Tam Nga Lei, He Xiao-Shun
Organ Transplantation Center, First Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510080, China.
Hepatobiliary Pancreat Dis Int. 2007 Oct;6(5):464-9.
Portopulmonary hypertension (PPH) is defined as the development of pulmonary arterial hypertension associated with increased pulmonary vascular resistance complicated by portal hypertension, with or without advanced hepatic disease. In spite of the relatively rare prevalence, the clinical implications of PPH are significant. It has high perioperative morbidity and mortality. This review is an update of current pathogenesis, diagnosis and therapy of PPH.
An English-language literature search was conducted using PubMed (1980-2006) on portopulmonary hypertension.
Echocardiographically identified patients with elevated pulmonary artery systolic pressure (>50 mmHg) receive right heart catheterization. Epoprostenol (prostacyclin), a potent pulmonary and systemic vasodilator with anti-platelet aggregating activity, and bosentan, an endothelin receptor antagonist, have so far proven beneficial to patients with PPH.
After an accurate diagnosis of PPH, treatment should (at a minimum) focus on reduction of mean pulmonary arterial pressure to less than 35 mmHg prior to orthotopic liver transplantation. However, orthotopic liver transplantation currently remains the only therapy to resolve PPH.
门肺高压（PPH）定义为与肺血管阻力增加相关的肺动脉高压的发展，并伴有门静脉高压，无论有无晚期肝病。尽管PPH的患病率相对较低，但其临床意义重大。它具有较高的围手术期发病率和死亡率。本综述是对PPH当前发病机制、诊断和治疗的更新。
使用PubMed（1980 - 2006年）对门肺高压进行了英文文献检索。
经超声心动图确定肺动脉收缩压升高（>50 mmHg）的患者接受右心导管检查。依前列醇（前列环素），一种具有抗血小板聚集活性的强效肺和全身血管扩张剂，以及波生坦，一种内皮素受体拮抗剂，目前已被证明对PPH患者有益。
在准确诊断PPH后，治疗应（至少）专注于在原位肝移植前将平均肺动脉压降至35 mmHg以下。然而，原位肝移植目前仍然是解决PPH的唯一疗法。
