title: Bosentan treatment of portopulmonary hypertension related to liver cirrhosis owing to hepatitis C.
abstract: Pulmonary arterial hypertension (PAH) with coexisting portal hypertension has been defined as portopulmonary hypertension (PPHTN). It is often related to liver cirrhosis of various aetiologies and is associated with a high mortality rate. Endothelin-1 (ET) is supposed to play an important role in the pathogenesis of PAH as well as portal hypertension. Therefore, therapy with an ET(A)/ET(B) receptor antagonist might be of use in the treatment of PPHTN. We report the case of a 76-year-old male with liver cirrhosis owing to chronic hepatitis C virus infection and PPHTN who was treated with the dual ET(A)/ET(B) receptor antagonist bosentan. The patient showed remarkable improvement of 6-min walking distance from 300 to 480 m after 2 weeks and to 540 m after 14 weeks, respectively. In addition, a significant decline of N-terminal pro B-type natriuretic peptide fraction (NT-proBNP) from 4928 ng mL(-1) to 640 ng mL(-1) was observed. Bosentan might be a promising new therapeutical option for patients suffering from PPHTN.
authors.affiliation: Department of Internal Medicine, Community Hospital Hall, Hall/Tirol, Austria. wilhelm.grander@bkh-hall.or.at
authors.forename: W
authors.initials: W
authors.lastname: Grander
authors.forename: P
authors.initials: P
authors.lastname: Eller
authors.forename: R
authors.initials: R
authors.lastname: Fuschelberger
authors.forename: H
authors.initials: H
authors.lastname: Tilg
mesh_terms: D000284:Administration, Oral; D000368:Aged; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists; D019698:Hepatitis C, Chronic / Q000150:complications*; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy* / Q000209:etiology; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008103:Liver Cirrhosis / Q000150:complications*; D008297:Male; D013449:Sulfonamides / Q000008:administration & dosage*; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan