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