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The study of etiologies and mechanisms in cardiomyopathies

Research Project

Project/Area Number 16590693
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Circulatory organs internal medicine
Research InstitutionKochi University

Principal Investigator

DOI Yoshinori  Kochi University, Medical School, Professor, 医学部, 教授 (90140144)

Co-Investigator(Kenkyū-buntansha) KITAOKA Hiroaki  Kochi University, Medical School, Assistant, 医学部, 助手 (10274375)
MATSUMURA Yoshihisa  Kochi University, Medical School, Assistant, 医学部, 助手 (10274391)
YAMASAKI Naohito  Kochi University, School Hospital, Assistant, 医学部附属病院, 助手 (90284447)
KUBO Toru  Kochi University, School Hospital, Senior resident, 医学部附属病院, 医員 (80325422)
Project Period (FY) 2004 – 2006
Project Status Completed (Fiscal Year 2006)
Budget Amount *help
¥2,900,000 (Direct Cost: ¥2,900,000)
Fiscal Year 2006: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 2005: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 2004: ¥1,000,000 (Direct Cost: ¥1,000,000)
Keywordscardiomyopathy / Kochi cardiomyopathy network / genetic analysis
Research Abstract

(1)Kochi Cardiomyopathy Network
We built the registration system for cardiomyopathy patients at Kochi prefecture in 2004 (9 hospitals) in order to evaluate the clinical significance of these patients in a Japanese community. Patients with diagnosis of cardiomyopathy were enrolled in each institution. We evaluated clinical profiles (age at diagnosis, diagnostic reason, symptoms, echo data, ECG, etc.). About 200 patients with hypertrophic cardiomyopathy and 100 patients with dilated cardiomyopathy were enrolled until today. We are planning to continue enrolling new patients and to take longitudinal clinical data.
(2)Analysis of etiology and mechanisms of cardiomyopathy
Hypertrophic cardiomyopathy (HCM)
(1)HCM is a heterogeneous myocardial disorder and the phenotype is not a static manifestation. The HCM phenotype itself is recognized to be a slowly progressive disorder that manifests remarkable evolution of clinical features throughout life. Few studies, however, have demonstrated longitudin … More al evolution of phenotype in relation to genotype. We examined the clinical course of patients with a founder mutation (V592fs/8) in the cardiac myosin-binding protein C gene from 15 unrelated proband families. We observed the longitudinal evolution of phenotype caused by this mutation and concluded that the patients with this mutation were likely to progress to ‘end-stage' HCM, characterized by LV systolic dysfunction and cavity dilatation, with advancing age. This is the first report demonstrating direct longitudinal evolution of phenotype in relation to genotype (J Am Coll Cardiol 2005:46:1737-1743).
(2)Several clinical and morphologic characteristics of HCM in elderly patients have been reported ; in those studies they showed a higher prevalence of diffuse LVH, and ovoid LV shape, a proximal septal bulge, and mitral annular calcification than young patients. However, the studies were carried out before gene testing became available and so it is possible that left ventricular hypertrophy (LVH) of heterogeneous cause or age-related morphologic changes were included. Therefore, uncertainty remain as to whether the previously reported morphologic characteristics are indeed common in elderly patients with HCM caused by sarcomere gene mutations. We revealed the morphologic features of HCM in elderly patients with mutations in the cardiac myosin-binding protein C gene (Circ J 2006;70:875-879). An abnormal crescent-shaped LV cavity was frequently present in the elderly as in the young when there are cardiac myosin-binding protein C gene mutations. It is possible that this morphologic feature could become useful for determining the etiology of HCM in elderly patients.
(3)Because of lifelong LV remodeling, clinical and morphologic presentations could well be changed in HCM in long-term observation. As there is little long term prognostic information in older HCM patients living in a rural community, we studied to assess the long-term prognosis in the older rural HCM patients. We concluded that heart failure death because of LV remodeling became equally important to sudden death when they were followed for more than 10 years, although HCM patients in a Japanese rural community showed relatively benign clinical course (Circ J 2006;70:1543-1549).
Dilated cardiomyopathy (DCM)
We studied to analyze the changes in the long-term prognosis in Japanese patients with DCM over the past 20 years and to identify the factors that might have influenced survival, because little information exists concerning changes in the prognosis of Japanese patients with DCM. To whom the recent trend in the survival should be informed, in relation to treatment. We concluded that the prognosis of Japanese patients with DCM has significantly improved over the past 20 years. This improvement may be explained partly through the increased ACEI/ARB and 6 blockers and a declining use of antiarrhythmics (Circ J 2006;70:376-383).
We reported other studies on cardiomyopathy as follows. Less

Report

(4 results)
  • 2006 Annual Research Report   Final Research Report Summary
  • 2005 Annual Research Report
  • 2004 Annual Research Report
  • Research Products

    (33 results)

All 2007 2006 2005 2004

All Journal Article (25 results) Book (8 results)

