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一例多发性单侧肺动静脉畸形复发:栓塞治疗的疗效

A case of Multiple Unilateral Pulmonary arteriovenous Malformation Relapse: Efficacy of embolization treatment.

作者信息

Masiello Rossella, Iadevaia Carlo, Grella Edoardo, Tranfa Carmelindo, Cerqua Francesco, Rossi Giovanni, Santoro Giuseppe, Amato Bruno, Rocca Aldo, De Dona Roberta, Lavoretano Sabrina, Perrotta Fabio

机构信息

Department of Cardio-Thoracic and Respiratory Sciences - Second University of Naples - Monaldi Hospital, Naples, Italy.

U.O.C. Radiology A.O.R.N dei Colli - Monaldi Hospital, Naples, Italy.

出版信息

Open Med (Wars). 2015 Dec 17;10(1):513-518. doi: 10.1515/med-2015-0087. eCollection 2015.

Abstract

Pulmonary arteriovenous Malformations (PAVMs) are a rare vascular alteration characterized by abnormal communications between the pulmonary arteries and veins resulting in an extracardiac right-to-left (R-L) shunt. The majority of PAVMs are associated with an autosomal dominant vascular disorder also known as Osler-Weber-Rendu Syndrome. PAVMs appearance can be both single and multiple. Clinical manifestations include hypoxemia, dyspnea cyanosis, hemoptysis and cerebrovascular ischemic events or abscesses. We report a case of an 18 year old female with severe respiratory failure caused by a relapse of multiple unilateral pulmonary arterovenous fistula. Symptoms at admission include dyspnea, cyanosis and clubbing. The patient underwent pulmonary angio-TC scan, brain CT and echocardiography. The thoracic angio-CT scan showed the presence of PAVMs of RUL and RLL; a marked increase of right bronchial artery caliber and its branches with an aneurismatic dilatation was also observed. The patient underwent percutaneous transcatheter embolization using Amplatzer Vascular Plug IV; a relevant clinical and functional improvement was subsequently recorded. Embolization is effective in the treatment of relapsing PAVMS.

摘要

肺动静脉畸形(PAVM)是一种罕见的血管改变,其特征是肺动脉和静脉之间存在异常交通,导致心外右向左(R-L)分流。大多数PAVM与一种常染色体显性血管疾病相关,也称为奥斯勒-韦伯-伦杜综合征。PAVM的表现可以是单发的,也可以是多发的。临床表现包括低氧血症、呼吸困难、发绀、咯血以及脑血管缺血事件或脓肿。我们报告一例18岁女性,因多发性单侧肺动静脉瘘复发导致严重呼吸衰竭。入院时症状包括呼吸困难、发绀和杵状指。患者接受了肺部血管增强CT扫描、脑部CT和超声心动图检查。胸部血管增强CT扫描显示右肺上叶和下叶存在PAVM;还观察到右支气管动脉及其分支管径明显增粗并伴有动脉瘤样扩张。患者接受了使用Amplatzer血管封堵器IV型的经皮经导管栓塞治疗;随后记录到了显著的临床和功能改善。栓塞治疗复发性PAVM有效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/12e7/5368875/122872f62010/med-2015-0087f1.jpg

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