HPS is by far the most frequent respiratory complication of cirrhosis leading to significantly increased mortality. The only therapeutic option is liver transplantation. Many patients with HPS who are screened for liver transplantation are not listed for transplantation due to several reasons. Therefore, an effective medical treatment of HPS would be of central importance and may revolutionise the management in this frequent syndrome.
Conditions
Interventions
Trade Name: Tracleer 62,5 mg-Filmtabletten
Product Name: Tracleer
Pharmaceutical Form: Film-coated tablet
INN or Proposed INN: bosentan
CAS Number: 157212-55-0
Current Sponsor code: C02KX01
Other desc
Sponsors
Med. Univ. Wien, Univ. Klinikum für Interne Medizin III
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1.) presence of hepatopulmonary syndrome 2.) age > 18 years Are the trial subjects under 18? no Number of subjects for this age range: 0 F.1.2 Adults (18-64 years) yes F.1.2.1 Number of subjects for this age range 18 F.1.3 Elderly (>=65 years) yes F.1.3.1 Number of subjects for this age range 18
Exclusion criteria
Exclusion criteria: 1.) intracardiac shunting
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: To investigate the efficacy and safety of bosentan in patients with liver mild cirrhosis and hepatopulmonary syndrome.;Secondary Objective: To assess safety and tolerability of bosentan To asses effect of bosentan treatment on quality of live To assess effects of bosentan on pulmonary gas exchange and exhaled nitric oxide levels To assess effects of bosentan on liver function parameters and endothelin levels and on hepatic hemodynamics ;Primary end point(s): Primary: - Changes in 6-minute walking distance before and after 12 weeks of therapy with bosentan, respectively in patients with bosentan and the placebo group - Changes in gas exchange (PaO2, AaDO2) before and after 12 weeks of therapy with bosentan, respectively in patients with bosentan and the placebo group ;Timepoint(s) of evaluation of this end point: see E.5.1 | — |
Secondary
| Measure | Time frame |
|---|---|
| Secondary end point(s): A variety of parameters will be assessed to characterize the effect of dual ET-receptor antagonism on hepatopulmonary syndrome in patients with cirrhosis: - Gas exchange (PaO2, AaDO2, exercise testing) - WHO functional class - Pulmonary hemodynamic changes - Hepatic hemodynamic changes (portal pressure – hepatic venous pressure gradient) - Quality of life assessment - Severity of liver cirrhosis (CHILD-Pugh classification and MELD score) - Complications of liver cirrhosis (GI-bleeding, infections, Ascites, hepatorenal syndrome, hepatic encephalopathy) - Pro-BNP-level - ET-levels - Indocyanine green clearance - Exhaled nitric oxide (NO) measurement - Liver histology (via transjugular liver biopsy at baseline and after 12 weeks) Evaluation of safety: All patients will be seen monthly in the outpatient clinics of the Department of Gastroenterology and Hepatology, Medical University Vienna, for a thorough clinical investigation, including assessment of NYHA Functional class, 6 minutes walking distance, blood gas analysis, pulmonary and liver function assessment and exhaled NO measurement. Liver enzymes, including alanine aminotransferase and aspartate aminotransferase, bilirubin levels and INR will be monitored twice monthly. Monitoring, dosage adjustment and discontinuation of therapy will be performed in patients developing aminotransferase elevations > 3 times upper limit of normal (10). ;Timepoint(s) of evaluation of this end point: see E.5.2 | — |
Countries
Austria
Contacts
Public ContactDepartment of Internal Medicine 3
Medical University Vienna
Outcome results
None listed