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Successful treatment of portopulmonary hypertension with bosentan: case report

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 ETA/ETB 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.