Zaka Andrew Z, Mangoura Safwat A, Ahmed Marwa A
Department of Medical Pharmacology, Faculty of Medicine, Assiut University, Assiut, 71515, Egypt.
Department of Medical Pharmacology, Faculty of Medicine, Assiut University, Assiut, 71515, Egypt; Department of Pharmacology and Toxicology, Faculty of Pharmacy, Badr University in Cairo (BUC), Badr, Cairo, 11829, Egypt.
Respir Med. 2025 Jan;236:107911. doi: 10.1016/j.rmed.2024.107911. Epub 2024 Dec 9.
Hepatopulmonary syndrome (HPS) is a serious pulmonary vascular complication that causes arterial hypoxemia in the setting of liver disease. HPS has a progressive course and is associated with a two-fold increased risk of mortality relative to cirrhotic patients without HPS. It primarily affects patients with portal hypertension. The key pathological features of HPS include intrapulmonary angiogenesis and vascular dilations (IPVDs). The prevalence of HPS varies widely due to inconsistent diagnostic criteria and a lack of standardized protocols. Despite advances in understanding its pathophysiology, no effective curative treatments for HPS exist. Liver transplantation remains the only definitive treatment, improving survival and altering the disease natural course. This review explores the pathophysiology, clinical features, and therapeutic strategies for HPS, highlighting recent advances in the literature.
肝肺综合征（HPS）是一种严重的肺血管并发症，在肝脏疾病背景下可导致动脉血氧不足。HPS呈进行性病程，与无HPS的肝硬化患者相比，死亡风险增加两倍。它主要影响门静脉高压患者。HPS的关键病理特征包括肺内血管生成和血管扩张（IPVDs）。由于诊断标准不一致和缺乏标准化方案，HPS的患病率差异很大。尽管在理解其病理生理学方面取得了进展，但目前尚无针对HPS的有效治愈性治疗方法。肝移植仍然是唯一的确定性治疗方法，可提高生存率并改变疾病的自然病程。本综述探讨了HPS的病理生理学、临床特征和治疗策略，重点介绍了文献中的最新进展。
