Clarification of the mechanism of pulmonary arteriopathy associated with congenital heart diseases and development of new treatment using lung biopsy speciments
| Project/Area Number |
16K15627
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| Research Category |
Grant-in-Aid for Challenging Exploratory Research
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| Allocation Type | Multi-year Fund |
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| Research Field |
Cardiovascular surgery
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| Research Institution | Tohoku University |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
川本 俊輔 東北大学, 医学系研究科, 非常勤講師 (20400244)
堀井 明 東北大学, 医学系研究科, 教授 (40249983)
遠藤 雅人 東北大学, 医学系研究科, 非常勤講師 (90282128)
齋木 由利子 東北大学, 医学系研究科, 准教授 (80311223)
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| Project Period (FY) |
2016-04-01 – 2019-03-31
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| Project Status |
Completed (Fiscal Year 2018)
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| Budget Amount *help |
¥3,380,000 (Direct Cost: ¥2,600,000、Indirect Cost: ¥780,000)
Fiscal Year 2018: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2017: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
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| Keywords | 先天性心疾患 / 肺動脈性肺高血圧症 / 肺生検 / 肺高血圧症 / 肺血管病変 / 肺静脈狭窄症 / 肺高血圧 / 肺動脈性肺高血圧 / PPAR-γ / アポトーシス |
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| Outline of Final Research Achievements |
We demonstrated the following histopathological findings in the patients with pulmonary arterial hypertension (PAH) associated with congenital heart diseases (CHD) using lung biopsy specimens. Pulmonary artery banding can induce pulmonary vascular reverse remodeling as demonstrated by significant pulmonary artery medial thinning and improvement in intimal lesions. In addition, early progression of pulmonary arteriopathy in patients with trisomy 21 was not proved compared with patients without trisomy 21. Moreover, microthrombotic lesions were detected in 7% of patients with CHD and these lesions would be associated with the progression of PAH.
We could not demonstrate the mechanisms of the PAH progression by the molecular biological methods using laser microdissection method and DNA microarray. We will perform further investigation and clarify novel mechanisms of the progression of PAH with CHD.
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| Academic Significance and Societal Importance of the Research Achievements |
先天性心疾患に伴う肺動脈性肺高血圧症における肺血管病変の組織学的特徴が示されたことにより、各症例に応じより良い治療選択が可能になる可能性が示された。加えて肺血管拡張薬のみならず、呼吸器やその他に対するアプローチの必要性や、これまでにそれほど重要視されていなかった血栓性病変に対する新たなアプローチの可能性が示唆された。今後、免疫組織化学的手法等より肺血管病変進行のさらなるメカニズム解明が進めば、新たな治療標的として新薬開発にもつながることが期待できると考えている。
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Report
(4 results)
Research Products
(3 results)
