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胸腔病例(三三一)肺部動靜脈畸形

摘要


肺動靜脈畸形(pulmonary arteriovenous malformation, PAVM) 乃指肺部動脈及靜脈間異常的連接。在一般族群中不常見,但在出現低血氧、肺部結節或咳血等症狀之患者則需列入鑑別診斷。肺動靜脈畸形可為遺傳性出血性血管擴張症(hereditary hemorrhagic telangiectasia, HHT 或稱Osler-Weber-Rendu syndrome) 的一種表現。本文介紹一位確診(definite) 遺傳性出血性血管擴張症合併肺動靜脈畸形的男性病例,接受血管栓塞術後症狀明顯改善,並針對肺動靜脈畸形以及遺傳性出血性血管擴張症做一簡單介紹。

參考文獻


James R Gossage, Pulmonary arteriovenous malformations: Epidemiology, etiology, and pathology in adults. Uptodate 2016.
Khalil A, Farres MT, Mangiapan G, et al. Pulmonary arteriovenous malformations. Chest 2000;117:1399-403.
James R Gossage, Pulmonary arteriovenous malformations: Clinical features and diagnostic evaluation in adults. Uptodate 2016.
Faughnan ME, Palda VA, Garcia-Tsao G, et al. International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet 2011;48:73-87.
Guttmacher AE, Marchuk DA, White RI Jr. Hereditary hemorrhagic telangiectasia. N Engl J Med 1995; 333:918-24.

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