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Acute Rupture of Chordae Tendineae of the Mitral Valve in Infants -A Nationwide Survey in Japan Exploring a New Syndrome

Research Project

Project/Area Number 24591594
Research Category

Grant-in-Aid for Scientific Research (C)

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

Principal Investigator

SHIRAISHI Isao  独立行政法人国立循環器病研究センター, 病院, 部長 (80295659)

Co-Investigator(Renkei-kenkyūsha) IKEDA Yoshihiko  国立研究開発法人, 国立循環器病研究センター病理部, 医長 (30393242)
Project Period (FY) 2012-04-01 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2014: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2013: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Fiscal Year 2012: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Keywords臨床医学 / 小児科 / 循環器 / 急性心不全 / 僧帽弁閉鎖不全 / 抗SSA抗体 / 川崎病 / 心内膜炎 / mitral choral rupture
Outline of Final Research Achievements

Ninety-five children with rupture of mitral chordae tendineae were identified in nationwide surveys of Japan diagnosed from 1995 to 2013. Eighty-one (85%) patients were between 4 and 6 months of age. The underlying conditions included Kawasaki disease (10 cases), and maternally derived anti-SSA antibodies (2 cases). Surgery was performed in 80 patients (94 operations), and the final operations included plasty of mitral chordae in 52 cases and mechanical valve replacement in 26 cases. The histopathologic examinations (n=28) revealed inflammatory reactions with predominant mononuclear cell infiltration in 18 cases (64%). Eight patients (8.4%) died and significant neurological complications persisted in 10 cases (11%).
Acute heart failure attributable to rupture of the mitral chordae tendineae in infants should be recognized as a significant cardiovascular disorder that may cause sudden onset of cardiogenic shock and death in infants.

Report

(4 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Research-status Report
  • 2012 Research-status Report
  • Research Products

    (10 results)

All 2014 2013 Other

All Journal Article (6 results) (of which Peer Reviewed: 1 results,  Acknowledgement Compliant: 1 results) Presentation (3 results) Remarks (1 results)

  • [Journal Article] Acute rupture of chordae tendineae of the mitral valve in infants: a nationwide survey in Japan exploring a new syndrome.2014

    • Author(s)
      Shiraishi I, Nishimura K, Sakaguchi H, Abe T, Kitano M, Kurosaki K, Kato H, Nakanishi T, Yamagishi H, Sagawa K, Ikeda Y, Morisaki T, Hoashi T, Kagisaki K, Ichikawa H.
    • Journal Title

      Circulation

      Volume: 130 Issue: 13 Pages: 1053-1061

    • DOI

      10.1161/circulationaha.114.008592

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] 乳児特発性僧帽弁腱索断裂2014

    • Author(s)
      白石 公
    • Journal Title

      小児疾患診療のための病態生理1. 改訂第5版, 小児内科

      Volume: 46 Pages: 259-262

    • Related Report
      2014 Annual Research Report
  • [Journal Article] 僧帽弁閉鎖不全2014

    • Author(s)
      白石 公
    • Journal Title

      小児科診療 2014年増刊号 小児の治療指針

      Volume: 77 Pages: 353-354

    • Related Report
      2014 Annual Research Report
  • [Journal Article] <最近注目されるようになった病気>乳児特発性僧帽弁腱索断裂2013

    • Author(s)
      白石 公
    • Journal Title

      小児内科

      Volume: 45 Pages: 1117-1121

    • Related Report
      2013 Research-status Report
  • [Journal Article] 乳児特発性僧帽弁腱索断裂による急性心不全2013

    • Author(s)
      白石 公、坂口平馬、北野正尚、黒嵜健一、池田義彦、帆足孝也、鍵崎康治、市川 肇
    • Journal Title

      循環器病研究の進歩

      Volume: 34 Pages: 52-57

    • Related Report
      2013 Research-status Report
  • [Journal Article] 特集 クローズアップ 新しい子どもの病気. 循環器疾患-最近注目されるようになった疾患 > 「乳児特発性僧帽弁腱索断裂」2013

    • Author(s)
      白石 公
    • Journal Title

      「小児内科」

      Volume: 45

    • Related Report
      2012 Research-status Report
  • [Presentation] 乳児特発性僧帽弁腱策断裂の腱索病理組織所見の検討-全国調査の結果から2013

    • Author(s)
      白石 公
    • Organizer
      第49回日本小児循環器学会総会・学術集会
    • Place of Presentation
      東京
    • Related Report
      2013 Research-status Report
  • [Presentation] Histopathological Features of Massive Mitral Regurgitation due to Rupture of Chordae Tendineae in Infants: Nationwide Survey in Japan2013

    • Author(s)
      Shiraishi I
    • Organizer
      第77回日本循環器学会
    • Place of Presentation
      横浜
    • Related Report
      2012 Research-status Report
  • [Presentation] Clinical and Histopathologic Features of Acute Rupture of Mitral Chordae Tendinae in Infants

    • Author(s)
      Shiraishi, I.
    • Organizer
      European Society of `cardiology 2013
    • Place of Presentation
      Amsterdam
    • Related Report
      2013 Research-status Report
  • [Remarks] 乳児特発性僧帽弁腱索断裂に関する全国実態調査結果について

    • URL

      http://www.ncvc.go.jp/pr/release/003855.html

    • Related Report
      2012 Research-status Report

URL: 

Published: 2013-05-31   Modified: 2019-07-29  

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