Background
Methods
Ethics approval and consent to participate
Patients
Statistical analysis
Results
Conventional PH and borderline PH group (Study 1)
Group | Conventional PH | Borderline PH | Non-PH | Total | ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
n | (%)# | % # | n | (%) # | % # | n | (%) # | % # | n | (%) # | % # | |
1 | 127 | 23.9 | 81.4 | 8 | 16.0 | 5.1 | 21 | 20.6 | 13.5 | 156 | 22.8 | 100 |
2 | 19 | 3.6 | 90.5 | 1 | 2.0 | 4.8 | 1 | 1.0 | 4.8 | 21 | 3.1 | 100 |
3 | 65 | 12.2 | 65.0 | 15 | 30.0 | 15.0 | 20 | 19.6 | 20.0 | 100 | 14.6 | 100 |
4 | 313 | 58.9 | 82.4 | 26 | 52.0 | 6.8 | 41 | 40.2 | 10.8 | 380 | 55.6 | 100 |
5 | 7 | 1.3 | 87.5 | 0 | 0.0 | 0.0 | 1 | 1.0 | 12.5 | 8 | 1.2 | 100 |
Unclassified | 0 | 0.0 | 0.0 | 0 | 0.0 | 0.0 | 18 | 17.6 | 100.0 | 18 | 2.6 | 100 |
All | 531 | 100.0 | 77.7 | 50 | 100.0 | 7.3 | 102 | 100.0 | 14.9 | 683 | 100.0 | 100 |
Total | Conventional | Borderline | Non-PH | p-value | |||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
n | n | n | |||||||||||
Age | 531 | 56.7 | ± | 15.2 | 50 | 56.2 | ± | 15.8 | 102 | 57.8 | ± | 16.1 | 0.7591 |
Sex(F/M) | 531 | 373 | / | 158 | 50 | 29 | / | 21 | 102 | 70 | / | 32 | 0.2152 |
mPAP(mmHg) | 531 | 42.9 | ± | 11.8 | 50 | 22.6 | ± | 1.2 | 102 | 16.2 | ± | 3.2 | < 0.0001 |
PVR (WU) | 531 | 8.7 | ± | 4.7 | 50 | 3.0 | ± | 1.3 | 102 | 2.2 | ± | 0.9 | < 0.0001 |
PAWP(mmHg) | 531 | 8.1 | ± | 3.8 | 50 | 8.0 | ± | 3.9 | 102 | 5.9 | ± | 3.0 | < 0.0001 |
CO(L/min) | 531 | 4.5 | ± | 1.5 | 50 | 5.4 | ± | 1.8 | 102 | 5.1 | ± | 1.4 | < 0.0001 |
6MWD(m) | 413 | 362.0 | ± | 105.3 | 32 | 410.4 | ± | 105.5 | 51 | 429.0 | ± | 112.6 | < 0.0001 |
%VC | 482 | 85.4 | ± | 21.5 | 39 | 88.6 | ± | 26.0 | 86 | 88.9 | ± | 24.3 | 0.3056 |
FEV1.0% | 182 | 75.5 | ± | 11.4 | 39 | 77.6 | ± | 18.5 | 86 | 78.0 | ± | 15.0 | 0.176 |
%DLCO/VA | 455 | 76.5 | ± | 27.4 | 38 | 74.1 | ± | 25.2 | 79 | 85.1 | ± | 31.1 | 0.0297 |
PaO2(mmHg) | 517 | 65.3 | ± | 22.1 | 50 | 74.3 | ± | 13.0 | 99 | 82.5 | ± | 17.6 | < 0.0001 |
PaCO2(mmHg) | 517 | 38.5 | ± | 6.4 | 50 | 40.6 | ± | 7.2 | 99 | 40.2 | ± | 5.3 | 0.0051 |
PVO2(mmHg) | 515 | 34.7 | ± | 4.9 | 50 | 38.1 | ± | 4.8 | 99 | 40.2 | ± | 7.9 | < 0.0001 |
O2 administration( +) | 75(14.1%) | 3(6.0%) | 8(7.8%) | 0.0536 | |||||||||
AaDo2(mmHg) | 516 | 39.0 | ± | 25.0 | 50 | 17.6 | ± | 11.1 | 99 | 19.6 | ± | 16.5 | < 0.0001 |
WHO-FC (I/II/III/IV) | (6/259/255/11) | (0/37/13/0) | (16/62/23/1) | < 0.0001 | |||||||||
Vasodilators( ±) | 531 | 316 | / | 215 | 50 | 8 | / | 42 | 102 | 2 | / | 100 | < 0.0001 |
Group 1 | |||||||||||||
Age | 127 | 48.6 | ± | 18.1 | 8 | 49.5 | ± | 6.2 | 21 | 58.7 | ± | 3.9 | 0.0546 |
Sex(F/M) | 127 | 103 | / | 24 | 8 | 5 | / | 3 | 21 | 20 | / | 1 | 0.0346 |
mPAP(mmHg) | 127 | 44.3 | ± | 12.1 | 8 | 22.8 | ± | 1.3 | 21 | 16.6 | ± | 2.8 | < 0.0001 |
PVR (WU) | 127 | 8.7 | ± | 4.8 | 8 | 3.0 | ± | 1.3 | 21 | 2.2 | ± | 1.1 | < 0.0001 |
PAWP(mmHg) | 127 | 7.5 | ± | 4.5 | 8 | 7.5 | ± | 4.5 | 21 | 5.8 | ± | 3.1 | 0.0337 |
CO(L/min) | 127 | 4.8 | ± | 1.7 | 8 | 6.1 | ± | 3.1 | 21 | 5.3 | ± | 1.5 | 0.1076 |
6MWD(m) | 97 | 398.5 | ± | 108.6 | 6 | 434.2 | ± | 61.6 | 14 | 455.2 | ± | 111.2 | 0.152 |
VC,% predicted | 115 | 85.2 | ± | 16.9 | 6 | 89.8 | ± | 9.8 | 19 | 86.1 | ± | 14.9 | 0.7914 |
FEV1.0,% predicted | 115 | 78.9 | ± | 9.8 | 6 | 79.2 | ± | 8.9 | 19 | 82.1 | ± | 10.1 | 0.432 |
DLCO/VA,% predicted | 111 | 74.7 | ± | 25.7 | 6 | 76.3 | ± | 16.5 | 18 | 81.2 | ± | 39.3 | 0.659 |
PaO2(mmHg) | 125 | 72.6 | ± | 18.9 | 8 | 75.9 | ± | 13.8 | 21 | 87.7 | ± | 15.8 | 0.0018 |
PaCO2(mmHg) | 125 | 36.6 | ± | 5.3 | 8 | 39.6 | ± | 1.2 | 21 | 40.7 | ± | 6.6 | 0.0037 |
PVO2(mmHg) | 123 | 37.7 | ± | 5.1 | 8 | 41.8 | ± | 8.2 | 21 | 42.3 | ± | 5.5 | 0.0005 |
O2 administration( +) | 18(14.2%) | 0(0.0%) | 0(0.0%) | 0.019 | |||||||||
AaDo2(mmHg) | 125 | 33.8 | ± | 19.3 | 8 | 27.1 | ± | 13.8 | 21 | 14.0 | ± | 12.4 | < 0.0001 |
WHO-FC (I/II/III/IV) | (3/83/40/1) | (0/7/1/0) | (1/18/2/0) | 0.2859 | |||||||||
Vasodilators( ±) | 127 | 95 | 94 | 33 | 8 | 4 | / | 4 | 21 | 1 | / | 20 | < 0.0001 |
Underlying diseases | (%) | (%) | (%) | ||||||||||
IPAH/HPAH/PVOD/PCH | 57 | 44.9 | - | - | - | - | |||||||
CTD | 39 | 30.7 | 4 | 50.0 | 8 | 38.1 | |||||||
Congenital | 19 | 15.0 | 2 | 25.0 | 3 | 14.3 | |||||||
Portal hypertension | 12 | 9.4 | 1 | 12.5 | 1 | 4.8 | |||||||
drug/HIV | 2 | 1.6 | 0 | 0.0 | 0 | 0.0 | |||||||
unknown | – | – | 1 | 12.5 | 9 | 42.9 | |||||||
Group 3 | |||||||||||||
Age | 65 | 61.5 | ± | 13.4 | 15 | 59.7 | ± | 18.7 | 20 | 60.1 | ± | 13.8 | 0.8723 |
Sex(F/M) | 65 | 33 | / | 32 | 15 | 6 | / | 9 | 20 | 5 | / | 15 | 0.0321 |
mPAP(mmHg) | 65 | 35.7 | ± | 10.8 | 15 | 22.4 | ± | 1.4 | 20 | 16.0 | ± | 3.4 | < 0.0001 |
PVR (WU) | 65 | 7.0 | ± | 5.2 | 15 | 3.3 | ± | 1.2 | 20 | 2.5 | ± | 0.9 | < 0.0001 |
PAWP(mmHg) | 65 | 7.1 | ± | 3.7 | 15 | 7.1 | ± | 3.7 | 20 | 4.8 | ± | 2.6 | 0.0016 |
CO(L/min) | 65 | 4.7 | ± | 1.6 | 15 | 5.2 | ± | 1.8 | 20 | 47.0 | ± | 1.2 | 0.5076 |
6MWD(m) | 38 | 281.5 | ± | 87.2 | 10 | 310.2 | ± | 121.7 | 14 | 376.1 | ± | 99.4 | 0.01 |
VC,% predicted | 58 | 58.7 | ± | 24.0 | 13 | 68.3 | ± | 28.6 | 20 | 71.5 | ± | 26.6 | 0.1134 |
FEV1.0,% predicted | 58 | 73.2 | ± | 18.9 | 13 | 75.4 | ± | 30.9 | 20 | 69.9 | ± | 24.2 | 0.7678 |
DLCO/VA,% predicted | 47 | 43.3 | ± | 29.1 | 12 | 53.3 | ± | 27.4 | 17 | 69.3 | ± | 22.4 | 0.0052 |
PaO2(mmHg) | 59 | 66.1 | ± | 38.0 | 15 | 67.0 | ± | 14.6 | 19 | 81.3 | ± | 18.4 | 0.1991 |
PaCO2(mmHg) | 59 | 46.8 | ± | 10.5 | 15 | 45.6 | ± | 9.9 | 19 | 42.2 | ± | 5.8 | 0.1983 |
PVO2(mmHg) | 59 | 35.3 | ± | 4.4 | 15 | 35.5 | ± | 2.9 | 19 | 38.8 | ± | 5.4 | 0.0134 |
O2 administration( +) | 65 | 1(6.7%) | 18(27.7%) | 3(15.0%) | 0.1096 | ||||||||
AaDo2(mmHg) | 58 | 28.9 | ± | 47.3 | 15 | 29.0 | ± | 13.3 | 19 | 18.7 | ± | 17.7 | 0.5993 |
WHO-FC (I/II/III/IV) | (0/14/49/2) | (0/6/9/0) | (1/4/14/1) | 0.3784 | |||||||||
Vasodilators | 65 | 35 | / | 30 | 15 | 0 | / | 15 | 20 | 0 | / | 20 | < 0.0001 |
Underlying diseases | (%) | (%) | (%) | ||||||||||
IP | 38 | 58.5 | 7 | 46.7 | 8 | 40.0 | |||||||
COPD | 13 | 20.0 | 5 | 33.3 | 7 | 35.0 | |||||||
BE | 8 | 12.3 | 1 | 6.7 | 0 | 0.0 | |||||||
Others | 6 | 9.2 | 2 | 13.3 | 1 | 5.0 | |||||||
Group 4 | |||||||||||||
Age | 313 | 58.3 | ± | 13.1 | 26 | 55.5 | ± | 14.4 | 41 | 57.0 | ± | 17.4 | 0.5267 |
Sex(F/M) | 313 | 223 | / | 90 | 26 | 18 | / | 8 | 41 | 29 | / | 12 | 0.9757 |
mPAP (mmHg) | 313 | 44.3 | ± | 11.4 | 26 | 22.7 | ± | 1.2 | 41 | 16.5 | ± | 3.0 | < 0.0001 |
PVR (WU) | 313 | 9.4 | ± | 4.5 | 26 | 3.0 | ± | 1.3 | 41 | 2.2 | ± | 0.8 | < 0.0001 |
PAWP (mmHg) | 313 | 7.6 | ± | 3.2 | 26 | 8.3 | ± | 3.5 | 41 | 5.9 | ± | 2.7 | 0.0018 |
CO (L/min) | 313 | 4.3 | ± | 1.1 | 26 | 5.2 | ± | 1.3 | 41 | 5.0 | ± | 1.1 | < 0.0001 |
6MWD (m) | 264 | 359.4 | ± | 100.2 | 15 | 465.7 | ± | 54.8 | 15 | 451.5 | ± | 119.5 | < 0.0002 |
VC,% predicted | 290 | 91.8 | ± | 18.0 | 19 | 103.4 | ± | 17.1 | 31 | 101.3 | ± | 21.1 | 0.0012 |
FEV1.0,% predicted | 290 | 75.0 | ± | 9.4 | 19 | 79.0 | ± | 7.8 | 31 | 78.2 | ± | 9.1 | 0.0631 |
DLCO/VA,% predicted | 278 | 82.5 | ± | 23.8 | 19 | 85.2 | ± | 18.0 | 30 | 96.9 | ± | 19.2 | 0.0054 |
PaO2 (mmHg) | 308 | 60.9 | ± | 18.0 | 26 | 78.2 | ± | 10.3 | 40 | 78.5 | ± | 15.8 | < 0.0001 |
PaCO2 (mmHg) | 308 | 37.6 | ± | 4.4 | 26 | 38.5 | ± | 4.4 | 40 | 39.7 | ± | 4.7 | 0.0114 |
PVO2 (mmHg) | 308 | 33.3 | ± | 4.3 | 26 | 38.7 | ± | 3.4 | 40 | 39.2 | ± | 3.4 | < 0.0001 |
O2 administration(+) | 36(9.5%) | 2(7.7%) | 0(0.0%) | 0.0115 | |||||||||
AaDo2(mmHg) | 308 | 44.4 | ± | 18.9 | 26 | 26.1 | ± | 8.4 | 40 | 24.3 | ± | 14.8 | < 0.0001 |
WHO-FC (I/II/III/IV) | (3/147/156/7) | (0/24/2/0) | (8/30/3/0) | < 0.0001 | |||||||||
Vasodilators | 313 | 177 | / | 136 | 26 | 4 | / | 22 | 41 | 1 | / | 40 | < 0.0001 |
PEA | 313 | 158 | / | 155 | 26 | 2 | / | 24 | 41 | 1 | / | 40 | < 0.0001 |
BPA | 313 | 53 | / | 260 | 26 | 0 | / | 26 | 41 | 0 | / | 41 | < 0.0001 |
Underlying diseases | (%) | (%) | (%) | ||||||||||
PE | 289 | 92.3 | 22 | 84.6 | 35 | 85.4 | |||||||
Pulmonary stenosis | 22 | 7.0 | 4 | 15.4 | 5 | 12.2 | |||||||
Others | 2 | 0.6 | 0 | 0.0 | 1 | 2.4 |
~ 2009 | ~ 2010 | |||
---|---|---|---|---|
n | % | n | % | |
Conventional | 9 | 40.9 | 56 | 71.8 |
Borderline | 4 | 18.2 | 11 | 14.1 |
Non-PH | 9 | 40.9 | 11 | 14.1 |
Total | 22 | 78 |
RHF | Lung disease | Malignancy | Lung transplantation | Others | Total | ||||||
---|---|---|---|---|---|---|---|---|---|---|---|
n | % | n | % | n | % | n | % | n | % | ||
Conventional | 10 | 29.4 | 14 | 41.2 | 1 | 2.9 | 2 | 5.9 | 7 | 20.6 | 34 |
Borderline | 0 | 0.0 | 1 | 50.0 | 0 | 0.0 | 0 | 0.0 | 1 | 50.0 | 2 |
Non-PH | 0 | 0.0 | 3 | 42.9 | 1 | 14.3 | 2 | 28.6 | 1 | 14.3 | 7 |
Characteristics and survival of pre-capillary PH patients (Study 2)
Conventional pre-capillary PH | Borderline pre-capillary PH | Total pre-capillary PH | |||||||
---|---|---|---|---|---|---|---|---|---|
Group | n | (%) # | % # | n | (%) # | % # | n | (%) # | % # |
1 | 123 | 25.2 | 97.6 | 3 | 2.4 | 13.6 | 126 | 24.7 | 100 |
3 | 59 | 12.1 | 86.8 | 9 | 13.2 | 40.9 | 68 | 13.3 | 100 |
