Data on treatment of patients with portopulmonary hypertension (PoPH) are limited, as they are usually excluded from randomized controlled trials with pulmonary arterial hypertension (PAH) specific therapies. This study investigated short- and long-term efficacy/safety of bosentan in these patients, as well as pharmacokinetics.All 34 consecutive patients with PoPH treated first-line with bosentan (December 2002 -July 2009) were retrospectively evaluated. Assessments included: New York Heart Association functional class (NYHA FC), blood tests, haemodynamics, 6-minute walk distance (6MWD) and event-free status. Pharmacokinetics of bosentan in 5 patients with Child-Pugh class B cirrhosis were compared with idiopathic PAH patients.Significant improvements from baseline were observed in NYHA FC, 6MWD, and haemodynamics, and largely maintained during follow up. Patients with C-P class B cirrhosis (n=9) had significantly larger haemodynamic improvement after 5±2 months. Mean follow-up was 43±19 months; 4 patients died; seven patients had significant elevation of liver enzymes (annual rate 5.5%). Plasma concentrations of bosentan were higher in patients with cirrhosis C-P class B than observed in idiopathic PAH.These data confirm the benefit of bosentan treatment for patients with PoPH. Haemodynamic improvements were particularly pronounced in patients with more severe cirrhosis. The safety profile of bosentan was consistent with previous studies.