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肺動静脈瘻(肺動静脈奇形)

著者: 塩谷隆信 秋田大学名誉教授、介護老人保健施設 ニコニコ苑

監修: 久保惠嗣 信州大学名誉教授・地方独立行政法人 長野県立病院機構理事長

著者校正/監修レビュー済:2020/07/16
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患者向け説明資料
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
塩谷隆信 : 特に申告事項無し[2021年]
監修:久保惠嗣 : 特に申告事項無し[2021年]

改訂のポイント:
  1. 最新の国際ステートメントを掲載し、肺動静脈瘻の病型、肺動静脈瘻の管理アルゴリズムを新しく記載した。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 肺動静脈瘻(肺動静脈奇形)とは、肺動脈と肺静脈が薄い壁を有する血管嚢あるいは迷路状拡張血管で交通する血管異常である。
 
肺動静脈瘻の病型

A:正常血管床 B:単純型(simple type) C:複雑型(complex type)
経カテーテル塞栓術の実施の際に、病型別に治療法が異なるために非常に重要である。

 
  1. 病態生理の大きな特徴は、右心系の血液が毛細血管を介さずに左心系に流入する右左シャントとなり低酸素血症を惹起する点である。
  1. 日本における肺動静脈瘻の疫学に関して、CT検診を用いた検討で10万人あたり38人と報告がされている[1]
  1. 一般的に肺動静脈瘻は男性に比較して女性に多く発症する。
  1. 肺動静脈瘻は、オスラー病(hereditary hemorrhagic telangiectasia、HHT)に合併することが多く、海外では、肺動静脈瘻の約70%はHHTに合併し、HHTの約15~35%に肺動静脈瘻が合併すると報告されている。<図表><図表>
  1. 日本の大規模な疫学調査では、肺動静脈瘻の約25%はHHTに合併し、HHT合併肺動静脈瘻には性差がないが、HHT非合併肺動静脈瘻の約80%は女性である。
問診・診察のポイント  
  1. 症状は、肺動静脈瘻の数や大きさにより異なり、まったくの無症状から、チアノーゼを伴う重症例、心不全や呼吸不全を呈するものまでさまざまである。低酸素血症がある場合には、バチ指となることがある。

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文献 

著者: Masayuki Nakayama, Takeshi Nawa, Tatsuya Chonan, Katsuyuki Endo, Satoshi Morikawa, Masashi Bando, Yasuhiko Wada, Takanobu Shioya, Yukihiko Sugiyama, Shimao Fukai
雑誌名: Intern Med. 2012;51(13):1677-81. Epub 2012 Jul 1.
Abstract/Text BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are rarely encountered in clinical practice. The prevalence of PAVMs associated with hereditary hemorrhagic telangiectasia (HHT) has been estimated based on the rate in the family members of HHT patients, but the prevalence of PAVMs in the general population remains unknown.
METHODS: We retrospectively examined the prevalence and clinical characteristics of PAVMs as detected by a low-dose thoracic CT screening program for lung cancer at the Hitachi Medical Center and the Hitachi General Health Care Center in the northern part of Ibaraki Prefecture, Japan.
RESULTS: From 2001 to 2007, we identified eight patients (seven females and one male) with PAVMs among 21,235 initial screening participants (the mean age of the patients with PAVMs and that of the screening participants was 60.6 years). The prevalence of PAVMs was estimated at 38 per 100,000 individuals [95% confidence interval (CI)=18-76]. The diameter of the PAVMs was a mean of 6.6 mm, and none of the lesions could be detected by chest X-ray. Females older than 60 years tended to have larger PAVMs than younger women did (p=0.06). Two patients (25%) were diagnosed with HHT. One patient had previously undergone surgery for a brain abscess.
CONCLUSION: PAVMs are more prevalent than previously reported, especially among females.

