Diagnosis | No PH | Manifest PH | ||||
---|---|---|---|---|---|---|
mPAP < 25 mm Hg | mPAP ≥ 25 mm Hg | |||||
n = 54 | n = 22 | |||||
Mean | SD | Mean | SD | P value | ||
Patient characteristics | Age, years | 54.0 | 14.4 | 67.6 | 8.8 | <0.001 |
Height, cm | 167.3 | 7.8 | 162.1 | 8.1 | 0.02 | |
Weight, kg | 70.0 | 16.0 | 66.0 | 10.5 | n.s. | |
Body surface area, m2 | 1.77 | 0.2 | 1.7 | 0.2 | n.s. | |
Duration of SSc, years | 10.7 | 9.9 | 15.6 | 14.4 | n.s. | |
Beginning of SSc, years | 44.5 | 15.0 | 52.4 | 16.0 | 0.04 | |
Modified Rodnan Skin Score | 13.3 | 8.5 | 18.4 | 11.4 | 0.04 | |
6MWD, m | 466 | 77 | 349 | 86 | <0.001 | |
Borg Dyspnoea 20 | 13 | 2 | 15 | 2 | 0.003 | |
WHO FC | 0-I | 17 (31.5 %) | 0 | |||
II | 26 (48.1 %) | 5 (22.7 %) | ||||
III | 10 (18.5 %) | 17 (77.3 %) | ||||
Unknown | 1 (1.9 %) | 0 | ||||
Laboratory | NT-proBNP, pg/mL | 290 | 424 | 846 | 773.0 | 0.02 |
Blood gas analysis | Oxygen saturation, % | 96.8 | 2.1 | 94.6 | 3.4 | 0.01 |
Lung function | Vital capacity, % | 95.8 | 24.2 | 88.2 | 29.9 | n.s. |
FEV1, % | 108.0 | 93.7 | 83.7 | 26.9 | n.s. | |
Total lung capacity, % | 95.5 | 21.4 | 83.8 | 23.2 | 0.042 | |
Residual volume, % | 2.0 | 0.8 | 1.9 | 0.6 | n.s. | |
Diffusion capacity | Diffusion capacity carbon monoxide, % | 55.7 | 13.5 | 42.1 | 12.5 | <0.001 |
Echocardio- | RV thickness, mm | 6.5 | 1.2 | 7.6 | 1.4 | 0.01 |
graphy | TAPSE, mm | 24.0 | 3.5 | 20.6 | 4.0 | 0.001 |
RA area, cm2 | 11.4 | 3.3 | 15.5 | 5.7 | <0.001 | |
RV area, cm2 | 14.6 | 3.7 | 17.7 | 4.2 | 0.003 | |
TRV, m/s | 2.3 | 0.4 | 3.4 | 0.6 | <0.001 | |
PASP, mm Hg | 25.6 | 7.3 | 52.0 | 18.0 | <0.001 | |
PASP max (exercise), mm Hg | 49.9 | 12.7 | 83.9 | 18.9 | <0.001 | |
Right heart | RAP/CVP, mm Hg | 3.8 | 2.6 | 6.3 | 4.5 | 0.02 |
catheterisation | mPAP, mm Hg | 14.8 | 3.4 | 32.6 | 7.5 | <0.001 |
at rest | PAWP, mm Hg | 7.2 | 3.2 | 12.4 | 6.0 | 0.001 |
Cardiac output, ml/min | 5.6 | 1.3 | 5.1 | 1.0 | n.s. | |
Cardiac index, l/min per m2 | 3.1 | 0.6 | 2.9 | 0.5 | n.s. | |
PVR, dynes*s/cm5 | 114 | 40 | 339 | 199 | <0.001 | |
During exercise | mPAP max, mm Hg | 31 | 7 | 50 | 7 | <0.001 |
PAWP max, mm Hg | 17 | 6 | 21 | 9 | n.s. | |
Cardiac output max, l/min | 11.5 | 3.4 | 8.3 | 2.9 | <0.001 | |
PVR max, dynes*s/cm5 | 96 | 42 | 323 | 207 | <0.001 |