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