Journal article

Predictors of mortality in connective tissue disease-associated pulmonary arterial hypertension: a cohort study

GS Ngian, W Stevens, D Prior, E Gabbay, J Roddy, A Tran, R Minson, C Hill, K Chow, J Sahhar, S Proudman, M Nikpour

Arthritis Research and Therapy | BioMed Central | Published : 2012


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 scler..

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