1 北海道大学小児科Department of Pediatrics, Hokkaido University Graduate School of Medicine ◇ Hokkaido, Japan
2 東京女子医科大学循環器小児・成人先天性心疾患科Department of Pediatric Cardiology and Adult Congenital Cardiology, Tokyo Women’s Medical University ◇ Tokyo, Japan
2018年2月にニースで開催された6th World Symposium on Pulmonary Hypertension （6th WSPH）の内容が，同年12月に成文化された．肺高血圧（pulmonary hypertension; PH）および肺動脈性肺高血圧症（pulmonary arterial hypertension; PAH）の定義が「安静時の平均肺動脈圧が20 mmHgを超えるもの」と変更されたこと，PAHの分類に「カルシウム拮抗薬に長期反応を示すPAH」が新たに追加されたこと，肺静脈閉塞性疾患pulmonary veno-occlusive disease: PVOD/肺毛細血管腫症pulmonary capillary haemangiomatosis: PCH「PVOD/PCH」が「PAH with overt features of venous/capillaries （PVOD/PCH） involvement」と修正されたことなど，前回の5th WSPH以上にさまざまな面で大幅な変更がなされた．
本稿では，はじめにこのNice Pulmonary Hypertension Classification 2018の概要について私見を交えながら解説する．さらにPAHの遺伝学的背景とその臨床現場における意義，遺伝学的情報を活用する上で留意すべき点，小児PAH診療における問題点などについて述べ，最後に筆者自身の基礎研究について記すこととする．
At the 6th World Symposium on Pulmonary Hypertension (WSPH) held in Nice in 2018, PH classification was updated. A major change in the classification was that PH and pulmonary arterial hypertension (PAH) were defined as mean pulmonary arterial pressure of ＞20 mmHg. Additionally “Calcium channel blocker responder for long period” was added to PAH classification. Furthermore, the subgroup “PAH with overt features of venous/capillaries (PVOD/PCH) involvement” was added to the updated PAH classification. This manuscript provides an outline of the Nice PH Classification 2018 and reviews the relationship between the genetic basis and clinical presentation of PAH and the difficulty of treating childhood PAH. In addition, it describes the author’s own research in this field.
Key words: pulmonary hypertension; pulmonary arterial hypertension; gene mutation; pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis
© 2019 特定非営利活動法人日本小児循環器学会© 2019 Japanese Society of Pediatric Cardiology and Cardiac Surgery
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