Grander W, Eller P, Fuschelberger R, Tilg H
Department of Internal Medicine, Community Hospital Hall, Hall/Tirol, Austria.
Eur J Clin Invest. 2006 Sep;36 Suppl 3:67-70. doi: 10.1111/j.1365-2362.2006.01687.x.
Pulmonary arterial hypertension (PAH) with coexisting portal hypertension has been defined as portopulmonary hypertension (PPHTN). It is often related to liver cirrhosis of various aetiologies and is associated with a high mortality rate. Endothelin-1 (ET) is supposed to play an important role in the pathogenesis of PAH as well as portal hypertension. Therefore, therapy with an ET(A)/ET(B) receptor antagonist might be of use in the treatment of PPHTN. We report the case of a 76-year-old male with liver cirrhosis owing to chronic hepatitis C virus infection and PPHTN who was treated with the dual ET(A)/ET(B) receptor antagonist bosentan. The patient showed remarkable improvement of 6-min walking distance from 300 to 480 m after 2 weeks and to 540 m after 14 weeks, respectively. In addition, a significant decline of N-terminal pro B-type natriuretic peptide fraction (NT-proBNP) from 4928 ng mL(-1) to 640 ng mL(-1) was observed. Bosentan might be a promising new therapeutical option for patients suffering from PPHTN.
肺动脉高压（PAH）合并门静脉高压已被定义为门肺高压（PPHTN）。它常与各种病因的肝硬化相关，且死亡率高。内皮素-1（ET）被认为在PAH以及门静脉高压的发病机制中起重要作用。因此，使用ET（A）/ET（B）受体拮抗剂进行治疗可能对PPHTN的治疗有用。我们报告了一例76岁男性患者，因慢性丙型肝炎病毒感染导致肝硬化并患有PPHTN，接受了ET（A）/ET（B）双重受体拮抗剂波生坦治疗。该患者在2周后6分钟步行距离从300米显著改善至480米，14周后进一步改善至540米。此外，观察到N末端B型利钠肽原（NT-proBNP）从4928 ng/mL显著下降至640 ng/mL。波生坦可能是PPHTN患者一种有前景的新治疗选择。
