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Table 1 Patient characteristics at baseline

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

Characteristic Mean + SD or n(%)
Total number of patients 117
Female 105 (89.7%)
Age at PAH diagnosis*, years 61.5 ± 11.4
Disease duration at PAH diagnosis*, years 11.7 ± 11.3
Race  
Caucasian 101 (86.3%)
Asian 10 (8.5%)
Aboriginal/Torres Strait Islander 3 (2.6%)
Hispanic 1 (0.9%)
Other 2 (1.7%)
Underlying CTD  
Limited SSc 79 (72.5%)
Diffuse SSc 25 (21.4%)
MCTD 5 (4.5%)
SLE 3 (2.8%)
RA 3 (2.8%)
Undifferentiated CTD 2 (1.8%)
Anti-centromere antibody positive 56 (47.9%)
Anti-Scl-70 antibody positive 9 (7.7%)
Anti-phospholipid antibodies 27 (23.1%)
WHO functional class  
Class I 9 (7.7%)
Class II 14 (12.0%)
Class III 88 (75.2%)
Class IV 6 (5.1%)
Baseline 6MWD, m 325 ± 127
Baseline mRAP, mm Hg 6.9 ± 4.2
Baseline mPAP, mm Hg 35.9 ± 12.4
Baseline PCWP, mmHg 9.7 ± 3.9
Baseline CI, L/min/m2 2.6 ± 0.8
Baseline PVR, Wood units 6.9 ± 6.1
Pericardial effusion 14 (12.0%)
Warfarin therapy 36 (30.8%)
Pulmonary vasodilator therapy  
   Monotherapy 70 (59.8%)
   Sequential monotherapy 12 (10.3%)
   Combination therapy 34 (29.0%)
  1. * date of PAH diagnosis is the date of right heart catheterisation
  2. 6MWD, six minute walk distance; CI, cardiac index; CTD, connective tissue disease; MCTD, mixed connective tissue disease; mPAP, mean pulmonary arterial pressure; mRAP, mean right atrial pressure; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance; RA, rheumatoid arthritis; SD, standard deviation; SLE, systemic lupus erythematosus; SSc, systemic sclerosis; WHO, World Health Organization