Table 1.
Baseline clinical characteristics of IPF patients stratified by PH severity
| All | Severe PH | Less severe PH | ERPE |
P value of Severe vs Less severe PH, Less severe PH vs ERPE, Severe PH vs ERPE |
|
|---|---|---|---|---|---|
| No. (male/female) | 49(36/13) | 4(0/4) | 21(19/2) | 24(17/7) |
0.0001* 0.14 0.0072* |
| Age (y.o.) | 69.27± 6.89 | 73.00± 5.83 | 68.62± 7.74 | 69.21± 6.30 |
0.37 0.95 0.37 |
| Height (cm) | 161.09±10.17 | 151.25±14.68 | 163.76±8.95 | 160.50±9.67 |
0.081 0.19 0.21 |
| Weight (kg) | 60.08±12.25 | 49.23±11.50 | 62.18±13.78 | 60.15±10.39 |
0.12 0.84 0.13 |
| ADL including exercise tolerance test | |||||
| mMRC grade 0/1/2/3/4 (n) | 0/17/10/14/8 | 0/0/0/0/4 | 0/5/4/9/3 | 0/12/6/5/1 |
0.0066* 0.15 <0.0001* |
| 6min walk | 310.28 | 131.24 | 290.81 | 358.36 | 0.0812 |
| distance (m) | ±139.56 | ±177.12 | ±118.18 | ±125.68 |
0.077 0.041* |
| Treadmil exercise test (METs) | 4.40±2.44 | 1.18±0.21 | 3.85±2.02 | 5.39±2.38 |
0.0019* 0.018* 0.0014* |
| Right heart catheter | |||||
| mPAP (mmHg) | 21.00±8.64 | 39.25±4.79 | 25.43±3.75 | 14.08±3.84 |
0.0018* <0.0001* 0.0015* |
| mPAWP (mmHg) | 6.69±3.76 | 10.00±4.55 | 7.65±3.67 | 5.33±3.23 |
0.31 0.047* 0.051 |
| mRVP (mmHg) | 12.51±4.69 | 14.75±8.26 | 14.38±4.79 | 10.50±2.96 |
0.50 0.0019* 0.37 |
| mRAP (mmHg) | 3.27±2.63 | 4.50±5.74 | 3.38±2.29 | 2.96±2.27 |
0.97 0.56 0.92 |
| CO (L/min) | 5.15±1.65 | 3.60±0.27 | 5.79±2.06 | 4.84±1.05 |
0.010* 0.14 0.0085* |
| CI (L/min/m2) | 3.27±1.40 | 2.56±0.35 | 3.75±1.93 | 2.96±0.65 |
0.049* 0.056 0.29 |
| PVR (wood) | 3.13±2.15 | 8.18±1.86 | 3.54±1.67 | 1.92±0.84 |
0.0038* 0.0008* 0.0016* |
| PVRI (wood) | 4.95±3.11 | 11.52±2.71 | 5.80±2.56 | 3.11±1.32 |
0.0076* 0.0002* 0.0016* |
| Pulmonary function test | |||||
| %VC (%) | 77.43±22.29 | 67.93±24.65 | 70.85±20.12 | 84.67±22.52 |
0.76 0.053 0.28 |
| FVC (L) | 2.34±0.81 | 1.79±1.34 | 2.28±0.69 | 2.47±0.84 |
0.32 0.44 0.24 |
| %DLco(%) | 42.36±27.36 | 15.57±6.93 | 32.92±24.50 | 54.06±26.19 |
0.17 0.0032* 0.0091* |
| ECG | |||||
| ET (msec) | 286.05±44.85 | 234.00±27.46 | 283.68±31.24 | 296.71±51.10 |
0.016* 0.46 0.018* |
| PAAcT (msec) | 100.54±26.87 | 99.00±61.48 | 94.65±16.39 | 105.71±26.49 |
0.29 0.18 0.26 |
| ICT (msec) | 14.41±18.84 | 8.75±10.31 | 14.25±21.33 | 15.48±18.12 |
0.78 0.37 0.49 |
| IRT (msec) | 55.82±47.42 | 76.25±72.41 | 59.83±45.50 | 49.08±45.47 |
0.94 0.35 0.43 |
| ICT+IRT (msec) | 72.37±57.57 | 85.00±70.24 | 83.66±56.05 | 60.84±57.08 |
0.94 0.11 0.54 |
| TEI index | 0.28±0.25 | 0.38±0.31 | 0.30±0.24 | 0.24±0.26 |
0.70 0.20 0.49 |
| TAPSE(cm) | 2.41±1.22 | 3.47±4.29 | 2.39±0.52 | 2.24±0.43 |
0.14 0.33 0.13 |
| Aortic Blood data at rest | |||||
| pH | 7.42±0.024 | 7.42±0.027 | 7.42±0.022 | 7.42±0.027 |
0.94 0.61 0.81 |
| PaO2 (mmHg) | 80.51±15.68 | 65.00±25.15 | 76.79±10.57 | 86.72±15.36 |
0.39 0.017* 0.12 |
| Aortic oxygen saturation (%) | 95.50±1.85 | 95.00±2.59 | 94.95±1.95 | 96.07±1.52 |
0.87 0.056 0.39 |
| NT-proBNP (pg/ml) | 361.57±1319.93 | 3040.75±3786.86 | 124.47±117.45 | 65.76±37.43 |
0.012* 0.16 0.0018* |
Data are presented as mean ± standard deviation or number (%)
PH Pulmonary hypertension, PAP Pulmonary artery pressure, PV Pulmonary vascular resistance, mPAP mean PAP, ERPE Exercise-Responsive PAP Elevation, mPAWP mean pulmonary artery wedge pressure, mRVP mean right ventricular pressure, mRAP mean right atrial pressure, CO Cardiac output, CI Cardiac index, ADL Activities of Daily Living, TMET Treadmill exercise tests, MET Maximum exercise tolerance, PaO2 Partial pressure of oxygen in arterial blood, ECG Echocardiography, ET Right ventricular ejection time, ICT Right ventricular isovolumetric contraction time, IRT Right ventricular isovolumetric relaxation time, TEI index right ventricular total ejection isovolumetric index, TAPSE Tricuspid annular plane systolic excursion, FVC Forced vital capacity, %VC Percent vital capacity/predicted vital capacity, %DLco Percent diffusing capacity for carbon monoxide / predicted, mMRC modified Medical Research Council
*P value for Wilcoxon signed-rank test to assess the difference between patients in the severe vs. less severe PH group (upper row), less severe vs. the ERPE group (middle row), and severe vs. the ERPE group (lower row)