4 | 301 | 61.6 | 96.8 | 10 | 3.2 | 45.5 | 311 | 60.9 | 100 |
5 | 6 | 1.2 | 100.0 | 0 | 0.0 | 0.0 | 6 | 1.2 | 100 |
Total | 489 | 100.0 | 95.7 | 22 | 4.3 | 100.0 | 511 | 100.0 | 100 |
Total | N | Conventional pre-capillry PH | n | Borderline pre-capillary PH | p-value | ||||
---|---|---|---|---|---|---|---|---|---|
Age | 489 | 56.1 | ± | 15.2 | 22 | 58.2 | ± | 13.9 | 0.5342 |
Sex(F/M) | 489 | 347 | / | 142 | 22 | 15 | / | 7 | 0.7808 |
mPAP(mmHg) | 489 | 43.7 | ± | 11.7 | 22 | 23.0 | ± | 1.2 | < 0.0001 |
PVR (WU) | 489 | 9.2 | ± | 4.6 | 22 | 4.2 | ± | 0.9 | < 0.0001 |
PAWP(mmHg) | 489 | 7.5 | ± | 3.1 | 22 | 5.7 | ± | 2.9 | 0.0057 |
CO(L/min) | 489 | 4.3 | ± | 1.2 | 22 | 4.2 | ± | 0.9 | 0.5689 |
6MWD(m) | 449 | 360.1 | ± | 104.7 | 12 | 403.2 | ± | 11.3 | 0.1704 |
VC,% predicted | 444 | 86.7 | ± | 21.2 | 17 | 86.0 | ± | 28.6 | 0.8827 |
FEV1.0,% predicted | 444 | 75.8 | ± | 10.8 | 17 | 73.9 | ± | 17.3 | 0.4922 |
DLCO/VA,% predicted | 420 | 75.8 | ± | 27.3 | 16 | 70.7 | ± | 21.8 | 0.4577 |
PaO2(mmHg) | 476 | 64.4 | ± | 21.5 | 22 | 74.6 | ± | 14.2 | 0.0281 |
PaCO2(mmHg) | 476 | 38.2 | ± | 6.1 | 22 | 41.4 | ± | 9.3 | 0.0212 |
PVO2(mmHg) | 475 | 34.4 | ± | 4.7 | 22 | 37.1 | ± | 3.7 | 0.0087 |
O2 administration( +) | 71(14.5%) | 1(4.6%) | 0.1348 | ||||||
AaDo2(mmHg) | 475 | 40.3 | ± | 23.8 | 22 | 26.3 | ± | 10.3 | 0.0063 |
WHO-FC (I/II/III/IV) | (6/231/241/11) | (0/17/5/0) | 0.0344 | ||||||
Vasodilators( ±) | 489 | 301 | / | 188 | 22 | 5 | / | 17 | < 0.0001 |
Group 1 | |||||||||
Age | 123 | 47.9 | ± | 17.8 | 3 | 51.0 | ± | 66.0 | 0.0357 |
Sex(F/M) | 123 | 99 | / | 24 | 3 | 2 | / | 1 | 0.1417 |
mPAP(mmHg) | 123 | 44.8 | ± | 12.0 | 3 | 23.3 | ± | 0.6 | < 0.0001 |
PVR (WU) | 123 | 9.0 | ± | 4.7 | 3 | 4.4 | ± | 0.5 | < 0.0001 |
PAWP(mmHg) | 123 | 7.7 | ± | 3.1 | 3 | 7.0 | ± | 1.7 | 0.0359 |
CO(L/min) | 123 | 4.7 | ± | 1.5 | 3 | 3.8 | ± | 0.7 | 0.1295 |
6MWD(m) | 95 | 398.0 | ± | 107.4 | 3 | 472.0 | ± | 61.5 | 0.2394 |
VC,% predicted | 111 | 85.6 | ± | 16.9 | 3 | 91.4 | ± | 9.5 | 0.8324 |
FEV1.0,% predicted | 111 | 79.1 | ± | 9.4 | 3 | 76.9 | ± | 9.9 | 0.4183 |
DLCO/VA,% predicted | 107 | 73.4 | ± | 23.9 | 3 | 74.4 | ± | 12.8 | 0.5192 |
PaO2(mmHg) | 121 | 72.6 | ± | 17.9 | 3 | 82.3 | ± | 3.6 | 0.0013 |
