TY - JOUR T1 - Pulmonary microvascular architecture in hereditary haemorrhagic telangiectasia JF - Thorax JO - Thorax SP - 861 LP - 863 DO - 10.1136/thoraxjnl-2016-209299 VL - 72 IS - 9 AU - Maximilian Ackermann AU - Steven J Mentzer AU - Wilfried Roth AU - Urban Geisthoff AU - Erich Stoelben Y1 - 2017/09/01 UR - http://thorax.bmj.com/content/72/9/861.abstract N2 - A 24-year-old Caucasian man was admitted with a known hereditary haemorrhagic telangiectasia (HHT) and heterozygous mutation of factor V Leiden following episodes of cerebral infarctions in occipital lobes, cerebellum and brainstem. In his case history, the patient underwent several interventional embolisation of arteriovenous (AV) malformations in the middle and lower lobes (figure 1). However, those were not completely successful as the malformations were diffuse. We performed video-assisted thoracoscopic surgery with a resection of the middle lobe and a wedge resection of segment 10.Figure 1 CT scans depict the pulmonary arteriovenous malformations after re-embolisation in the middle lobe.HHT (Osler-Weber-Rendu disease) is an autosomal-dominant disease determined by multiple dilated vessels including mucocutaneous telangiectasias and vascular malformations of visceral organs resulting in recurrent epistaxis, gastrointestinal bleedings, paradoxical emboli, cerebral infarctions and abscesses.1 In about 55% of patients with HHT type 1 (HHT-1) and about 10% of patients with HHT type 2 (HHT-2), large pulmonary arteriovenous malformations (PAVMs) are … ER -