Divisions of Gastroenterology and Hepatology, Department of Internal Medicine, Medical University of Graz, Austria.
Eur J Gastroenterol Hepatol. 2010 Apr;22(4):385-90. doi: 10.1097/MEG.0b013e3283337130.
Portopulmonary hypertension (PoPH) is an underrecognized complication of portal hypertension, related to cirrhosis and noncirrhotic portal hypertension. PoPH has been found in 5-6% of patients with decompensated liver disease and may adversely affect outcome after liver transplantation. The prevalence of PoPH is unrelated to the severity of liver disease but associated with female sex and underlying autoimmune liver disease. Diagnosis of PoPH is based on screening with Doppler echocardiography and confirmation by right-heart catheterization. Treatment options with proven efficacy in idiopathic pulmonary hypertension include endothelin receptor antagonists, prostanoids, and sildenafil. In PoPH, such targeted treatment was found to be safe in small uncontrolled studies but randomized trials demonstrating its benefit are lacking.
肝肺高压（PoPH）是门静脉高压的一种未被充分认识的并发症，与肝硬化和非肝硬化门静脉高压有关。在失代偿性肝病患者中，PoPH 的发现率为 5-6%，可能会对肝移植后的预后产生不利影响。PoPH 的患病率与肝病的严重程度无关，但与女性和潜在自身免疫性肝病有关。PoPH 的诊断基于多普勒超声心动图筛查，并通过右心导管检查确认。在特发性肺动脉高压中，经证实有效的治疗选择包括内皮素受体拮抗剂、前列腺素和西地那非。在 PoPH 中，在小型非对照研究中发现此类靶向治疗是安全的，但缺乏证明其获益的随机试验。
