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Survival after the initiation of combination therapy in patients with pulmonary arterial hypertension


Keogh, A and Strange, G and Kotlyar, E and Williams, T and Kilpatrick, D and Macdonald, P and Brown, K and Pidoux, A and Kermeen, F and Steele, P and Dalton, B and Gabbay, E, Survival after the initiation of combination therapy in patients with pulmonary arterial hypertension, Internal Medicine Journal, 41, (3) pp. 235-244. ISSN 1445-5994 (2011) [Refereed Article]

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DOI: doi:10.1111/j.1445-5994.2010.02403.x


BACKGROUND:   Several cellular pathways are implicated in the pathogenesis of pulmonary arterial hypertension (PAH) and attempts to arrest disease progression with a single drug would not be expected to succeed in the medium term. In clinical practice, combination therapy is often used in patients deteriorating on monotherapy, despite the absence of firm evidence from randomized controlled controls. METHODS:   From January 2005 to August 2009, 112 patients with World Health Organisation Functional Class (FC) II-IV PAH deteriorating on monotherapy received non-parenteral combination therapy at six Australian PAH expert hospitals. Combination therapy included bosentan, sitaxentan, ambrisentan, iloprost and sildenafil. Data were prospectively collected for survival status, 6-min walk distance, FC and echocardiographic parameters at the start of monotherapy through to commencement of combination therapy and at 6-monthly intervals thereafter. RESULTS:   After varying periods of monotherapy (18.713.4onths), survival estimates on combination therapy were 88%, 71% and 61% for the additional 1, 2 and 3years respectively. Survival on dual therapy in patients with idiopathic PAH/familial PAH was 93% at 1year and 79% at 2years, and for scleroderma-related PAH, 72% at 1 year and 48% at year 2 after initiation of combination therapy. In survivors, dual therapy reversed the deterioration in FC, from 3.10.6 on monotherapy to 2.20.6 at 12months. Similarly, dual therapy improved 6-min walk distance from 316119m to 406129m at 12months, and sequential echocardiography demonstrated a fall in pulmonary artery systolic pressure and improved right ventricular function. CONCLUSIONS:   Dual non-parenteral therapy appears safe and effective and should be considered for PAH patients who are deteriorating on monotherapy to improve long-term outcomes

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:Kilpatrick, D (Professor David Kilpatrick)
UTAS Author:Dalton, B (Mr Brad Dalton)
ID Code:65443
Year Published:2011
Web of Science® Times Cited:26
Deposited By:Health Sciences A
Deposited On:2010-11-16
Last Modified:2017-11-02

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