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The bosentan patient registry: long-term survival in pulmonary arterial hypertension


Keogh, A and Strange, G and McNeil, K and Williams, TJ and Gabbay, E and Proudman, S and Weintraub, RG and Wlodarczyk, J and Dalton, B, The bosentan patient registry: long-term survival in pulmonary arterial hypertension, Internal Medicine Journal, 41, (3) pp. 227-234. ISSN 1444-0903 (2011) [Refereed Article]

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DOI: doi:10.1111/j.1445-5994.2009.02139.x


BACKGROUND/AIMS: The Bosentan Patient Registry (BPR) was a prospective, multicentre, Australian registry funded by Actelion Pharmaceuticals. The primary aim of the registry was to collect survival data in patients with pulmonary arterial hypertension (PAH) treated with bosentan. METHODS: The BPR was initiated in 15 specialized PAH centres. All patients on or starting bosentan were invited to enrol. Treating physicians notified the registry if patients discontinued bosentan, because of either a change in therapy, transplantation, intervention or death. Survival data were validated against the Australian Institute of Health and Welfare National Death Index. RESULTS: Between 2004 and 2007, a total of 528 patients (mean age 59 17 years) were enrolled representing 69% of patients either previously taking or initiated on bosentan during that time. The BPR population was generally older with more advanced functional deficit than patients enrolled in randomized, placebo-controlled trials. Aetiology was idiopathic (iPAH) in 58% and connective tissue disease related (scleroderma (SSc)-PAH) in 42%. For iPAH patients, World Health Organisation functional classes II, III and IV at enrolment was 8.2%, 66.4% and 20.5%, and for the SSc-PAH cohort, 3.2%, 75.8% and 17.9% respectively. The observed annual mortality was 11.8% in patients with iPAH and 16.6% in patients SSc-PAH. CONCLUSION: This large Australian registry provides 'real life' information on the characteristics and management of PAH in clinical practice. Treatment with bosentan improved survival outcomes in both iPAH and SSc-PAH compared with historical controls. Age, disease severity and aetiology were critical factors in determining clinical outcome

Item Details

Item Type:Refereed Article
Research Division:Biomedical and Clinical Sciences
Research Group:Cardiovascular medicine and haematology
Research Field:Cardiology (incl. cardiovascular diseases)
Objective Division:Health
Objective Group:Clinical health
Objective Field:Clinical health not elsewhere classified
UTAS Author:Dalton, B (Mr Brad Dalton)
ID Code:71775
Year Published:2011
Web of Science® Times Cited:24
Deposited By:Health Sciences A
Deposited On:2011-08-05
Last Modified:2012-04-18

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