Successful treatment of portopulmonary hypertension with bosentan: case report

Authors: Stähler, G.; von Hunnius, P.

Source: European Journal of Clinical Investigation, Volume 36, Supplement 3, September 2006 , pp. 62-66(5)

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Abstract:

Pulmonary arterial hypertension (PAH) is found in 2-20% of cirrhosis patients who have portal hypertension (portopulmonary hypertension, PPHT). Endothelin (ET), a potent vasoconstrictor, is likely to play a role in the pathogenesis of portal hypertension. We describe the long-term successful use of the dual ET_A/ET_B receptor antagonist bosentan in a 43-year-old male with alcohol-related cirrhosis (Child-Pugh A), right ventricular enlargement and dysfunction, respectively, and moderate PAH. Elevated pulmonary arterial pressure was substantially reduced and exercise capacity increased. Improvement was maintained over 2 years, and bosentan treatment continues in this patient. Our report is in line with a series of current reports in PPHT that support the use of bosentan in this subset of PAH patients.

Eur J Clin Invest 2006; 36 (Suppl. 3): 62-66

Keywords: Bosentan; endothelin; liver cirrhosis; portal hypertension; portopulmonary hypertension; pulmonary arterial hypertension

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1365-2362.2006.01693.x

Publication date: 2006-09-01