Assessment of morphology/function of cardiac and skeletal muscle in lung disease-associated pulmonary hypertension

2020 Fiscal Year Final Research Report

• Project/Area Number: 18K08129
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 53030:Respiratory medicine-related
• Research Institution: Hokkaido University
• Principal Investigator: Tsujino Ichizo 北海道大学, 医学研究院, 特任教授 (00344507)
• Co-Investigator(Kenkyū-buntansha): 今野 哲 北海道大学, 医学研究院, 教授 (20399835) ; 大平 洋 北海道大学, 大学病院, 助教 (20528301) ; 真鍋 徳子 自治医科大学, 医学部, 教授 (70463742)
• Project Period (FY): 2018-04-01 – 2021-03-31
• Keywords: 肺高血圧症 / 右心不全 / 間質性肺疾患 / 慢性閉塞性肺疾患

Outline of Final Research Achievements

In lung disease-associated pulmonary hypertension (Lung-PH), right ventricular (RV) contractility was increased whereas RV-pulmonary arterial coupling was impaired. Alternatively, RV stiffness was increased, potentially causing RV diastolic dysfunction in these patients.
The most common cause of death of Lung-PH patients was PH-related death (56%), followed by PH death (23%) and PH-unrelated death (21%). These results were different from those for pulmonary arterial hypertension (PAH) where PH itself was the most common cause of death (61%), and for group 4 PH where malignant disease at least partly contributed to the death by 60%. These findings were useful to further improve the management and outcome of patients with each PH type.
Lastly, in the pathological evaluations of the autopsied lungs of interstitial lung disease (ILD), arteriopathy at the site of active fibrosis was more prominent in those with PH than those without it.

Free Research Field

肺循環疾患

Academic Significance and Societal Importance of the Research Achievements

肺高血圧症の5病型の一つである「肺疾患に伴う肺高血圧症」は、他の病型の肺高血圧症と比較して、診断後の余命が短く、疾患の正しい理解や治療法の改善が求められていた。今回の研究では、①肺疾患合併肺高血圧症の発症には肺の線維化が進行中の場所における肺動脈の病変の有無が重要であること、②肺動脈圧上昇の結果右心室の機能異常が起こっていること（右室・肺動脈カップリング障害と右室の硬化）、さらに③死因は肺高血圧症そのものよりも肺疾患のことの方が多いことを明らかにした。これらは「肺疾患に伴う肺高血圧症」に対する治療方法と診断後の患者さんを今後どのようにして改善していくかを考える上で有用な情報である。