title: Successful switch from inhalative iloprost to oral bosentan in portopulmonary hypertension associated with liver cirrhosis.
abstract: Portopulmonary hypertension (PPHTN) is a rare complication of liver cirrhosis. Prostanoids have been shown to be effective in the treatment of PPHTN and have been used as a bridge to orthotopic liver transplantation. However, inhibition of platelet aggregation might be a limitation of prostacyclin therapy in patients with end-stage liver disease having an increased risk of bleeding from esophageal varices. The effect of oral bosentan, a dual endothelin-receptor antagonist in the reversal of PPHTN, is still unclear. We report a case of PPHTN (mean pulmonary artery pressure [mPAP] of 51 mmHg) that was successfully switched from inhalative iloprost to oral bosentan therapy. Hemodynamic and symptomatic improvements were maintained after a 12-month long-term treatment with inhalative iloprost as well as after single oral bosentan therapy. This is the first reported case of a successful switch from therapy with an inhalative prostacyclin analogue to oral bosentan in a patient suffering from PPHTN. Thus, oral bosentan therapy might be a promising new option for patients suffering from PPHTN.
authors.affiliation: Pneumology Service, Clinical Department of Internal Medicine, Innsbruck Medical University, Innsbruck, Austria.
authors.forename: Clemens
authors.initials: C
authors.lastname: Molnar
authors.forename: Hannes
authors.initials: H
authors.lastname: Alber
authors.forename: Daniela
authors.initials: D
authors.lastname: Colleselli
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Vogel
authors.forename: Christian M
authors.initials: CM
authors.lastname: Kähler
mesh_terms: D000280:Administration, Inhalation; D000284:Administration, Oral; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D000077300:Bosentan; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage*; D008103:Liver Cirrhosis / Q000150:complications* / Q000503:physiopathology; D016031:Liver Transplantation; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage*; D013449:Sulfonamides / Q000008:administration & dosage*; D013997:Time Factors
chemical_list: D000959:Antihypertensive Agents; D010975:Platelet Aggregation Inhibitors; D013449:Sulfonamides; D016285:Iloprost; D000077300:Bosentan