Portopulmonary Hypertension: Bosentan Could Give A Survival Advantage

Main Category: Respiratory / Asthma
Also Included In: Hypertension;  Liver Disease / Hepatitis
Article Date: 27 Nov 2007 - 1:00 PDT

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The term portopulmonary hypertension refers to the development of pulmonary arterial hypertension (PAH) in patients with liver disease and portal hypertension. Approximately 1-2% of patients with chronic liver disease develop portopulmonary hypertension and the mortality associated with this condition reaches up to 50% within one year after diagnosis. Recently, several new treatments have been developed for other forms of PAH but patients with portopulmonary hypertension were usually excluded from clinical trials in this area. Therefore, it is unknown whether PAH- specific treatments are safe and efficacious in this patient population.

In a retrospective analysis, Marius Hoeper (Hannover Medical School, Germany) and his colleagues compare the effects of treatment with inhaled iloprost, a prostacyclin derivative, and oral bosentan, an endothelin receptor antagonist, in patients with portopulmonary hypertension.

The most striking finding of this study is a statistically significant survival advantage in bosentan-treated patients. In this group, survival at three years is 89%, compared with 46% in the iloprost group. According to the authors, the most likely explanation for this finding is a more pronounced effect on haemodynamics with bosentan therapy.

Follow-up right heart catheter examinations after approximately one year of therapy reveals that haemodynamics tend to deteriorate in the iloprost group, whereas they markedly improve in the bosentan group.

Although bosentan has a known hepatotoxic potential, the drug proved to be safe in the study. However, it is important to note that all patients had only mildly impaired liver function, e.g. Child A disease. Bosentan is not approved for patients with more severe liver dysfunction.

In summary, this study provides preliminary evidence for a survival advantage of portopulmonary hypertension patients treated with bosentan compared with those treated with inhaled iloprost. These findings call for further head-to-head comparisons of these treatments, not only in patients with portopulmonary hypertension but also in other forms of PAH.

The European Respiratory Journal is the peer-reviewed scientific publication of the European Respiratory Society (more than 8,000 specialists in lung diseases and respiratory medicine in Europe, the United States and Australia).

European Respiratory Society