Table 5.
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Withdrawn | 3 (2.7%) | 1 (2.6%) | |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 |
Probability of PAH
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