Ambrisentan for Treatment of Portopulmonary Hypertension
Ambrisentan for Treatment of Portopulmonary Hypertension
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.
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