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Bosentan may prolong survival in portopulmonary hypertension

Tuesday, December 04 2007 | Comments

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What's This? By Karla Gale

NEW YORK (Reuters Health) - In cirrhotic patients with well preserved liver function and severe portopulmonary hypertension (PPHT), treatment with bosentan appears to improve clinical status and offer a survival advantage, German researchers report.

Lead author Dr. Marius M. Hoeper and his associates add that in their small case series, the inhaled prostanoid iloprost appears to be as safe as bosentan, an endothelin receptor antagonist, but it appeared to provide no clinical benefits.

"PPHT is diagnosed when pulmonary arterial hypertension develops in a patient with liver disease and portal hypertension," Dr. Hoeper told Reuters Health. "The underlying mechanisms are unknown although the good response to endothelin receptor antagonists suggests that ET-1 is involved."

While bosentan and iloprost have been shown effective in pulmonary arterial hypertension, the long-term safety and effectiveness of these treatments have not been evaluated in patients with PPHT, Dr. Hoeper, at Hannover Medical School, and his team note in the European Respiratory Journal for December.

Their analysis included 31 patients with Child class A or B cirrhosis and severe PPHT treated for up to 3 years with iloprost (n = 13) or bosentan (n = 18), at the discretion of their physicians.

Right heart catheterization conducted 3 to 18 months after treatment initiation demonstrated substantially better hemodynamics only in patients taking bosentan. Bosentan also led to more sustained improvement in exercise capacity.

Three-year survival rates were 89% in the bosentan group and 46% in the iloprost group (p = 0.029).

Both drugs were well tolerated, the authors note. Only one patient experienced reversible aminotransferase increases to more than three times the upper level of normal. The team emphasizes that bosentan is not approved for patients with Child class B or C cirrhosis.

"For the time being, there is no approved treatment for PPHT and I would strongly recommend bosentan as a therapeutic option as long as liver function is well preserved (Child A)," Dr. Hoeper added. "As in all forms of pulmonary arterial hypertension, treatment should probably start as soon as possible."

As for other options, "I think that iloprost should no longer be considered for PPHT unless other treatments are not applicable (which is unlikely to occur)," he concluded. "Based on recent publications, sildenafil may also be an option for these patients and there are also positive reports on intravenous epoprostenol."

Eur Respir J 2007;30:1096-1102.

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