Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension

Continuous intravenous infusion of epoprostenol for the treatment of portopulmonary hypertension
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DOI:
10.1097/00007890-199702270-00020
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发表时间:
1997-02-27
期刊:
影响因子:
6.2
通讯作者:
Rubin, LJ
Rubin, LJ
中科院分区:
医学2区
文献类型:
--
作者:
Kuo, PC;Johnson, LB;Rubin, LJ

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肺动脉高压与门脉高压相关,也称为门脉高压(PPHTN),是慢性肝病的已知并发症。以前,PPHTN的存在被认为是原位肝移植(OLT)的禁忌症。虽然在PPHTN的设置中有一些成功的OLT的病例报告,但与OLT和PPHTN相关的死亡率过高。到目前为止,PPHTN的慢性治疗还缺乏治疗,最近,持续静脉注射环前列腺素被证明可以改善普通人群的症状和原发性肺动脉高压患者的存活率。我们现在报告在PPHTN的更具体的实例中使用环前列腺素。在6-14个月的时间里,在4名患者中,依普列汀(10-28 ng/kg/min)治疗后,平均肺动脉压下降29-46%,肺血管阻力下降22-71%,心输出量增加25%-75%。这些结果表明,PPHTN的有效慢性治疗是可行的。联合吸入一氧化氮作为术中急性治疗,注射环前列腺素是治疗肝移植候选患者PPHTN的另一种治疗选择。
The association of pulmonary hypertension with portal hypertension, also called portopulmonary hypertension (PPHTN), is a known complication of chronic liver disease. Previously, the presence of PPHTN was considered to be a contraindication to orthotopic liver transplantation (OLT). Although there are selected case reports of successful OLT in the setting of PPHTN, an excessive mortality rate is associated with OLT and PPHTN. Heretofore, therapy for chronic management of PPHTN was lacking, Recently, continuous intravenous infusion of epoprostenol has been demonstrated to improve symptomatology and survival in the general population of patients with primary pulmonary hypertension. We now report the use of epoprostenol in the more specific instance of PPHTN. Over a period of 6-14 months, epoprostenol (10-28 ng/kg/min) therapy was associated with a 29-46% decrease in mean pulmonary artery pressure, a 22-71% decrease in pulmonary vascular resistance, and a 25-75% increase in cardiac output in a group of four patients. These results suggest that effective chronic therapy for PPHTN is available. In conjunction with inhaled nitric oxide as acute intraoperative therapy, epoprostenol infusion represents an additional therapeutic option for treatment of PPHTN in the liver transplant candidate.