Ambrisentan for Treatment of Portopulmonary Hypertension
Study Details
Study Description
Brief Summary
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.
Condition or Disease | Intervention/Treatment | Phase |
---|---|---|
Phase 1/Phase 2 |
Detailed Description
Patients with clinically significant PoPH (resting mean pulmonary arterial pressure >25 mm Hg, pulmonary vascular resistance >400 dynesscm-5) will be offered treatment with ambrisentan. Patients will be followed clinically and hemodynamically up to 12 months after start of treatment.
Study Design
Arms and Interventions
Arm | Intervention/Treatment |
---|---|
Experimental: 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. |
Drug: ambrisentan
Ambrisentan (Volibris 5 mg film-coated tablets, Glaxo Smith-Kline) will be started at 5 mg every other day and increased to 5 mg daily after 4 weeks if tolerated well.
Other Names:
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Outcome Measures
Primary Outcome Measures
- pulmonary vascular resistance [week 24]
Secondary Outcome Measures
- mean arterial pulmonary pressure [week 24]
- hepatic venous pressure gradient [week 24]
- exercise capacity [week 24, 48]
- quality of life [week 24, 48]
Eligibility Criteria
Criteria
Inclusion Criteria:
-
Adult patients with portal hypertension, age >18 years
-
Cirrhosis of any etiology; Child-Pugh class A and B
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Noncirrhotic portal hypertension (e.g. chronic portal vein thrombosis)
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Informed consent
Exclusion Criteria:
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Presence of other causes for pulmonary arterial hypertension
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History of pulmonary embolism or myocardial infarction within 6 months before study start
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Child-Pugh class C
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Presence of hepatocellular carcinoma
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Liver transplantation
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HIV infection
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Severe obstructive or restrictive pulmonary disease (predicted FEV1 or VC <65%, respectively)
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Severe dilated cardiomyopathy (EF <50%)
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Latent left-heart insufficiency
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Pregnancy and lactation
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Esophageal variceal hemorrhage within the last 6 months
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Refractory ascites
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Hepatorenal syndrome
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Persistent hepatic encephalopathy > grade 1
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Bilirubin >3.0 mg/dl
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AST and/or ALT >3x ULN
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Creatinine >2.0 mg/dl
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Known hypersensitivity to ambrisentan
Contacts and Locations
Locations
Site | City | State | Country | Postal Code | |
---|---|---|---|---|---|
1 | Medical University of Graz | Graz | Austria | 8036 |
Sponsors and Collaborators
- Medical University of Graz
Investigators
- Principal Investigator: Rudolf E. Stauber, MD, Gastroenterology & Hepatology
- Study Director: Horst Olschewski, MD, Pulmonology
Study Documents (Full-Text)
None provided.More Information
Publications
None provided.- PoPH-GRZ ambrisentan