|
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Withdrawn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
