Estimation of response to medical therapy in patients with atypical pulmonary arterial hypertension

2022 Fiscal Year Final Research Report

• Project/Area Number: 20K08465
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Review Section: Basic Section 53020:Cardiology-related
• Research Institution: Gunma University
• Principal Investigator: Takama Noriaki 群馬大学, 医学部附属病院, 助教 (80638982)
• Co-Investigator(Kenkyū-buntansha): 倉林 正彦 群馬大学, その他部局等, 名誉教授 (00215047)
• Project Period (FY): 2020-04-01 – 2023-03-31
• Keywords: 肺動脈性肺高血圧症 / 薬物治療

Outline of Final Research Achievements

It was reported that high-dose epoprostenol treatment could improve not only hemodynamic improvements but also long-term prognosis for patients with severe pulmonary arterial hypertension. We estimated the relationships between response to medical therapy and their prognoses in patients with pulmonary arterial hypertension initiating epoprostenol treatment. We concluded that bleeding event in acute period is major poor prognosis factor for patients with severe pulmonary arterial hypertension initiating epoprostenol treatment. After acute period, one-year prognosis in patients with severe pulmonary arterial hypertension initiating epoprostenol treatment can show equal good prognosis compared with patients with oral combination therapy. On the other hand, high-dose epoprostenol therapy shows higher rate of endocrine diseases requiring treatment including thyroid, pituitary and pancreas disease.

Free Research Field

循環器内科学

Academic Significance and Societal Importance of the Research Achievements

今回の研究では、肺動脈性肺高血圧症症例において初期に適切な診断を行い、その症例の持つ重症度を評価することで治療介入後適切に状態ならびに液性因子の値も改善することが判明した。一方、本邦で重症例に対して実施されることがあるエポプロステノール高容量持続点滴治療に関しては、初期に出血事象で予後不良となることも確認できた。また治療介入後の経過を追ったことによりその後、重症例の約1/3の症例にて内分泌疾患(甲状腺、下垂体疾患、膵疾患など)を発症し治療介入が必要なことも判明した。