  • [Journal Article] Prevalence, clinical significance and genetic basis of hypertrophic cardiomyopathy with restrictive phenotype2007

    • Author(s)
      Toru Kubo, et al.
    • Journal Title

      Journal of the American College of Cardiology (印刷中)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Prevalence, clinical significance and genetic basis of hypertrophic cardiomyopathy with restrictive phenotype2007

    • Author(s)
      Toru Kubo, et al.
    • Journal Title

      Journal of the American College of Cardiology (in press)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Prevalence, clinical significance and genetic basis of hypertrophic cardiomyopathy with restrictive phenotype2007

    • Author(s)
      Toru Kubo, Juan R.Gimeno, Ajay Bahl, et al.
    • Journal Title

      Journal of the American College of Cardiology (発表予定)(確定)

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Left ventricular remodeling of hypertrophic cardiomyopathy : longitudinal observation in a rural community2006

    • Author(s)
      Hiroaki Kitaoka, et al.
    • Journal Title

      Circulation Journal 70

      Pages: 1543-1549

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Morphological characteristics of hypertrophic cardiomyopathy of the elderly with cardiac myosin-binding protein C gene mutations2006

    • Author(s)
      Takayoshi Hirota, et al.
    • Journal Title

      Circulation Journal 70

      Pages: 875-879

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Long-term prognosis of dilated cardiomyopathy revisited : an improvement in survival over the past 20 years2006

    • Author(s)
      Yoshihisa Matsumura, et al.
    • Journal Title

      Circulation Journal 70

      Pages: 376-383

    • NAID

      110004497221

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Low-dose dobutamine stress echocardiography predicts the improvement of left ventricular systolic function and long-term prognosis in patients with idiopathic dilated cardiomyopathy2006

    • Author(s)
      Yoshihisa Matsumura, et al.
    • Journal Title

      J Med Ultrasonics 33

      Pages: 17-22

    • NAID

      10018115975

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Arrhythmogenic right ventricular cardiomyopathy with pulmonary embolism : a case report2006

    • Author(s)
      Takayoshi Hirota, et al.
    • Journal Title

      JOURNAL of CARDIOLOGY 47

      Pages: 255-260

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] 運動耐容能からみた肥大型心筋症における血中BNP濃度の意義2006

    • Author(s)
      北岡裕章, 他
    • Journal Title

      心臓 38

      Pages: 40-41

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Left ventricular remodeling of hypertrophic cardiomyopathy : longitudinal observation in a rural community2006

    • Author(s)
      Hiroaki Kitaoka, Toru Kubo, Makoto Okawa, et al.
    • Journal Title

      Circulation Journal 70

      Pages: 1543-1549

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Morphological characteristics of hypertrophic cardiomyopathy of the elderly with cardiac myosin-binding protein C gene mutations2006

    • Author(s)
      Takayoshi Hirota, Hiroaki Kitaoka, Toru Kubo, et al.
    • Journal Title

      Circulation Journal 70

      Pages: 875-879

    • NAID

      110004741053

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Long-term prognosis of dilated cardiomyopathy revisited : an improvement in survival over the past 20 years2006

    • Author(s)
      Yoshihisa Matsumura, Jun Takata, Hiroaki Kitaoka, et al.
    • Journal Title

      Circulation Journal 70

      Pages: 376-383

    • NAID

      110004497221

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Arrhythmogenic right ventricular cardiomyopathy with pulmonary embolism : a case report2006

    • Author(s)
      Takayoshi Hirota, Hiroaki Kitaoka, Toru Kubo, et al.
    • Journal Title

      J Cardiol 47

      Pages: 255-260

    • NAID

      10017492241

    • Related Report
      2006 Annual Research Report
  • [Journal Article] 運動耐容能からみた肥大型心筋症における血中BNP濃度の意義2006

    • Author(s)
      北岡裕章, 久保享, 大川真理 他
    • Journal Title

      心臓 38

      Pages: 40-41

    • Related Report
      2006 Annual Research Report
  • [Journal Article] Low-dose dobutamine stress echocardiography predicts the improvement of left ventricular systolic function and long-term prognosis in patients with idiopathic dilated cardiomyopathy2006

    • Author(s)
      Yoshihisa Matsumura, Jun Takata, Hiroaki Kitaoka, et al.
    • Journal Title

      J Med Ultrasonics 33

      Pages: 17-22

    • NAID

      10018115975

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Functional assessment by myocardial performance index (Teiindex) correlates with plasma brain natriuretic peptide concentration in patients with hypertrophic cardiomyopathy2005

    • Author(s)
      Makoto Okawa, et al.
    • Journal Title

      Circulation Journal 69

      Pages: 951-957

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Lifelong left ventricular remodeling of hypertrophic cardiomyopathy caused by a founder frameshift deletion mutation in the cardiac myosin-binding protein C gene among Japanese2005

