クリッペル・トレナウナイ症候群 慢性血栓塞栓性肺高血圧 バルーン肺血管整形手術で治療
Michael Westhoff1,2, Thorsten Hardebusch1, Andreas Breithecker3
1Pneumology, DGD Lungenklinik Hemer, Hemer, Germany.
Pneumologie (Stuttgart, Germany)
|August 29, 2025
まとめ
クリッペル- トレナウナイ症候群における血管性病変は慢性肺栓塞性高血圧 (CTEPH) を引き起こします. バルーン肺血管形成手術 (BPA) は,CTEPH患者の症状と右心機能を有意に改善しました.
科学分野:
- 心臓病科
- 肺科
- 血管医学
背景:
- クリッペル- トレナウナイ症候群 (KTS) の特徴である血管病変は,血栓塞栓的事件を引き起こす可能性があります.
- 慢性肺栓塞性高血圧 (CTEPH) は,進行性呼吸不全と右心筋緊張を引き起こす可能性のある合併症です.
研究 の 目的:
- CTEPHでKTSの症例を報告する
- 診断と治療の課題を強調する
- バルーン肺血管造形 (BPA) を含む治療法について話し合う.
主な方法:
- 診断にはCTスキャン,エコーカルディオグラフィー,右心経キャセテリゼーション,肺血管撮影が含まれていました.
- 治療には抗凝固薬,リオシグアトの治療,BPAの5回の投与が含まれていました.
- BPAは肺の10箇所をカバーしました
主要な成果:
- 肺動脈圧の上昇 (平均PAP 43 mmHg) と肺血管抵抗 (PVR 7. 5 WU) によって確認されたCTEPHによる呼吸不全を示した.
- BPA投与後,呼吸不全,右心機能,肺 perfusion の有意な改善が観察されました.
- 治療後のPVRは5WUに減少した.
結論:
- KTS患者は血栓塞栓症とCTEPHの発症のリスクがあります.
- KTSにおけるCTEPHの治療方法は,肺内関節切除術 (PEA),医療療法,介入性BPAです.
- PEAが実現できない場合,BPAはKTS患者におけるCTEPHの有効な治療法です.
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