生まれながらの心疾患を有する成人の肺血管疾患

Gerhard-Paul Diller¹, Michael A Gatzoulis

¹Adult Congenital Heart Center, Royal Brompton Hospital, Sydney St, London, SW3 6NP, UK.

Circulation

|February 28, 2007

まとめ

肺動脈高血圧は,先天性心疾患を持つ成人に多く見られ,時にはアイゼンメンガー症候群に繋がります. このレビューでは,この複雑な状態の原因,症状,治療法について説明します.

科学分野:

心臓病学心臓病学
肺内科肺内科肺内科
大人の先天性心疾患

背景:

肺動脈高血圧 (PAH) は,成人の先天性心疾患 (ACHD) を頻繁に合併させる.
ACHDにおけるPAHは,欠陥の特徴と手術歴によって影響を受け,シアノシス (アイゼンメンガー症候群) があるか否かによって現れます.

研究の目的:

ACHDの成人患者におけるPAHの病因,臨床表現,予後,および管理を包括的に検討する.
アイゼンメンガー症候群の多システム合併症について議論します.
ACHDにおけるPAHの現在の管理と新たな治療法を概説する.

主な方法:

大人の先天性心疾患における肺動脈高血圧に関する利用可能なデータの文献レビュー.
エチオロジー,臨床的特徴,予後,治療戦略に関する情報の合成.
アイゼンメンゲル症候群の合併症と治療の進歩についての議論.

主要な成果:

肺動脈高血圧は,大人の先天性心疾患における一般的な併発症である.
アイゼンメンゲル症候群は,重大な合併症を持つ複雑なマルチシステム障害を表しています.
一般的なアプローチから標的型療法まで,さまざまな管理戦略が利用できます.

結論:

ACHDにおけるPAHの効果的な管理には,その多様なプレゼンテーションとアイゼンメンガー症候群の複雑さを理解する必要があります.
疾病を標的とする新興の治療法は,これらの患者の治療結果を改善するための新しい希望を提供します.

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