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Uncovering the mechanism of LVNC; functional study of in vitro study using with iPSCs from the patient's blood sample

Research Project

Project/Area Number 25860848
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Pediatrics
Research InstitutionUniversity of Toyama

Principal Investigator

Hirono Keiichi  富山大学, 大学病院, 助教 (80456384)

Co-Investigator(Renkei-kenkyūsha) ICHIDA Fukiko  富山大学, 大学院医学薬学研究部(医学), 准教授 (30223100)
KINOSHITA Koshi  富山大学, 大学院医学薬学研究部(医学), 助教 (10585920)
Project Period (FY) 2013-04-01 – 2016-03-31
Project Status Completed (Fiscal Year 2015)
Budget Amount *help
¥4,030,000 (Direct Cost: ¥3,100,000、Indirect Cost: ¥930,000)
Fiscal Year 2014: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2013: ¥2,860,000 (Direct Cost: ¥2,200,000、Indirect Cost: ¥660,000)
Keywordscardiomyopathy / 心筋症 / 遺伝子解析 / 心筋緻密化障害
Outline of Final Research Achievements

The aim of this study is to clarify the difference between infantile and juvenile cases and identify the risk factor of poor prognosis of LVNC in the largest series to date.Based on nationwide surveys of LVNC in Japanese children, we compared the clinical features and anatomical properties of infantile cases of LVNC with that of juvenile cases. Although most patients in the infantile group had clinical signs of heart failure at initial presentation, the majority of juvenile cases were asymptomatic and identified when screened for cardiac abnormalities. Neither noncompaction score nor N/C score was significantly different between groups. LVEF at initial presentation was significantly lower in the infantile group than in the juvenile group. Although survival analysis showed poor prognosis in the infantile group.LVNC in both infantile and juvenile groups showed poor prognosis when correlated with depressed LVEF at initial presentation.

Report

(4 results)
  • 2015 Annual Research Report   Final Research Report ( PDF )
  • 2014 Research-status Report
  • 2013 Research-status Report
  • Research Products

    (15 results)

All 2015 2014

All Journal Article (4 results) (of which Peer Reviewed: 4 results,  Acknowledgement Compliant: 3 results,  Open Access: 2 results) Presentation (9 results) (of which Int'l Joint Research: 1 results) Book (2 results)

  • [Journal Article] A Novel MYH7 Gene Mutation in a Fetus With Left Ventricular Noncompaction.2015

    • Author(s)
      Nomura Y, Momoi N, Hirono K, Hata Y, Takasaki A, Nishida N, Ichida F.
    • Journal Title

      Can J Cardiol.

      Volume: 31

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] A novel ACTC1 gene mutation in a young boy with left ventricular noncompaction and arrhythmias.2015

    • Author(s)
      Yoshida Y, Keiichi Hirono, Kae Nakamura, Tsugutoshi Suzuki, Yukiko Hata, Naoki Nishida
    • Journal Title

      Heart Rhythm Case Report

      Volume: 1 Issue: 1 Pages: 0-0

    • DOI

      10.1016/j.hrcr.2015.11.008

    • Related Report
      2015 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] A novel MYH7 gene mutation in a fetus with left ventricular noncompaction.2015

    • Author(s)
      Nomura Y, Momoi N, Hirono K, Hata Y, Takasaki A, Nishida N, Ichida F.
    • Journal Title

      Can J Cardiol.

      Volume: 1 Issue: 1 Pages: 1-3

    • DOI

      10.1016/j.cjca.2014.11.012

    • Related Report
      2014 Research-status Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Familial Ebstein's anomaly, left ventricular noncompaction, and ventricular septal defect associated with an MYH7 mutation.2014

    • Author(s)
      Hirono K, Hata Y, Ibuki K, Yoshimura N.
    • Journal Title

      J Thorac Cardiovasc Surg

      Volume: 148 Issue: 5 Pages: 223-226

    • DOI

      10.1016/j.jtcvs.2014.08.049

    • Related Report
      2014 Research-status Report
    • Peer Reviewed
  • [Presentation] Clinical Features and Prognosis of Patients with Left Ventricular Noncompaction Cardiomyopathy: A Comparison between Infantile and Juvenile Types.2015

    • Author(s)
      Asami Takasaki, Sayaka Ozawa, Nariaki Miyao, Hideyuki Nakaoka, Keijiro Ibuki, Keiichi Hirono, Naoki Yoshimura, Fukiko Ichida
    • Organizer
      The 88th Scientific sessions of AHA 2015
    • Place of Presentation
      米国フロリダ
    • Year and Date
      2015-11-09
    • Related Report
      2015 Annual Research Report
    • Int'l Joint Research
  • [Presentation] TAZ遺伝子変異を伴う左室心筋緻密化障害の臨床遺伝学的検討2015

