Bosentan therapy for pulmonary arterial hypertension associated with hereditary haemorrhagic telangiectasia.
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.
Bonderman
D
D
Department of Cardiology, Medical University of Vienna, Vienna, Austria.
Nowotny
R
R
Skoro-Sajer
N
N
Adlbrecht
C
C
Lang
I M
IM
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
D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan