Ambrisentan for Treatment of Portopulmonary Hypertension

Medical University of Graz

Status and phase: Withdrawn; Phase 2; Phase 1

Conditions: Portopulmonary Hypertension

Treatments: Drug: ambrisentan

Study type: Interventional

Funder types: Other

Identifiers

NCT01733095

PoPH-GRZ ambrisentan

Details and patient eligibility

About

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.

Full description

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.

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• 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

Exclusion criteria

• 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

Trial design

Primary purpose: Treatment

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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0 participants in 1 patient group

ambrisentan

Experimental group

Description:

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.

Treatment:

Drug: ambrisentan