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

Arthritis Res Ther. 2012 Oct 5;14(5):R213. doi: 10.1186/ar4051.

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.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Australia
  • Blood Pressure / physiology
  • Cohort Studies
  • Connective Tissue Diseases / complications*
  • Connective Tissue Diseases / epidemiology
  • Connective Tissue Diseases / physiopathology
  • Female
  • Follow-Up Studies
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / mortality*
  • Hypertension, Pulmonary / physiopathology
  • Male
  • Middle Aged
  • Pericardial Effusion / complications
  • Pericardial Effusion / diagnosis
  • Prognosis
  • Proportional Hazards Models
  • Survival Rate
  • Walking / physiology
  • Warfarin / therapeutic use

Substances

  • Warfarin