Table 2.
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 |
PH pulmonary hypertension, mPAP mean pulmonary arterial pressure, SD standard deviation, SSc systemic sclerosis, 6MWD 6-minute walking distance, WHO FC World Health Organization functional class, NT-proBNP N-terminal of the prohormone brain natriuretic peptide, n.s. not significant, FEV1 forced expiratory volume in 1 second, RV right ventricle, TAPSE tricuspid annular plane systolic excursion, RA right atrium, TRV tricuspid regurgitation velocity, PASP systolic pulmonary arterial pressure, RAP right atrial pressure, CVP central venous pressure, PAWP pulmonary arterial wedge pressure, PVR pulmonary vascular resistance
P values are based on two-sided, unpaired Student’s t tests and Mann-Whitney U test for Borg Dyspnoea Scale score. For Borg Dyspnoea Scale score, median values equal means. Values are mean±SD. For the category of NT-proBNP, values were missing for 14 patients in the mPAP < 25 mm Hg and eight patients in the mPAP ≥ 25 mm Hg group. For the category of RV thickness, values were missing for seven patients for each group. For the categories of RA area, RV area, and TRV, values for one patient were missing in the mPAP < 25 mm Hg group. For the category of PASP, values were missing for three patients in the mPAP < 25 mm Hg group. For the category of PASP max (exercise), values were missing for three patients in the mPAP < 25 mm Hg group. For the categories of cardiac index, mPAP max, PWAP max, cardiac output max, and cardiac index max, values for one patient was missing for each group, respectively