Bosentan appears to improve clinical status and offer a survival advantage in cirrhotic patients with well preserved liver function and severe portopulmonary hypertension (PPHT), according to research published in the European Respiratory Journal.
The retrospective analysis included data from 31 patients with Child class A or B cirrhosis and severe portopulmonary hypertension (PPHT) who were treated for up to three years with either inhaled iloprost (n = 13) or bosentan (n = 18). Iloprost was given six times daily at a dose of 5 mcg at the mouthpiece using standard nebulisers.
Bosentan was started at a dose of 62.5mg twice daily for four weeks followed by 125mg twice daily as target maintenance dose.
The primary study outcome was survival; secondary outcomes included event-free survival, haemodynamics, functional class, six-minute walk distance and safety.
In the iloprost group, the survival rates at one, two and three years were 77, 62 and 46%, respectively.
In the bosentan group, the respective survival rates were 94, 89 and 89% (the difference between both groups was statistically significant (p = 0.029).
Event-free survival rates were also statistically significantly better in the bosentan group (p=0.017).
However, the researchers concluded that although bosentan appeared to have better outcomes than iloprost, these findings came from a retrospective analysis and further larger prospective trials were needed to confirm this.