Hepatology Research Unit, Ghent University, Ghent, Belgium.
Department of Basic and Applied Medical Sciences, Gut-Liver Immunopharmacology Unit, Ghent University, Ghent, Belgium.
Acta Gastroenterol Belg. 2021 Jan-Mar;84(1):95-99. doi: 10.51821/84.1.200.
Liver disease, cirrhosis and portal hypertension can be complicated by pulmonary vascular disease, which may affect prognosis and influence liver transplantation (LT) candidacy. Pulmonary vascular complications comprise hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). Although these two conditions develop on a same background and share a common trigger, pulmonary responses are distinct and occur at different anatomical sites of the pulmonary circulation. HPS affects 10-30% of patients referred for LT, and is characterized by gas exchange abnormalities due to pulmonary vasodilation and right-to-left shunting. POPH occurs in 5%, and is defined by pulmonary arterial hypertension due to increased pulmonary vascular resistance, which leads to hemodynamic failure. Even though HPS and POPH may have a substantial negative impact on survival, both entities are clinically underrecognized and frequently misdiagnosed. Without intervention, the 5-year survival rate is 23% in HPS and 14% in POPH. Their presence should be actively sought by organized screening in patients presenting with dyspnea and in all patients on the waitlist for LT, also because clinical symptoms are commonly non-specific or even absent. LT may lead to resolution, however, advanced stages of either HPS or POPH may jeopardize safe and successful LT. This implicates the need of proper identification of HPS and POPH cases, as well as the need to be able to successfully 'bridge' patients to LT by medical intervention. A review article on this topic has been published in this journal in 2007 (1). This updated review focuses on recent advances in the diagnosis and management of these 2 liver-induced pulmonary vascular disorders and incorporates results from our recent work.
肝脏疾病、肝硬化和门静脉高压症可并发肺部血管疾病，这可能影响预后并影响肝移植（LT）的候选资格。肺部血管并发症包括肝肺综合征（HPS）和门肺高血压（POPH）。尽管这两种情况在同一背景下发展并具有共同的诱因，但肺部反应是不同的，发生在肺循环的不同解剖部位。HPS 影响 10-30%接受 LT 的患者，其特征是由于肺血管扩张和右向左分流导致气体交换异常。POPH 发生在 5%，其定义为由于肺血管阻力增加导致的肺动脉高压，导致血流动力学衰竭。尽管 HPS 和 POPH 可能对生存有实质性的负面影响，但这两种情况在临床上都未得到充分认识，并且经常被误诊。如果不进行干预，HPS 的 5 年生存率为 23%，POPH 的 5 年生存率为 14%。在出现呼吸困难的患者和所有等待 LT 的患者中，应通过有组织的筛查积极寻找这些情况，因为临床症状通常是非特异性的，甚至不存在。LT 可能会导致其缓解，然而，HPS 或 POPH 的晚期阶段可能会危及 LT 的安全和成功。这意味着需要正确识别 HPS 和 POPH 病例，并且需要能够通过医疗干预成功“桥接”患者进行 LT。该杂志于 2007 年发表了一篇关于该主题的综述文章（1）。本综述重点介绍了这两种与肝脏相关的肺部血管疾病的诊断和管理方面的最新进展，并纳入了我们最近的工作结果。