    • Author(s)
      廣野恵一、畑由紀子、桃井伸緒、鮎沢衛、阿部百合子、辻井信之、林環、坂口平馬、阿部忠朗、宮尾成明、高崎麻美、仲岡英幸、伊吹圭二郎、小澤綾佳、西田尚樹、市田蕗子
    • Organizer
      第24回小児心筋疾患学会
    • Place of Presentation
      大阪
    • Year and Date
      2015-10-24
    • Related Report
      2015 Annual Research Report
  • [Presentation] 胎児期・新生児期発症の左室心筋緻密化障害における臨床遺伝学的検討2015

    • Author(s)
      廣野 恵一, 畑 由紀子, 宮尾 成明, 仲岡 英幸,高崎 麻美, 伊吹 圭二郎, 小澤 綾佳, 西田 尚樹, 市田 蕗子
    • Organizer
      第51回日本小児循環器学会総会・学術集会
    • Place of Presentation
      東京
    • Year and Date
      2015-07-16
    • Related Report
      2015 Annual Research Report
  • [Presentation] TAZ遺伝子変異を伴う左室心筋緻密化障害における臨床遺伝学的検討2015

    • Author(s)
      廣野恵一、畑由紀子、宮尾成明、高崎麻美、仲岡英幸、伊吹圭二郎、小澤綾佳、西田尚樹、市田蕗子
    • Organizer
      第1回日本心筋症研究会
    • Place of Presentation
      東京
    • Year and Date
      2015-07-04
    • Related Report
      2015 Annual Research Report
  • [Presentation] 若年性左室心筋緻密化障害におけるサルコメア遺伝子異常とその臨床的意義2015

    • Author(s)
      高崎麻美、廣野恵一、畑由紀子、仲岡英幸、伊吹圭二郎、小澤綾佳、西田尚樹、芳村直樹、関根道和、市田蕗子
    • Organizer
      第1回日本心筋症研究会
    • Place of Presentation
      東京
    • Year and Date
      2015-07-04
    • Related Report
      2015 Annual Research Report
  • [Presentation] Sarcomere gene mutations in the patients with left ventricular noncompaction.2015

    • Author(s)
      Keiichi Hirono, Asami Takasaki, Yukiko Hata, Hideyuki Nakaoka, Keijiro Ibuki, Sayaka Ozawa, Michikazu Sekine, Naoki Yoshimura, Naoki Nishida, and Fukiko Ichida
    • Organizer
      第79回日本循環器学会
    • Place of Presentation
      大阪
    • Year and Date
      2015-04-25
    • Related Report
      2015 Annual Research Report
  • [Presentation] Targeted Next-generation Sequencing for Postmortem Molecular Analysis of Sudden Cardiac Death.2015

    • Author(s)
      Yukiko Hata, Koshi Kinoshita, Fukiko Ichida, Keiich Hirono, Hisashi Mori, Naoki Nishida
    • Organizer
      第79回日本循環器学会
    • Place of Presentation
      大阪
    • Year and Date
      2015-04-25
    • Related Report
      2015 Annual Research Report
  • [Presentation] MYH7遺伝子変異を認めた左室心筋緻密化障害および心室中隔欠損を伴うEbstein奇形の姉弟例2014

    • Author(s)
      廣野恵一
    • Organizer
      第50回日本小児循環器学会
    • Place of Presentation
      岡山
    • Year and Date
      2014-07-04
    • Related Report
      2014 Research-status Report
  • [Presentation] 胎児期に心不全を契機に発見されたMYH7遺伝子変異を有する左室心筋緻密化障害の一例2014

    • Author(s)
      廣野恵一
    • Organizer
      第50回日本小児循環器学会
    • Place of Presentation
      岡山
    • Year and Date
      2014-07-04
    • Related Report
      2014 Research-status Report
  • [Book] 肥大型心筋症類縁疾患2015

    • Author(s)
      廣野恵一、市田蕗子
    • Total Pages
      4
    • Publisher
      医学書院
    • Related Report
      2015 Annual Research Report
  • [Book] 心筋緻密化障害2015

    • Author(s)
      廣野恵一、市田蕗子
    • Total Pages
      4
    • Publisher
      南江堂
    • Related Report
      2015 Annual Research Report

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Published: 2014-07-25   Modified: 2019-07-29  

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