RT杂志文章SR电子T1西地那非治疗门脉性肺动脉高压JF欧洲呼吸杂志JO Eur Respir J FD欧洲呼吸学会SP 563 OP 567 DO 10.1183/09031936.06.00030206 VO 28 IS 3 A1 F。188bet官网地址莱辛伯格A1 R.沃斯温克尔A1 E.史提夫林A1 B.恩克A1 A。Kreckel A1 H. Olschewski A1 F.;格里明格A1 W。Seeger A1 H. a . Ghofrani YR 2006 UL //www.qdcxjkg.com/content/28/3/563.abstract AB portoppulmonary hypertension (POPH)被认为是肺动脉高压(PAH)的一个亚型;然而，现有的多环芳烃疗法尚未对这种情况进行评估。目前作者治疗了14例患者(4名男性，10名女性;平均年龄55岁(39-75岁)，有中度(n = 1)或重度(n = 13)由酒精性肝病(n = 7)、慢性病毒性肝炎(n = 3)、自身免疫性肝炎(n = 3)引起的POPH，以及口服西地那非引起的遗传性出血性毛细血管扩张(n = 1)。8例患者新近开始肺部血管活性治疗，6例患者已开始吸入前列腺素治疗(伊洛前列素，n = 5;Treprostinil, n = 1)。 During treatment with sildenafil, mean±sd 6-min walk distance increased from 312±111 m to 397±99 m after 3 months, and 407±97 m after 12 months. Mean±sd pro-brain natriuretic peptide levels decreased from 582±315 ng·mL-1 to 230±278 ng·mL-1, and to 189±274ng·mL-1 after 3 and 12 months, respectively. Two patients died after 1 and 2 months from liver failure and cardiac failure, respectively. There was a similar response to sildenafil treatment after 3 and 12 months in patients on monotherapy and those on combination therapy. In conclusion, sildenafil might be effective in monotherapy and in combination therapy with inhaled prostanoids in portopulmonary hypertension, leading to significant improvement by 3 months and sustained response over 12 months.