    • Author(s)
      Toru Kubo, et al.
    • Journal Title

      Journal of the American College of Cardiology 46

      Pages: 1737-1743

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Prevalence and clinical significance of systolic impairment in hypertrophic cardiomyopathy2005

    • Author(s)
      Rajesh Thaman, et al.
    • Journal Title

      Heart 91

      Pages: 920-925

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Hypertrophic cardiomyopathy with progression from apical hypertrophy to asymmetrical septal hypertrophy : a case report2005

    • Author(s)
      Hiroaki Kitaoka, et al.
    • Journal Title

      JOURNAL of CARDIOLOGY 45

      Pages: 155-159

    • NAID

      10015468333

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Functional assessment by myocardial performance index (Tei index) correlates with plasma brain natriuretic peptide concentration in patients with hypertrophic cardiomyopathy2005

    • Author(s)
      Makoto Okawa, et al.
    • Journal Title

      Circulation Journal 69

      Pages: 951-957

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Lifelong left ventricular remodeling of hypertrophic cardiomyopathy caused by a founder frameshift deletion mutation in the cardiac myosin-binding protein C gene among Japanese2005

    • Author(s)
      Toru Kubo, Hiroaki Kitaoka, Makoto Okawa et al.
    • Journal Title

      Journal of the American College of Cardiology 46

      Pages: 1737-1743

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Functional assessment by myocardial performance index (Tei index) correlates with plasma brain natriuretic peptide concentration in patients with hypertrophic cardiomyopathy2005

    • Author(s)
      Makoto Okawa, Hiroaki Kitaoka, Yoshihisa Matsumura, et al.
    • Journal Title

      Circulation Journal 69

      Pages: 951-957

    • NAID

      110002696207

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Hypertrophic cardiomyopathy with progression from apical hypertrophy to asymmetrical septal hypertrophy : a case report2005

    • Author(s)
      Hiroaki Kitaoka, Tomoo Kuwahara, Koji Nishida, et al.
    • Journal Title

      Journal of Cardiology 45

      Pages: 155-159

    • NAID

      10015468333

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Novel mutation in cardiac troponin I in recessive idiopathic dilated cardiomyopathy2004

    • Author(s)
      Ross T.Murphy, et al.
    • Journal Title

      Lancet 363

      Pages: 371-372

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Journal Article] Frequency and clinical expression of cardiac troponin I mutations in 748 consecutive families with hypertrophic cardiomyopathy2004

    • Author(s)
      Jens Mogensen, et al.
    • Journal Title

      Journal of the American College of Cardiology 44

      Pages: 2315-2325

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Book] 肥大型心筋症ハンドブック;肥大型心筋症の予後と死因2007

    • Author(s)
      人見信彦, 北岡裕章, 土居義典
    • Total Pages
      7
    • Publisher
      日本医事新報社
    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Annual Research Report 2006 Final Research Report Summary
  • [Book] 肥大型心筋症ハンドブツク;肥大型心筋症の予後予測因子2007

    • Author(s)
      大川真理, 松村敬久, 土居義典
    • Total Pages
      8
    • Publisher
      日本医事新報社
    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Book] 肥大型心筋症ハンドブック;肥大型心筋症の自然歴 (life-longremodeling)2007

    • Author(s)
      久保亨, 矢部敏和, 土居義典
    • Total Pages
      8
    • Publisher
      日本医事新報社
    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Book] 肥大型心筋症ハンドブック;肥大型心筋症の予後予測因子2007

    • Author(s)
      大川真理, 松村敬久, 土居義典
    • Total Pages
      8
    • Publisher
      日本医事新報社
    • Related Report
      2006 Annual Research Report
  • [Book] 肥大型心筋症ハンドブック;肥大型心筋症の自然歴 (life-long remodeling)2007

    • Author(s)
      久保享, 矢部敏和, 土居義典
    • Total Pages
      8
    • Publisher
      日本医事新報社
    • Related Report
      2006 Annual Research Report
  • [Book] Diagnosis and Management of Hypertrophic Cardiomyopathy2004

    • Author(s)
      Yoshinori Doi, Hiroaki Kitaoka, Nobuhiko Hitomi, et al.
    • Total Pages
      10
    • Publisher
      Blackwell/Futura
    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2006 Final Research Report Summary
  • [Book] Diagnosis and Management of Hypertrophic Cardiomyopathy2004

    • Author(s)
      Yoshinori Doi
    • Total Pages
      10
    • Publisher
      Blackwell Publishing / Futura (Malden, Massachusetts, USA)
    • Related Report
      2004 Annual Research Report
  • [Book] Heart View「不整脈死を防ぐ」;心筋症2004

    • Author(s)
      久保亨
    • Total Pages
      5
    • Publisher
      メジカルビュー社
    • Related Report
      2004 Annual Research Report

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Published: 2004-04-01   Modified: 2016-04-21  

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