title: Bosentan therapy for pulmonary arterial hypertension associated with hereditary haemorrhagic telangiectasia.
abstract: Hereditary haemorrhagic telangiectasia (HHT) is a disorder of arteriovenous malformations and telangiectases. In rare cases affected individuals may develop typical pulmonary arterial hypertension (PAH). Vasodilator therapy has not been recommended because of a potential increase in arteriovenous shunt volume. This report is on two patients with severe HHT-associated PAH who were treated with bosentan, an oral endothelin ET(A)/ET(B) receptor antagonist. After 1 year, symptomatic and functional improvements were confirmed by haemodynamic evaluation demonstrating a significant decrease of mean pulmonary artery pressures and an increase in cardiac index, without evidence for an increase in effective shunt volume.
authors.affiliation: Department of Cardiology, Medical University of Vienna, Vienna, Austria.
authors.forename: D
authors.initials: D
authors.lastname: Bonderman
authors.forename: R
authors.initials: R
authors.lastname: Nowotny
authors.forename: N
authors.initials: N
authors.lastname: Skoro-Sajer
authors.forename: C
authors.initials: C
authors.lastname: Adlbrecht
authors.forename: I M
authors.initials: IM
authors.lastname: Lang
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D000077300:Bosentan; D065128:Endothelin Receptor Antagonists; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008875:Middle Aged; D013449:Sulfonamides / Q000008:administration & dosage*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000503:physiopathology; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan