Verstraeten Maïté, Lefere Sander, Raevens Sarah
Department of Gastroenterology and Hepatology, Ghent University Hospital, Ghent, Belgium.
Hepatology Research Unit, Department of Internal Medicine and Pediatrics, Liver Research Center, Ghent University, Ghent, Belgium.
Acta Clin Belg. 2024 Oct;79(5):384-391. doi: 10.1080/17843286.2025.2456697. Epub 2025 Jan 28.
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH) are two distinct pulmonary vascular complications seen in patients with liver disease and/or portal hypertension. HPS is characterized by disturbed gas exchange and hypoxemia because of intrapulmonary vascular dilatations. POPH is defined by pulmonary arterial hypertension, which might lead to right heart failure. HPS affects up to 30% of patients with end-stage liver disease requiring liver transplantation. POPH is rarer and affects 1-5% of this patient population. If not recognized and left untreated, these disorders result in significant mortality. This review provides an update on HPS and POPH and discusses their clinical characteristics, screening and diagnostic modalities, and management, including the place of liver transplantation.
肝肺综合征（HPS）和门脉性肺动脉高压（POPH）是肝病和/或门脉高压患者中出现的两种不同的肺血管并发症。HPS的特征是由于肺内血管扩张导致气体交换紊乱和低氧血症。POPH由肺动脉高压定义，这可能导致右心衰竭。HPS影响高达30%需要肝移植的终末期肝病患者。POPH较为罕见，影响该患者群体的1%-5%。如果未被识别且未得到治疗，这些疾病会导致显著的死亡率。本综述提供了关于HPS和POPH的最新信息，并讨论了它们的临床特征、筛查和诊断方法以及管理，包括肝移植的地位。
