Hepatopulmonary syndrome (HPS) is a severe complication of advanced liver disease associated with an extremely poor prognosis. HPS is diagnosed in 4-47% of patients with cirrhosis and in 15-20% of candidates for liver transplantation. In addition, severe hypoxia is associated with a high risk of complications of liver transplantation (a 30% chance during the first 90 days) and increases the gap between transplantation and improving arterial oxygenation. The pathogenesis of HPS is not fully understood, and no effective pharmacological treatment has been developed yet. Currently, the treatment of choice for HPS is orthotopic liver transplantation. Non-specific clinical criteria and the lack of standardized diagnostic criteria for determining HPS can lead to diagnostic errors. Portopulmonary hypertension and hereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu syndrome, are pulmonary complications of liver disease which should be differentially diagnosed from HPS.

中文翻译：

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肝肺综合征（HPS）的鉴别诊断：门肺高压（PPH）和遗传性出血性毛细血管扩张（HHT）。

肝肺综合征（HPS）是晚期肝病的严重并发症，预后极差。HPS在肝硬化患者中诊断为4-47％，在肝移植患者中诊断为15-20％。另外，严重的缺氧与肝移植并发症的高风险相关（前90天的机会为30％），并增加了移植与改善动脉氧合之间的距离。HPS的发病机理尚未完全了解，并且尚未开发出有效的药物治疗方法。目前，HPS选择的治疗方法是原位肝移植。非特异性临床标准和缺乏确定HPS的标准化诊断标准会导致诊断错误。肺动脉高压和遗传性出血性毛细血管扩张，