Bosentan treatment of portopulmonary hypertension related to liver cirrhosis owing to hepatitis C.
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.
Grander
W
W
Department of Internal Medicine, Community Hospital Hall, Hall/Tirol, Austria. wilhelm.grander@bkh-hall.or.at
Eller
P
P
Fuschelberger
R
R
Tilg
H
H
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
D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan