medicina52巻11号

2015年10月発行

連載 いま知りたい 肺高血圧症・1【新連載】

肺高血圧症の成因・病態

著者： 木岡秀隆¹ 瀧原圭子¹²

所属機関： ¹大阪大学大学院医学系研究科循環器内科学 ²大阪大学保健センター

ページ範囲：P.1994 - P.1998

　肺高血圧症（pulmonary hypertension：PH）はさまざまな病態に関連して肺動脈圧の上昇をきたす疾患の総称であり，右心カテーテル検査により評価される安静時平均肺動脈圧25 mmHg以上と定義される^1）．最新の肺高血圧症分類を表1に示すが^2），病因・病態に基づき5つに分類されている．本稿では，第1群肺高血圧症，すなわち肺動脈性肺高血圧症（pulmonary arterial hypertension：PAH）を中心にその成因と病態について，最新の知見を含め解説する．
　かつてPAHは有効な治療法も少なく，進行すれば右心不全^3）をきたすきわめて予後不良の疾患であった．しかし2000年に責任遺伝子が同定されて以降，急速にPAHの発症および進展の分子メカニズムの解明が進み，同時に有効な治療法が相次いで開発されることとなった．循環器内科学において，最も急速に治療が進歩した領域の1つであり，理解をUpdateしておく必要がある．

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