Division of Pulmonary and Critical Care, Department of Medicine, University of Wisconsin School of Medicine and Public Health, 600 Highland Avenue, Madison, WI 53792, USA.
Liver Transpl. 2012 Jun;18(6):686-95. doi: 10.1002/lt.23407.
Portopulmonary hypertension (PoPH) refers to pulmonary arterial hypertension associated with portal hypertension with or without evidence of an underlying liver disease. Despite the potential for curing PoPH with liver transplantation, the presence of moderate or severe PoPH is associated with increased morbidity and mortality and is, therefore, a contraindication to transplantation. Previous studies have predominantly used intravenous epoprostenol for treatment in order to qualify patients for liver transplantation. In this retrospective case series, we describe the clinical course of 11 patients whom we successfully treated (predominantly with oral sildenafil and subcutaneous treprostinil) in order to qualify them for liver transplantation. The mean pulmonary artery pressure significantly improved from 44 to 32.9 mm Hg, and the pulmonary vascular resistance decreased from 431 to 173 dyn second cm(-5) . There were significant improvements in the cardiac output and the transpulmonary gradient with these therapies as well. All 11 patients subsequently received liver transplants with a 0% mortality rate to date; the duration of follow-up ranged from 7 to 60 months. After transplantation, 7 of the 11 patients (64%) were off all pulmonary vasodilators, and only 2 patients required transiently increased doses of prostacyclins. In conclusion, an aggressive approach to the treatment of PoPH with sildenafil and/or treprostinil and subsequent liver transplantation may be curative for PoPH in some patients.
肝肺高压（PoPH）是指与门静脉高压相关的肺动脉高压，无论是否存在潜在的肝脏疾病。尽管肝移植可能治愈 PoPH，但中重度 PoPH 的存在与更高的发病率和死亡率相关，因此是肝移植的禁忌症。先前的研究主要使用静脉内依前列醇来治疗以使患者有资格进行肝移植。在本回顾性病例系列研究中，我们描述了 11 例患者的临床病程，我们通过使用口服西地那非和皮下曲前列尼尔成功治疗了这些患者，以使他们有资格进行肝移植。平均肺动脉压从 44 毫米汞柱显著改善至 32.9 毫米汞柱，肺血管阻力从 431 降低至 173 达因秒平方厘米(-5) 。这些治疗方法也显著改善了心输出量和跨肺梯度。所有 11 例患者随后均接受了肝移植，截至目前死亡率为 0%；随访时间从 7 至 60 个月不等。肝移植后，11 例患者中的 7 例（64%）停用了所有肺动脉扩张剂，仅 2 例患者需要暂时增加前列腺素的剂量。总之，用西地那非和/或曲前列尼尔积极治疗 PoPH 并随后进行肝移植可能对某些患者的 PoPH 具有治愈作用。
