Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/77980
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Type: Journal article
Title: Predictors of mortality in connective tissue disease-associated pulmonary arterial hypertension: a cohort study
Author: Ngian, G.
Stevens, W.
Prior, D.
Gabbay, E.
Roddy, J.
Tran, A.
Minson, R.
Hill, C.
Chow, K.
Sahhar, J.
Proudman, S.
Nikpour, M.
Citation: Arthritis Research & Therapy, 2012; 14(R213):1-9
Publisher: BioMed Central Ltd.
Issue Date: 2012
ISSN: 1478-6354
1478-6362
Statement of
Responsibility: 
Gene-Siew Ngian, Wendy Stevens, David Prior, Eli Gabbay, Janet Roddy, Ai Tran, Robert Minson, Catherine Hill, Ken Chow, Joanne Sahhar, Susanna Proudman and Mandana Nikpour
Abstract: Introduction: Pulmonary arterial hypertension (PAH) is a major cause of mortality in connective tissue disease (CTD). We sought to quantify survival and determine factors predictive of mortality in a cohort of patients with CTD-associated PAH (CTD-PAH) in the current era of advanced PAH therapy. Methods: Patients with right heart catheter proven CTD-PAH were recruited from six specialised PAH treatment centres across Australia and followed prospectively. Using survival methods including Cox proportional hazards regression, we modelled for all-cause mortality. Independent variables included demographic, clinical and hemodynamic data. Results: Among 117 patients (104 (94.9%) with systemic sclerosis), during 2.6 ± 1.8 (mean ± SD) years of follow-up from PAH diagnosis, there were 32 (27.4%) deaths. One-, two- and three-year survivals were 94%, 89% and 73%, respectively. In multiple regression analysis, higher mean right atrial pressure (mRAP) at diagnosis (hazard ratio (HR) = 1.13, 95% CI: 1.04 to 1.24, P = 0.007), lower baseline six-minute walk distance (HR = 0.64, 95% CI: 0.43 to 0.97, P = 0.04), higher baseline World Health Organization functional class (HR = 3.42, 95% CI: 1.25 to 9.36, P = 0.04) and presence of a pericardial effusion (HR = 3.39, 95% CI: 1.07 to 10.68, P = 0.04) were predictive of mortality. Warfarin (HR = 0.20, 95% CI: 0.05 to 0.78, P = 0.02) and combination PAH therapy (HR = 0.20, 95% CI: 0.05 to 0.83, P = 0.03) were protective. Conclusions: In this cohort of CTD-PAH patients, three-year survival was 73%. Independent therapeutic predictors of survival included warfarin and combination PAH therapy. Our findings suggest that anticoagulation and combination PAH therapy may improve survival in CTD-PAH. This observation merits further evaluation in randomised controlled trials.
Keywords: Connective tissue disease; mortality; prognosis
Description: Extent: 9p.
Rights: © 2012 Ngian et al.; licensee BioMed Central Ltd.This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
RMID: 0020127790
DOI: 10.1186/ar4051
Appears in Collections:Medicine publications

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