Vergnon J M, De Bonadona J F, Riffat J, Fournel P, Defour-Decousus M, Tabib A, Boucheron S, Emonot A
Rev Mal Respir. 1986;3(3):145-52.
Pulmonary arterio-venous shunts represent a rare cause of hypoxia in cirrhosis. We present two cases, the first was an alcoholic cirrhotic with anthracosilicosis. This patient rapidly developed a picture of significant hypoxaemia with a raised alveolar-arterial oxygen gradient. The presence of this shunt was confirmed by a scintigraphic analysis. A porto-pulmonary localisation was excluded by a changing angiographic picture. Death supervened after refractory hypoxaemia. Post mortem microangiographic studies confirmed the pulmonary nature of the shunt and its pre-capillary localisation. The second patient was alcoholic, with a compensated cirrhosis and developed severe hypoxaemia which progressed to death in less than two months, when he was being treated effectively with corticosteroids for a diffuse interstitial pulmonary fibrosis confirmed histologically. The pulmonary localisation of the shunt was confirmed by an analysis of the angioscintigraphs. The different techniques for the investigation of intra-pulmonary shunt are discussed, as well as the patho-physiological mechanisms involved. The hormone levels measured (sex hormones, serotonin, prostaglandins, intestinal hormones) remained normal. The therapeutic trials tried out (oestrogen, CPD Choline, indomethacin) were ineffective.
肺动静脉分流是肝硬化患者缺氧的罕见原因。我们报告两例病例,第一例是一名患有煤矽肺的酒精性肝硬化患者。该患者迅速出现严重低氧血症,肺泡-动脉血氧梯度升高。闪烁扫描分析证实了该分流的存在。血管造影图像的变化排除了肝肺综合征的可能性。在难治性低氧血症后患者死亡。尸检微血管造影研究证实了分流的肺部性质及其毛细血管前定位。第二例患者为酒精性,患有代偿期肝硬化,出现严重低氧血症,在不到两个月的时间内进展为死亡,当时他正在接受皮质类固醇治疗以治疗经组织学证实的弥漫性间质性肺纤维化。血管闪烁造影分析证实了分流的肺部定位。本文讨论了肺内分流的不同检查技术以及相关的病理生理机制。所检测的激素水平(性激素、血清素、前列腺素、肠激素)均保持正常。所尝试的治疗试验(雌激素、CPD胆碱、消炎痛)均无效。