PMID 22790125  Intern Med. 2012;51(13):1677-81. Epub 2012 Jul 1.
著者: M E Faughnan, V A Palda, G Garcia-Tsao, U W Geisthoff, J McDonald, D D Proctor, J Spears, D H Brown, E Buscarini, M S Chesnutt, V Cottin, A Ganguly, J R Gossage, A E Guttmacher, R H Hyland, S J Kennedy, J Korzenik, J J Mager, A P Ozanne, J F Piccirillo, D Picus, H Plauchu, M E M Porteous, R E Pyeritz, D A Ross, C Sabba, K Swanson, P Terry, M C Wallace, C J J Westermann, R I White, L H Young, R Zarrabeitia, HHT Foundation International - Guidelines Working Group
雑誌名: J Med Genet. 2011 Feb;48(2):73-87. doi: 10.1136/jmg.2009.069013. Epub 2009 Jun 23.
Abstract/Text BACKGROUND: HHT is an autosomal dominant disease with an estimated prevalence of at least 1/5000 which can frequently be complicated by the presence of clinically significant arteriovenous malformations in the brain, lung, gastrointestinal tract and liver. HHT is under-diagnosed and families may be unaware of the available screening and treatment, leading to unnecessary stroke and life-threatening hemorrhage in children and adults.
OBJECTIVE: The goal of this international HHT guidelines process was to develop evidence-informed consensus guidelines regarding the diagnosis of HHT and the prevention of HHT-related complications and treatment of symptomatic disease.
METHODS: The overall guidelines process was developed using the AGREE framework, using a systematic search strategy and literature retrieval with incorporation of expert evidence in a structured consensus process where published literature was lacking. The Guidelines Working Group included experts (clinical and genetic) from eleven countries, in all aspects of HHT, guidelines methodologists, health care workers, health care administrators, HHT clinic staff, medical trainees, patient advocacy representatives and patients with HHT. The Working Group determined clinically relevant questions during the pre-conference process. The literature search was conducted using the OVID MEDLINE database, from 1966 to October 2006. The Working Group subsequently convened at the Guidelines Conference to partake in a structured consensus process using the evidence tables generated from the systematic searches.
RESULTS: The outcome of the conference was the generation of 33 recommendations for the diagnosis and management of HHT, with at least 80% agreement amongst the expert panel for 30 of the 33 recommendations.

PMID 19553198  J Med Genet. 2011 Feb;48(2):73-87. doi: 10.1136/jmg.200・・・
著者: R I White, J S Pollak, J A Wirth
雑誌名: J Vasc Interv Radiol. 1996 Nov-Dec;7(6):787-804.
Abstract/Text The recent long-term studies from England, France, and the Netherlands, as well as our own, indicate that transcatheter embolotherapy is definitive treatment for PAVM. More recently, Puskas et al have questioned transcatheter embolotherapy as a primary treatment for patients with PAVM (4,56). Their opinion was based on two recurrences among five patients treated with transcatheter embolotherapy. It is not clear why one of the late recurrences in the series by Puskas et al happened, and the other recurrence could have been dut to early deflation of the balloon. Nevertheless, we believe that the collective experience in the larger series reporting on transcatheter embolotherapy of PAVM supports the use of embolotherapy as a primary modality of treatment. Because many patients have bilateral pulmonary malformations and many pulmonary malformations will grow with time, repeated surgical intervention is not ideal therapy. The recurrence rate of 8% reported by Remy et al using coils, and 2% reported by Pollak et al using balloons and coils supports our contention that transcatheter embolotherapy is durable and should be the initial treatment. Also, recurrences are easily retreated by transcatheter embolotherapy with durable results (54). We favor detachable balloons over coils for occluding PAVMs because immediate cross-sectional occlusion of the segmental artery is obtained in a position that preserves the most normal branches. The necessity for repeated introduction of coils, when using the coil method, contributes to longer procedure times with an increased risk of air introduction and, in our experience, a greater risk of postprocedure pleurisy. At the same time, we appreciate that approximately 70% of PAVMs can be occluded equally well with balloons or coils. We also believe that coils have unique advantages over balloons in specific anatomic situations including oversized arteries (where coils are the only option) and for occlusion of the aneurysm of a PAVM. As with all forms of embolotherapy, the interventionalist is best served by having more than one option of treatment, which for PAVM includes both balloons and coils. In summary, PAVMs are effectively managed by means of transcatheter embolotherapy. This therapy has been demonstrated to be safe and durable. Careful technique with modifications depending on the angioarchitecture of the PAVM is required. Patients with PAVMs require follow-up at 1 month and 1 year. While observations documenting serial growth of small PAVMs are somewhat limited, there is published evidence to support their growth with time (35,36). Because of these reports and our unpublished observations, we believe that patients with treated PAVM need long-term follow-up every 5 years to detect growth of small PAVMs that will ultimately reach a size where they may cause paradoxical embolization and stroke (1).

