Department of Pulmonology, St Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, the Netherlands.
Department of Interventional Radiology, St Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, the Netherlands.
Clin Radiol. 2021 May;76(5):394.e1-394.e8. doi: 10.1016/j.crad.2021.01.011. Epub 2021 Feb 27.
To determine whether there are differences between idiopathic and hereditary haemorrhagic telangiectasia (HHT) associated pulmonary arteriovenous malformations (PAVMs) (HHT-PAVM) regarding clinical and radiographic characteristics, and the results of embolotherapy.
A retrospective analysis was undertaken of all adult and adolescent patients who were diagnosed with a PAVM on chest computed tomography (CT) from January 2006 until August 2019.
In total, 41 patients with idiopathic PAVMs and 194 patients with genetically confirmed HHT and PAVMs were included. Idiopathic PAVMs were more frequently observed in female patients, were more solitary, and predominantly located in the lower lobes. The diameter of the feeding artery and type of PAVM (simple versus complex) were similar. Embolotherapy results were comparable between both groups with similar re-embolisation rates.
PAVMs of idiopathic origin are predominantly found in women, more frequently located in the lower lobes, and solitary compared to HHT-PAVMs; however, the outcome of treatment is the same, suggesting that treatment and follow-up should be similar in both groups.
确定特发性和遗传性出血性毛细血管扩张症（HHT）相关肺动静脉畸形（PAVM）（HHT-PAVM）之间在临床和影像学特征以及栓塞治疗结果方面是否存在差异。
回顾性分析了 2006 年 1 月至 2019 年 8 月期间所有因胸部计算机断层扫描（CT）诊断为 PAVM 的成年和青少年患者。
共纳入 41 例特发性 PAVM 患者和 194 例经基因证实的 HHT 合并 PAVM 患者。特发性 PAVM 更常见于女性，多为单发，主要位于下叶。供血动脉的直径和 PAVM 的类型（单纯型与复杂型）相似。两组的栓塞治疗结果相当，再栓塞率也相似。
与 HHT-PAVM 相比，特发性 PAVM 主要发生在女性，更常位于下叶，且多为单发；然而，治疗效果相同，提示两组的治疗和随访应相似。
