title: Predictors of mortality in connective tissue disease-associated pulmonary arterial hypertension: a cohort study.
abstract: BACKGROUND
  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.
authors.forename: Gene-Siew
authors.initials: GS
authors.lastname: Ngian
authors.forename: Wendy
authors.initials: W
authors.lastname: Stevens
authors.forename: David
authors.initials: D
authors.lastname: Prior
authors.forename: Eli
authors.initials: E
authors.lastname: Gabbay
authors.forename: Janet
authors.initials: J
authors.lastname: Roddy
authors.forename: Ai
authors.initials: A
authors.lastname: Tran
authors.forename: Robert
authors.initials: R
authors.lastname: Minson
authors.forename: Catherine
authors.initials: C
authors.lastname: Hill
authors.forename: Ken
authors.initials: K
authors.lastname: Chow
authors.forename: Joanne
authors.initials: J
authors.lastname: Sahhar
authors.forename: Susanna
authors.initials: S
authors.lastname: Proudman
authors.forename: Mandana
authors.initials: M
authors.lastname: Nikpour
mesh_terms: D000368:Aged; D001315:Australia; D001794:Blood Pressure / Q000502:physiology; D015331:Cohort Studies; D003240:Connective Tissue Diseases / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000401:mortality* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010490:Pericardial Effusion / Q000150:complications / Q000175:diagnosis; D011379:Prognosis; D016016:Proportional Hazards Models; D015996:Survival Rate; D016138:Walking / Q000502:physiology; D014859:Warfarin / Q000627:therapeutic use
chemical_list: D014859:Warfarin