Introduction
Methods
Study population and design
Transthoracic Doppler echocardiography at rest
Stress Doppler echocardiography
Right heart catheter
Cutoff values of systolic pulmonary arterial pressures at rest and during mild to moderate exercise
Ethics statement
Statistical methods
Results
Study population
Number of included patients | 76 | ||
---|---|---|---|
Gender, female/male | 64 | / | 12 |
Age, years | 57.9 | ± | 14.4 |
Duration of systemic sclerosis, years | 12.0 | ± | 11.3 |
Body weight, kg | 68.8 | ± | 14.0 |
Body height, cm | 165.5 | ± | 8.1 |
Body surface area, m2
| 1.8 | ± | 0.2 |
WHO functional class | |||
0-I | 17 | 22 % | |
II | 31 | 41 % | |
III | 27 | 36 % | |
6-minute-walking-distance, m | 435 | ± | 94.4 |
Borg Dyspnoea Scale (6-20) | 13.8 | ± | 2.4 |
Haemodynamics by right heart catheter | |||
mPAP, mm Hg | 20.1 | ± | 9.6 |
PVR, dynes*sec per cm5
| 181.4 | ± | 151.9 |
PAWP, mm Hg | 8.8 | ± | 4.9 |
Cardiac output, l/min | 5.4 | ± | 1.2 |
Cardiac index, l/min per m2
| 3.0 | ± | 0.6 |
Diagnosis of manifest pulmonary hypertension by right heart catheterisation
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 |
Comparison between patients without and with pulmonary hypertension
Sensitivity and specificity of echocardiography at rest versus stress Doppler echocardiography during exercise
Approach | False negatives, % (missed diagnoses) | Sensitivity, % | Specificity, % | PPV, % | NPV, % |
---|---|---|---|---|---|
SDE at rest and during exercise | 1 | 96 | 82 | 68 | 98 |
N = 76 | |||||
DETECT algorithm | 4 | 96 | 48 | 35 | 98 |
N = 319 | |||||
DETECT algorithm with | 15 | 85 | 72 | 47 | 94 |
65 % specificity at step 2 | |||||
N = 319 | |||||
DETECT data with algorithm from | |||||
ESC/ERS guidelines* | 29 | 71 | 69 | 40 | 89 |
N = 371 |