Ambrisentan for Treatment of Portopulmonary Hypertension (PoPH): a Pilot Study
试验速览
- 阶段
- 1 期
- 状态
- 撤回
- 发起方
- 试验地点
- 1
- 主要终点
- pulmonary vascular resistance
研究概览
简要总结
Portopulmonary hypertension denotes pulmonary hypertension complicating portal hypertension and is present in approximately 5% of cirrhotic patients. Treatment options include prostanoids, sildenafil, and the endothelin-receptor antagonists, bosentan and ambrisentan.
This study investigates the safety and efficacy of ambrisentan in portopulmonary hypertension.
详细描述
Patients with clinically significant PoPH (resting mean pulmonary arterial pressure >25 mm Hg, pulmonary vascular resistance >400 dynes*s*cm-5) will be offered treatment with ambrisentan. Patients will be followed clinically and hemodynamically up to 12 months after start of treatment.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Adult patients with portal hypertension, age >18 years
- •Cirrhosis of any etiology; Child-Pugh class A and B
- •Noncirrhotic portal hypertension (e.g. chronic portal vein thrombosis)
- •Informed consent
排除标准
- •Presence of other causes for pulmonary arterial hypertension
- •History of pulmonary embolism or myocardial infarction within 6 months before study start
- •Child-Pugh class C
- •Presence of hepatocellular carcinoma
- •Liver transplantation
- •HIV infection
- •Severe obstructive or restrictive pulmonary disease (predicted FEV1 or VC <65%, respectively)
- •Severe dilated cardiomyopathy (EF <50%)
- •Latent left-heart insufficiency
- •Pregnancy and lactation
- •Esophageal variceal hemorrhage within the last 6 months
- •Refractory ascites
- •Hepatorenal syndrome
- •Persistent hepatic encephalopathy > grade 1
- •Bilirubin >3.0 mg/dl
- •AST and/or ALT >3x ULN
- •Creatinine >2.0 mg/dl
- •Known hypersensitivity to ambrisentan
研究组 & 干预措施
ambrisentan
In all patients with clinically significant PoPH, ambrisentan will be administered orally using a low ascending dose regime (see below). Duration of treatment will be 12 months.
干预措施: ambrisentan (Drug)
结局指标
主要结局
pulmonary vascular resistance
时间窗: week 24
次要结局
- hepatic venous pressure gradient(week 24)
- mean arterial pulmonary pressure(week 24)
- exercise capacity(week 24, 48)
- quality of life(week 24, 48)