| Project/Area Number |
16590693
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Circulatory organs internal medicine
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| Research Institution | Kochi University |
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Principal Investigator |
DOI Yoshinori Kochi University, Medical School, Professor, 医学部, 教授 (90140144)
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| 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)
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| Project Period (FY) |
2004 – 2006
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| Project Status |
Completed (Fiscal Year 2006)
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| 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)
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| Keywords | cardiomyopathy / Kochi cardiomyopathy network / genetic analysis |
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| 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
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