Ambrisentan for Treatment of Portopulmonary Hypertension

Medical University of Graz

Overview

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.

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.

Study Type

INTERVENTIONAL

Allocation

Purpose

TREATMENT

Masking

NONE

Conditions

Portopulmonary Hypertension

Interventions

ambrisentanDRUG

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.

Eligibility

Sex: ALLMin age: 18 Years

Medical Language ↔ Plain English

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

Locations (1)

Medical University of Graz

Graz, Austria

Outcomes

Primary Outcomes

pulmonary vascular resistance

Time frame: week 24

Secondary Outcomes

mean arterial pulmonary pressure

Time frame: week 24

hepatic venous pressure gradient

Time frame: week 24

exercise capacity

Time frame: week 24, 48

quality of life

Time frame: week 24, 48

Data from ClinicalTrials.gov

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