano M, Kitaichi T, Kagami S.
    • Journal Title

      Pulm Circ

      Volume: 印刷中 Issue: 1 Pages: 1-3

    • DOI

      10.1177/2045894018814774

    • NAID

      120006772545

    • Related Report
      2019 Research-status Report
    • Peer Reviewed / Open Access
  • [Presentation] 前負荷増加による左室・右室の拡張期圧の変化は心エコー指標で予想可能である2021

    • Author(s)
      本間友佳子 早渕康信
    • Organizer
      第32回日本心エコー図学会学術集会
    • Related Report
      2021 Annual Research Report
  • [Presentation] Fontan症例の肺動脈 vasa vasorum -3次元構築と経時的変化-2021

    • Author(s)
      早渕康信 本間友佳子
    • Organizer
      第6回日本肺高血圧・肺循環学会学術集会
    • Related Report
      2021 Annual Research Report
  • [Presentation] 光干渉断層像(Optical Coherence Tomography:OCT)による画像描出・解像度の向上に関する検討2021

    • Author(s)
      本間友佳子 早渕康信
    • Organizer
      第57回日本小児循環器学会総会・学術集会
    • Related Report
      2021 Annual Research Report
  • [Presentation] Fontan循環症例における肺動脈および体静脈の血管形態に関する光干渉断層像(OCT画像)を用いた経時的観察による検討2021

    • Author(s)
      早渕康信 本間友佳子
    • Organizer
      第57回日本小児循環器学会総会・学術集会
    • Related Report
      2021 Annual Research Report
  • [Presentation] ファロー四徴症術後症例における右室拡張能評価はガイドラインのアルゴリズムを使用できない2021

    • Author(s)
      本間友佳子 早渕康信
    • Organizer
      第57回日本小児循環器学会総会・学術集会
    • Related Report
      2021 Annual Research Report
  • [Presentation] 右室流入血流拡張早期波形(E波形)解析による右室拡張能に関わる因子 (Elastic recoil, Relaxation, Load)の評価2021

    • Author(s)
      早渕康信 本間友佳子
    • Organizer
      第57回日本小児循環器学会総会・学術集会
    • Related Report
      2021 Annual Research Report
  • [Presentation] 11年間に8回のコイル塞栓術を施行した両側肺全区域びまん性多発性肺動静脈瘻の1例2020

    • Author(s)
      本間友佳子 早渕康信 香美祥二
    • Organizer
      第31回日本Pediatric Interventional Cardiology学会学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] 肺高血の進行を来した心房中隔欠損(小欠損孔) を伴う Inflammatory skin and bowel disease, neonatal, 1 (NISBD1) 疑いの一例2020

    • Author(s)
      本間友佳子、早渕康信、杉本真弓、須賀健一、香美祥二、井本逸勢
    • Organizer
      第26回日本小児肺循環研究会
    • Related Report
      2020 Research-status Report
  • [Presentation] ファロー四徴症手術後症例 -診断のポイントと管理-2020

    • Author(s)
      早渕康信
    • Organizer
      第31回日本心エコー図学会学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] ファロー四徴症術後症例における右室拡張能:心エコーによるドプラ解析と心臓カテーテル検査による圧解析の比較検討2020

    • Author(s)
      本間友佳子 早渕康信
    • Organizer
      第31回日本心エコー図学会学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] 右室圧から得られる新しい指標 RVωはTei index (MPI)を反映し、ファロー四徴症術後症例における右心不全を評価できる2020

    • Author(s)
      早渕康信 本間友佳子
    • Organizer
      第56回日本小児循環器学会総会・学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] 肺動脈におけるvasa vasorumの3次元画像構築 ― Fontan candidateにおける経時的変化と臨床的有用性-2020

    • Author(s)
      早渕康信 本間友佳子
    • Organizer
      第56回日本小児循環器学会総会・学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] 光干渉断層像(Optical Coherence Tomography:OCT)による肺動脈・冠動脈病変の3次元画像評価2020

    • Author(s)
      本間友佳子 早渕康信
    • Organizer
      第56回日本小児循環器学会総会・学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] ファロー四徴症術後症例における右室拡張能:心エコーによるドプラ解析と心臓カテーテル検査による圧解析の比較検討2020

    • Author(s)
      本間友佳子 早渕康信
    • Organizer
      第56回日本小児循環器学会総会・学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] Compound heterozygoteのため若年発症した重症不整脈原性右室心筋症の女児例および一家系2020

    • Author(s)
      小野朱美 森一博 早渕康信
    • Organizer
      第56回日本小児循環器学会総会・学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] 左上大静脈左房灌流に対する心房内rerouting後に機能的僧帽弁狭窄を来した一例2020

    • Author(s)
      菅野幹雄 本間友佳子 藤本鋭貴 北市隆 早渕康信 秦広樹
    • Organizer
      第56回日本小児循環器学会総会・学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] Dual Gate Dopplerを用いた肝静脈-下行大動脈血流波形による胎児不整脈評価の有用性と限界:当院での10年間の経験から2020

    • Author(s)
      加地剛 祖川英至 吉田あつ子 米谷直人 早渕康信 苛原稔
    • Organizer
      第56回日本小児循環器学会総会・学術集会
    • Related Report
      2020 Research-status Report
  • [Presentation] 両側肺静脈閉塞があるにもかかわらずFontan循環が成立した無脾症、総肺静脈還流異常、単心室の1例2019

    • Author(s)
      本間友佳子 早渕康信 小野朱美 香美祥二 菅野幹雄 北市隆 北川哲也
    • Organizer
      第25回日本小児肺循環研究会
    • Related Report
      2019 Research-status Report
  • [Presentation] Optical Coherence Tomography: Evaluation of Pulmonary Circulation2019

    • Author(s)
      Yasunobu Hayabuchi
    • Organizer
      15th Korea Japan China Pediatric Heart Forum
    • Related Report
      2019 Research-status Report
    • Invited
  • [Presentation] シンポジウム「生涯病としても先天性心疾患:ファロー四徴症」 ファロー四徴症は何故、生涯病であるのか?2019

    • Author(s)
      早渕康信
    • Organizer
      第30回日本心エコー図学会・学術集会
    • Related Report
      2019 Research-status Report
    • Invited
  • [Presentation] Recent advances in the treatment and echocardiography for cardiac disorder in children with neuromuscular diseases.2019

    • Author(s)
      Yasunobu Hayabuchi
    • Organizer
      KSE-JSE Joint Symposium
    • Related Report
      2019 Research-status Report
    • Invited

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Published: 2019-04-18   Modified: 2023-01-30  

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