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Bosentan therapy for pulmonary arterial hypertension associated with hereditary haemorrhagic telangiectasia
Author(s) -
Bonderman D.,
Nowotny R.,
SkoroSajer N.,
Adlbrecht C.,
Lang I. M.
Publication year - 2006
Publication title -
european journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.164
H-Index - 107
eISSN - 1365-2362
pISSN - 0014-2972
DOI - 10.1111/j.1365-2362.2006.01683.x
Subject(s) - bosentan , medicine , telangiectases , cardiology , telangiectasia , pulmonary hypertension , vasodilation , pulmonary artery , endothelin receptor antagonist , endothelin receptor , surgery , receptor
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.