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 31(6): 352-357 (2015)
doi:10.9794/jspccs.31.352

症例報告Case Report

正常肺組織を犠牲にすることを前提としてコイル塞栓術を施行した両側肺全区域びまん性肺動静脈瘻の1例A Case of Diffuse Pulmonary Arteriovenous Malformationss Successfully Treated by Percutaneous Transcatheter Embolization with Sacrifice of Normal Pulmonary Arteries

徳島大学大学院ヘルスバイオサイエンス研究部小児科学分野Department of Pediatrics, Institute of Health Biosciences, The University of Tokushima ◇ 〒770-8503 徳島県徳島市蔵本町三丁目18番地の15Kuramoto-cho 3-18-15, Tokushima-shi, Tokushima 770-8503, Japan

受付日:2015年5月21日Received: May 21, 2015
受理日:2015年9月4日Accepted: September 4, 2015
発行日:2015年11月1日Published: November 1, 2015
HTMLPDFEPUB3

Endoglin遺伝子(ENG)変異(p. Ala160del, c.479_481 delCTG; ex4)を有した遺伝性出血性毛細血管拡張症に合併した両側肺全区域びまん性肺動静脈瘻の女児例を経験した.9歳で診断され,5年間に5回のカテーテル治療を施行した.コイル塞栓術直後はSpO2 90%以上に上昇するが徐々に80%程度まで繰り返し低下していた.びまん性肺動静脈瘻は,瘻内や流入動脈の塞栓では新たな瘻への流入血管が再度出現・増悪し,瘻への流入血流が増加する.瘻内のみへのコイル塞栓では血流阻害が不十分と考え,以降は正常肺動脈を犠牲にしてコイル塞栓術を施行した.正常肺血管の塞栓は肺血管床を減少させ,肺高血圧を惹起するため最小限にとどめることに留意した.治療後はSpO2 90%以上を維持し,肺高血圧症は認めていない.治療に難渋する肺動静脈瘻では正常肺を犠牲にすることを前提としたカテーテル治療も1つの選択肢と考えられた.今後は長期的な肺高血圧症の発症も含めて,慎重な経過観察が必要である.

We report the case of a 14-year-old girl with diffuse pulmonary arteriovenous malformations (PAVM) associated with mutations of the endoglin (ENG) gene (p. Ala160del, c.479_481 delCTG; ex4). She was diagnosed at 9 years of age and had undergone percutaneous transcatheter embolization (TCE) five times since the diagnosis. However, the effects were limited. Initial oxygen saturation after coil embolization was over 90%, gradually falling to 80% after every treatment. With diffuse PAVM, embolization of the fistulas or feeding vessels still leaves a chance for enlargement of another feeding vessel. Enlarged feeding vessels increase fistula inflow and consequently induce PAVM development and hypoxia. We considered this as the cause of the repeated hypoxemia. Because embolization of the fistulas alone did not seem sufficient to interrupt the blood supply, we decided to perform coil embolizations and sacrifice normal pulmonary artery trees. Occluding normal pulmonary arteries is known to reduce pulmonary capillary beds and can induce pulmonary arterial hypertension. However, to improve hypoxemia, we considered that TCE with sacrifice of normal pulmonary arteries would be necessary. Repeated TCE brought about a good clinical course without pulmonary artery pressure elevation. TCE with sacrifice of normal pulmonary arteries may offer a promising and effective choice of treatment in some cases of PAVM. It is necessary to pay close attention to the development of pulmonary arterial hypertension in long-term follow-up.

Key words: pulmonary arteriovenous malformations; coil embolization; pulmonary arterial hypertension

This page was created on 2015-11-04T09:11:36.272+09:00
This page was last modified on 2015-11-27T17:50:00.813+09:00


このサイトは(株)国際文献社によって運用されています。