Liver Unit, Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham B15 2TH, UK.
Best Pract Res Clin Gastroenterol. 2013 Aug;27(4):497-512. doi: 10.1016/j.bpg.2013.06.020.
Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are two fascinating and incompletely understood pulmonary vascular conditions seen in the setting of cirrhotic patients. Of the two HPS is more common and is primarily caused by pulmonary vasodilatation resulting in hypoxaemia and hyperdynamic circulation. PoPH is less common and conversely, pulmonary vasoconstriction and vascular remodelling occurs resulting in increased pulmonary vascular resistance. However, both conditions can co-exist and it is usually PoPH which develops in a patient with pre-existing HPS. Although these two pulmonary conditions are not common complications of chronic liver diseases, the treatment options are mainly limited to liver transplantation. Cirrhotic cardiomyopathy is closely related to haemodynamic changes in portal hypertension. The key features are normal cardiac pressures at rest, with reduced ability to compensate for physiological or iatrogenic stresses such as drug therapy or TIPSS. There is no effective therapy and outcomes after liver transplantation are variable.
肝肺综合征（HPS）和门肺高血压（PoPH）是两种在肝硬化患者中出现的引人入胜但尚未完全了解的肺血管疾病。在这两种情况下，HPS 更为常见，主要是由肺血管扩张导致低氧血症和高动力循环引起的。PoPH 则较为少见，相反，会发生肺血管收缩和血管重塑，导致肺血管阻力增加。然而，这两种情况可以同时存在，通常是在患有先前存在的 HPS 的患者中发生 PoPH。尽管这两种肺部疾病并不常见的慢性肝脏疾病的并发症，但治疗选择主要限于肝移植。肝硬化性心肌病与门静脉高压的血流动力学变化密切相关。其主要特征是静息时心脏压力正常，但对药物治疗或 TIPSS 等生理或医源性压力的代偿能力降低。目前尚无有效的治疗方法，肝移植后的结果也各不相同。
