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肺动静脉畸形

Pulmonary Arteriovenous Malformation

作者信息

Danyalian Aunie, Sankari Abdulghani, Hernandez Felix

机构信息

Aventura Hospital and Medical Center

Wayne State University

Abstract

Pulmonary arteriovenous malformation (PAVM) is a rare pulmonary condition characterized by structurally abnormal communication between the pulmonary artery and pulmonary vein, leading to a pathological intrapulmonary right-to-left shunt. These malformations are also known as pulmonary arteriovenous fistulae, aneurysms, and pulmonary hemangiomas. Initially described by Churton in 1897, these lesions were later identified in association with hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome. While a significant proportion of individuals with PAVMs may remain asymptomatic, those who go undiagnosed could later experience severe complications, including ischemic stroke, myocardial infarctions, cerebral abscesses, massive hemoptysis, and hemothorax. Despite its relatively low incidence, it is crucial to consider PAVMs in the differential diagnosis for patients with symptoms like epistaxis, hypoxemia, and exertional dyspnea. This activity aims to provide insights into the etiology, epidemiology, clinical presentation, evaluation, and management of patients with PAVMs.

摘要

肺动静脉畸形(PAVM)是一种罕见的肺部疾病,其特征是肺动脉与肺静脉之间存在结构异常的连通,导致病理性的肺内右向左分流。这些畸形也被称为肺动静脉瘘、动脉瘤和肺血管瘤。1897年由Churton首次描述,这些病变后来被发现与遗传性出血性毛细血管扩张症(HHT)有关,HHT也被称为奥斯勒-韦伯-伦杜综合征。虽然相当一部分患有PAVM的人可能没有症状,但那些未被诊断出来的人后来可能会出现严重并发症,包括缺血性中风、心肌梗死、脑脓肿、大量咯血和血胸。尽管其发病率相对较低,但对于有鼻出血、低氧血症和劳力性呼吸困难等症状的患者,在鉴别诊断中考虑PAVM至关重要。本活动旨在深入了解PAVM患者的病因、流行病学、临床表现、评估和管理。

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