Treatment of portopulmonary hypertension (high blood pressure in the pulmonary circulation) with ambrisentan (a medicinal product for the therapy of portopulmonary hypertension).

Phase 1

• Conditions: Portopulmonary Hypertension (PoPH) and Hepatopulmonary Syndrome HPS are present in a considerable number of patients with compensated cirrhosis. Treatment of PoPH with ambrisentan is well tolerated and improves hemodynamics as well as symptoms and physical capacity.; MedDRA version: 14.0 Level: PT Classification code 10067281 Term: Portopulmonary hypertension System Organ Class: 10038738 - Respiratory, thoracic and mediastinal disorders; Therapeutic area: Diseases [C] - Respiratory Tract Diseases [C08]

• Registration Number: EUCTR2011-001139-22-AT
• Lead Sponsor: Medizinische Universität Graz, Klin. Abteilung für Gastroenterologie und Hepatologie

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Results First Posted: 1900-01-01
• Study Start: 2011-05-11
• Last Update Posted: 11 March 2020

Recruitment & Eligibility

• Status: ot Recruiting
• Sex: Not specified
• Target Recruitment: 12

Inclusion Criteria

Adult patients with portal hypertension, age >18 years
Cirrhosis of any etiology; Child-Pugh class A, B, or C
Noncirrhotic portal hypertension (e.g. chronic portal vein thrombosis)
Signed Informed consent

Are the trial subjects under 18? no
Number of subjects for this age range:
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range
F.1.3 Elderly (>=65 years) yes
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

- Presence of other causes for PAH
- History of pulmonary embolism or myocardial infarction within 6 months before study start
-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%)
-Pregnancy
-Esophageal variceal hemorrhage within the last 6 weeks
-Refractory ascites
-Hepatorenal syndrome
-Persistent hepatic encephalopathy > grade 1

Study & Design

• Study Type: Interventional clinical trial of medicinal product
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Main Objective: To assess the prevalence of PoPH and HPS in patients with portal hypertension seen in a referral center, using sensitive screening tools.;<br> Secondary Objective: 2. To relate PoPH with gender, etiology of cirrhosis, and the degree of liver dysfunction as estimated by the Child-Pugh score or the model for end-stage liver disease (MELD)<br> 3. To relate PVR with portal hemodynamic parameters and circulating vasoactive mediators such as ADMA<br> 4. To evaluate the efficacy and safety of treatment with the endothelin receptor antagonist ambrisentan in patients with clinically significant PoPH<br> ;<br> Primary end point(s): Resting Pulmonary Vascular Resistance (PVR)<br> ;Timepoint(s) of evaluation of this end point: At baseline and 6 months after the start of treatment

Secondary Outcome Measures

Name	Time	Method
Timepoint(s) of evaluation of this end point: At baseline, 6 and 12 months after the start of treatment, respectively. For details please refer to the protocoll.;Secondary end point(s): Secondary endpoints include mPAP, HVPG, 6-min walk distance, peak VO2, VAS, SF-36, CAMPHOR score, safety (including detailed liver function tests)