PMID 8951745  J Vasc Interv Radiol. 1996 Nov-Dec;7(6):787-804.
著者: Claire L Shovlin, Robin Condliffe, James W Donaldson, David G Kiely, Stephen J Wort, British Thoracic Society
雑誌名: Thorax. 2017 Dec;72(12):1154-1163. doi: 10.1136/thoraxjnl-2017-210764.
Abstract/Text Pulmonary arteriovenous malformations (PAVMs) are structurally abnormal vascular communications that provide a continuous right-to-left shunt between pulmonary arteries and veins. Their importance stems from the risks they pose (>1 in 4 patients will have a paradoxical embolic stroke, abscess or myocardial infarction while life-threatening haemorrhage affects 1 in 100 women in pregnancy), opportunities for risk prevention, surprisingly high prevalence and under-appreciation, thus representing a challenging condition for practising healthcare professionals. The driver for the current Clinical Statement was the plethora of new data since previous hereditary haemorrhagic telangiectasia (HHT) guidelines generated in 2006 and a systematic Cochrane Review for PAVM embolisation in 2011. The British Thoracic Society (BTS) identified key areas in which there is now evidence to drive a change in practice. Due to the paucity of data in children, this Statement focused on adults over 16 years. The Statement spans the management of PAVMs already known to be present (interventional and medical), screening and diagnosis (for PAVMs and HHT) and follow-up of patients following a first diagnosis, intervention or negative screen for PAVMs. The Good Practice Points (in bold) were generated for a target audience of general respiratory, medical and specialist clinicians and were approved by the BTS Standards of Care Committee, before formal peer review and public consultation. The Statement spans embolisation treatment, accessory medical management and issues related to the likelihood of underlying HHT.

© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
PMID 29141890  Thorax. 2017 Dec;72(12):1154-1163. doi: 10.1136/thoraxj・・・
著者: Rodrigo Cartin-Ceba, Karen L Swanson, Michael J Krowka
雑誌名: Chest. 2013 Sep;144(3):1033-1044. doi: 10.1378/chest.12-0924.
Abstract/Text Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular structures that most often connect a pulmonary artery to a pulmonary vein, bypassing the normal pulmonary capillary bed and resulting in an intrapulmonary right-to-left shunt. As a consequence, patients with PAVM can have hypoxemia and paradoxical embolization complications, including stroke and brain abscess. PAVMs may be single or multiple, unilateral or bilateral, and simple or complex. Most PAVMs are hereditary and occur in hereditary hemorrhagic telangiectasia, an autosomal dominant vascular disorder, and screening for PAVM is indicated in this subgroup. PAVMs may also be idiopathic, occur as a result of trauma and infection, or be secondary to hepatopulmonary syndrome and bidirectional cavopulmonary shunting. Diagnostic testing involves identifying an intrapulmonary shunt, with the most sensitive test being transthoracic contrast echocardiography. Chest CT scan is useful in characterizing PAVM in patients with positive intrapulmonary shunting. Transcatheter embolotherapy is the treatment of choice for PAVM. Lifelong follow-up is important because recanalization and collateralization may occur after embolization therapy. Surgical resection is rarely necessary and reserved for patients who are not candidates for embolization. Antibiotic prophylaxis for procedures with a risk of bacteremia (eg, dental procedures) is recommended in all patients with PAVM because of the risk of cerebral abscess.

PMID 24008954  Chest. 2013 Sep;144(3):1033-1044. doi: 10.1378/chest.12・・・

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