title: Efficacy and safety of bosentan treatment for portopulmonary hypertension associated with syncope.
abstract: Pulmonary arterial hypertension (PAH) in patients with portal hypertension is also referred to as portopulmonary hypertension (PPHTN). Here, we report a case of PPHTN caused by alcoholic liver cirrhosis in a 43-year-old male who experienced repetitive syncope on exertion. The continuous monitoring of pulmonary artery pressure and radial artery pressure revealed that his PAH was aggravated with a drop in systemic arterial pressure during an exercise test. Bosentan, an endothelin A/B receptor antagonist, improved the patient's hemodynamic parameters and abolished his syncope without adverse effects. This is the first report that bosentan may be effective and safe for PPHTN associated with syncope.
authors.affiliation: Department of Cardiology, Niigata Prefectural Shibata Hospital, Niigata, Japan.
authors.forename: Akinori
authors.initials: A
authors.lastname: Sato
authors.forename: Kouichiro
authors.initials: K
authors.lastname: Maie
authors.forename: Yukako
authors.initials: Y
authors.lastname: Ohno
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Yoshida
authors.forename: Eiichi
authors.initials: E
authors.lastname: Ito
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Tanabe
authors.forename: Yoshifusa
authors.initials: Y
authors.lastname: Aizawa
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000077300:Bosentan; D004305:Dose-Response Relationship, Drug; D005500:Follow-Up Studies; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D008991:Monitoring, Physiologic; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use*; D013575:Syncope / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan