title: Successful treatment of portopulmonary hypertension with bosentan: case report.
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.
authors.affiliation: Medical Clinic I, Klinik Löwenstein, Germany. gerd.staehler@klinik-loewenstein.de
authors.forename: G
authors.initials: G
authors.lastname: Stähler
authors.forename: P
authors.initials: P
authors.lastname: von Hunnius
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D000077300:Bosentan; D004452:Echocardiography / Q000379:methods; D065128:Endothelin Receptor Antagonists; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy* / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D008104:Liver Cirrhosis, Alcoholic / Q000150:complications / Q000503:physiopathology; D008297:Male; D013449:Sulfonamides / Q000008:administration & dosage*; D016896:Treatment Outcome; D014664:Vasodilation / Q000187:drug effects
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan