Experience with inhaled iloprost and bosentan in portopulmonary hypertension

¹ Dept of Respiratory Medicine, Hannover Medical School, Hannover, Germany
² Dept of Respiratory Medicine, University of Leipzig, Germany
³ Dept of Internal Medicine I, Carl-Gustav-Carus University Dresden, Germany

^* To whom correspondence should be addressed. E-mail: hoeper.marius{at}mh-hannover.de.

	Abstract

Novel treatments such as prostanoids or endothelin receptor antagonists have been introduced for various forms of pulmonary arterial hypertension but the long-term effects of these treatments on portopulmonary hypertension are unknown.

In a retrospective analysis, we assessed the safety and efficacy of inhaled iloprost, a prostacyclin analogue, and bosentan, an endothelin receptor antagonist, in patients with portopulmonary hypertension. Thirty-one consecutive patients with Child A or B cirrhosis and severe portopulmonary hypertension were treated up to three years with either inhaled iloprost (n=13) or bosentan (n=18) and the effects on exercise capacity, hemodynamics and survival were evaluated.

In the iloprost group, the survival rates at 1, 2 and 3 years were 77%, 62% and 46%, respectively. In the bosentan group, the respective survival rates were 94%, 89% and 89% (p=0.029 by log-rank analysis). Event-free survival rates, i.e. survival without transplantation, right heart failure or clinical worsening requiring the introduction of a new treatment for pulmonary hypertension, was also significantly better in the bosentan group (p=0.017 by log-rank analysis). Bosentan had significantly better effects than inhaled iloprost on exercise capacity as determined by the 6 min walk test as well as on hemodynamics. Both treatments proved to be safe, especially in regards of liver function.

In this case series of patients with well-preserved liver function and severe portopulmonary hypertension, treatment with both inhaled iloprost and bosentan appeared to be safe. Patients treated with bosentan had higher survival rates, but prospective controlled studies are required to confirm these findings.

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