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  • Title: Successful treatment of portopulmonary hypertension with bosentan: case report.
    Author: Stähler G, von Hunnius P.
    Journal: Eur J Clin Invest; 2006 Sep; 36 Suppl 3():62-6. PubMed ID: 16919013.
    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.
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