Stähler G, von Hunnius P
Medical Clinic I, Klinik Löwenstein, Germany.
Eur J Clin Invest. 2006 Sep;36 Suppl 3:62-6. doi: 10.1111/j.1365-2362.2006.01693.x.
Pulmonary arterial hypertension (PAH) is found in 2-20% of cirrhosis patients who have portal hypertension (portopulmonary hypertension, PPHT). Endothelin (ET), a potent vasoconstrictor, is likely to play a role in the pathogenesis of portal hypertension. We describe the long-term successful use of the dual ET(A)/ET(B) receptor antagonist bosentan in a 43-year-old male with alcohol-related cirrhosis (Child-Pugh A), right ventricular enlargement and dysfunction, respectively, and moderate PAH. Elevated pulmonary arterial pressure was substantially reduced and exercise capacity increased. Improvement was maintained over 2 years, and bosentan treatment continues in this patient. Our report is in line with a series of current reports in PPHT that support the use of bosentan in this subset of PAH patients.
在患有门静脉高压症（门肺高压，PPHT）的肝硬化患者中，肺动脉高压（PAH）的发生率为2%至20%。内皮素（ET）是一种强效血管收缩剂，可能在门静脉高压的发病机制中起作用。我们描述了43岁男性酒精性肝硬化（Child-Pugh A级）患者长期成功使用双重ET(A)/ET(B)受体拮抗剂波生坦的情况，该患者分别出现右心室扩大和功能障碍，以及中度PAH。肺动脉压力显著降低，运动能力增强。改善情况持续了2年，该患者仍在继续接受波生坦治疗。我们的报告与目前一系列关于PPHT的报告一致，这些报告支持在这部分PAH患者中使用波生坦。
