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Table 5 Comparison of demographic, clinical and haemodynamic characteristics of patients with PAH diagnosed at first screen and PAH diagnosed at subsequent screens

From: Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme

Characteristic PAH detected on first screening PAH detected on subsequent screening P value
mean ± SD or % mean ± SD or %
Number of patients 122 38  
Age at PAH diagnosis 63.9 ± 11.0 62.7 ± 9.5 0.56
Disease duration at PAH diagnosis 13.4 ± 12.8 14.6 ± 8.7 0.62
Female 96 (85.7%) 32 (84.2%) 0.82
Disease subtype
 Limited 81 (75.7%) 23 (62.2%) 0.03
 Diffuse 17 (15.9%) 13 (35.1%)
 MCTD 9 (8.4%) 1 (2.7%)
Status
 Alive 54 (48.2%) 26 (68.4%) 0.03
 Dead 55 (49.1%) 10 (26.3%)
 Withdrawn 3 (2.7%) 1 (2.6%)
 Unable to contact 0 (0%) 1 (2.6%)
Follow up, years 3.3 ± 2.4 5.9 ± 1.9 <0.001
WHO class at PAH diagnosis
 Class I 4 (4.0%) 0 (0%) 0.01
 Class II 16 (16.2%) 15 (40.5%)
 Class III 63 (75.9%) 20 (54.1%)
 Class IV 16 (16.2%) 2 (5.4%)
6MWD at PAH diagnosis 295.9 ± 118.3 340.6 ± 115.6 0.05
mPAP on RHC 38.2 ± 11.4 32.5 ± 8.3 0.005
mRAP on RHC 9.7 ± 8.5 8.4 ± 3.9 0.40
PVR on RHC 5.5 ± 3.1 3.8 ± 1.7 0.005
CI on RHC 2.9 ± 1.6 2.9 ± 0.7 0.87
Pericardial effusion 16 (16.0%) 1 (2.7%) 0.03
  1. Probability of PAH
  2. PAH pulmonary arterial hypertension, MCTD mixed connective tissue disease, WHO World Health Organisation, 6MWD six-minute walk distance, mPAP mean pulmonary arterial pressure, RHC right-heart catheterization, mRAP mean right atrial pressure, PVR pulmonary vascular resistance, CI cardiac index, NT-pro-BNP N-terminal pro b-type natriuretic peptide