Portopulmonary Hypertension
Conditions
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.
Detailed 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.
Interventions
DRUGambrisentan
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.
Sponsors
Medical University of Graz
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| pulmonary vascular resistance | week 24 |
Secondary
| Measure | Time frame |
|---|---|
| mean arterial pulmonary pressure | week 24 |
| hepatic venous pressure gradient | week 24 |
| exercise capacity | week 24, 48 |
| quality of life | week 24, 48 |
Countries
Austria
Outcome results
None listed