Chen Jiaxin, Guo Yangkun, Zhang Xiaoxun, Zhou Dengcheng, Zhou Yongfang, Pan Qiong, Chai Jin, Gao Jinhang
Department of Gastroenterology, Lab of Gastroenterology and Hepatology, West China Hospital, Sichuan University, Chengdu, China.
Department of Gastroenterology, Institute of Digestive Disease of PLA, Cholestatic Liver Diseases Center and Center for Metabolic Associated Fatty Liver Disease, The First Affiliated Hospital (Southwest Hospital), Third Military Medical University (Army Medical University), Chongqing, China.
J Cell Mol Med. 2025 May;29(9):e70585. doi: 10.1111/jcmm.70585.
Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of liver disease and/or portal hypertension. HPS manifests as impaired gas exchange and hypoxemia due to intrapulmonary vascular dilatations and shunts. In response to primary liver disease, the abnormal adaptation of respiratory epithelial cells, pulmonary endothelial cells and immune cells leads to pulmonary microenvironment disequilibrium and HPS. In this review, we explore the pathophysiologic mechanisms of HPS, including vascular dilation, angiogenesis and alveolar dysfunction. The liver is the primary contributor to HPS, and liver transplantation is the only treatment that generally reverses HPS. We then discuss how disruption of hepatic sinusoidal homeostasis may impact the progression of HPS, mainly focusing on hepatocytes, cholangiocytes, LSECs and macrophages. As HPS occurs more commonly in advanced liver cirrhosis, we also discuss that normalisation of liver dysfunction and portal hypertension is crucial for the resolution of HPS. In conclusion, liver-targeted therapies may be effective in treating HPS.
肝肺综合征（HPS）是一种肝脏疾病和/或门静脉高压引起的肺血管并发症。HPS表现为由于肺内血管扩张和分流导致的气体交换受损和低氧血症。针对原发性肝脏疾病，呼吸上皮细胞、肺内皮细胞和免疫细胞的异常适应性改变导致肺微环境失衡和HPS。在这篇综述中，我们探讨了HPS的病理生理机制，包括血管扩张、血管生成和肺泡功能障碍。肝脏是HPS的主要促成因素，肝移植是通常能逆转HPS的唯一治疗方法。然后我们讨论肝血窦稳态的破坏如何影响HPS的进展，主要关注肝细胞、胆管细胞、肝窦内皮细胞和巨噬细胞。由于HPS更常见于晚期肝硬化，我们还讨论了肝功能和门静脉高压的正常化对于HPS的解决至关重要。总之，针对肝脏的治疗方法可能对治疗HPS有效。
