日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2187-2988 Print ISSN: 0911-1794
特定非営利活動法人日本小児循環器学会 Japanese Society of Pediatric Cardiology and Cardiac Surgery
〒162-0801東京都新宿区山吹町358-5アカデミーセンター Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Pediatric Cardiology and Cardiac Surgery 33(4): 297-311 (2017)
doi:10.9794/jspccs.33.297

ReviewReview

小児肺動脈性肺高血圧症:治療の最前線Advanced Therapies for the Pharmacological Treatment of Pediatric Pulmonary Arterial Hypertension

東邦大学医療センター大森病院小児科Department of Pediatrics, Toho University Omori Medical Center ◇ Tokyo, Japan

発行日:2017年7月1日Published: July 1, 2017
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小児期発症の肺動脈性肺高血圧症は,特発性および遺伝性肺動脈性肺高血圧症や先天性心疾患による肺高血圧が多く,成人期発症の肺高血圧症とは異なる.また,小児の肺高血圧症では,臨床症状,治療反応性,予後因子などにおいて,成人といくつかの相違点や類似点が存在することがわかってきている.しかし,治療に関しては,前向き研究やランダム化比較試験が少ないため,未だ承認されている薬剤が少なく,成人の肺高血圧治療戦略を参考に,現在も模索されている状況である.その使用経験の報告からは小児患者においても成人と同等の効果が得られることがわかってきたが,小児への治療戦略は未だ確立されていない.成人における治療をそのまま小児患者に置き換えることは難しいため,近年小児の治療戦略に関して新しい提言が報告されはじめてきた.本稿では,小児期発症の特発性,遺伝性肺動脈性肺高血圧症および先天性心疾患に伴う肺高血圧症における未承認薬を含む薬物治療の有効性や安全性,治療戦略に関する新たな知見をまとめて紹介する.

Pulmonary arterial hypertension (PAH) is a crucial determinant of morbidity and mortality in children and adults. It can present at any age from infancy to adulthood. The clinical features of pediatric PAH, however, differ from those of adults, and in children, there is a predominance of idiopathic PAH (IPAH) or that associated with congenital heart disease (CHD). Without appropriate treatment, the median survival rate after diagnosis of IPAH in children is considerably worse than that in adults. Additionally, though PAH related to CHD in most children may resolve after surgical correction, some children may develop an irreversible pulmonary vascular disease. Recent studies of pediatric PAH have highlighted the unique aspects of pathogenesis and challenging treatments in IPAH or PAH associated with CHD. Treatment with new selective pulmonary vasodilators offers hemodynamic and functional improvement in pediatric populations. Survival within the first 5 years following diagnosis has been reported to be between 62% and 90%, and the survival rate has been similar between IPAH and PAH associated with CHD. However, there is currently limited data from randomized controlled trials in children with PAH for evaluating the safety and efficacy of vasodilator therapies, which are approved for adult patients. Therefore, further studies are required for development of specific strategies for treating children with PAH. This review provides a brief overview of recent information regarding current approaches to PAH in children.

Key words: treatment strategy; Eisenmenger syndrome; upfront combination therapy; sequential combination therapy; congenital heart disease

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This page was last modified on 2017-08-14T17:10:46.589+09:00


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