PaCO2(mmHg) | 121 | 36.6 | ± | 5.3 | 3 | 39.3 | ± | 0.7 | 0.0072 |
PVO2(mmHg) | 120 | 37.3 | ± | 4.8 | 3 | 39.8 | ± | 1.4 | 0.0003 |
O2 administration( +) | 17(13.8%) | 0(0%) | 0.3479 | ||||||
AaDo2(mmHg) | 121 | 33.9 | ± | 19.2 | 3 | 21.0 | ± | 4.4 | < 0.0001 |
WHO-FC (I/II/III/IV) | 123 | (3/79/40/1) | 3 | (0/3/0/0) | 0.4546 | ||||
Vasodilators( ±) | 123 | 92 | / | 31 | 3 | 2 | / | 1 | < 0.0001 |
Underlying diseases | (%) | (%) | |||||||
IPAH/HPAH/PVOD/PCH | 55 | 44.7 | - | - | |||||
CTD | 38 | 30.9 | 1 | 33.3 | |||||
Congenital | 16 | 13.0 | 1 | 33.3 | |||||
Portal hypertension | 12 | 9.8 | 0 | 0.0 | |||||
drug/HIV | 2 | 1.6 | 0 | 0.0 | |||||
unknown | – | 1 | 33.3 | ||||||
Group 3 | |||||||||
Age | 59 | 62.3 | ± | 13.2 | 9 | 63.3 | ± | 13.5 | 0.8404 |
Sex(F/M) | 59 | 28 | / | 31 | 9 | 5 | / | 4 | 0.6506 |
mPAP(mmHg) | 59 | 36.5 | ± | 11.0 | 9 | 22.4 | ± | 1.3 | 0.0003 |
PVR (WU) | 59 | 7.4 | ± | 5.2 | 9 | 4.0 | ± | 0.8 | 0.0546 |
PAWP(mmHg) | 59 | 4.5 | ± | 3.2 | 9 | 5.7 | ± | 3.7 | 0.12 |
CO(L/min) | 59 | 4.4 | ± | 1.2 | 9 | 4.3 | ± | 1.2 | 0.8225 |
6MWD(m) | 36 | 281.2 | ± | 89.5 | 5 | 317.6 | ± | 107.4 | 0.4098 |
VC,% predicted | 52 | 59.8 | ± | 24.2 | 7 | 66.9 | ± | 33.5 | 0.4882 |
FEV1.0,% predicted | 52 | 73.6 | ± | 17.2 | 7 | 68.6 | ± | 24.5 | 0.498 |
DLCO/VA,% predicted | 43 | 42.0 | ± | 30.0 | 6 | 51.9 | ± | 18.7 | 0.4353 |
PaO2(mmHg) | 53 | 64.4 | ± | 35.4 | 9 | 65.8 | ± | 15.6 | 0.9052 |
PaCO2(mmHg) | 53 | 46.2 | ± | 9.6 | 9 | 47.0 | ± | 12.1 | 0.8248 |
PVO2(mmHg) | 53 | 34.9 | ± | 4.0 | 9 | 35.1 | ± | 3.0 | 0.9152 |
O2 administration( +) | 17(28.8%) | 1(11.1%) | 0.2265 | ||||||
AaDo2(mmHg) | 52 | 31.4 | ± | 42.9 | 9 | 28.5 | ± | 13.7 | 0.8458 |
WHO-FC (I/II/III/IV) | (0/11/46/2) | (0/5/4/0) | 0.0671 | ||||||
Vasodilators( ±) | 59 | 35 | / | 24 | 9 | 0 | / | 9 | 0.0001 |
Underlying diseases | (%) | (%) | |||||||
ILD | 36 | 61.0 | 4 | 44.4 | |||||
COPD | 13 | 22.0 | 3 | 33.3 | |||||
BE | 6 | 10.2 | 1 | 11.1 | |||||
Others | 4 | 6.8 | 1 | 11.1 | |||||
Group 4 | |||||||||
Age | 301 | 58.2 | ± | 13.1 | 10 | 55.7 | ± | 15.2 | 0.5475 |
Sex(F/M) | 301 | 216 | / | 85 | 10 | 8 | / | 2 | 0.5551 |
mPAP(mmHg) | 301 | 44.7 | ± | 11.2 | 10 | 23.4 | ± | 0.7 | < 0.0001 |
PVR (WU) | 301 | 9.6 | ± | 4.4 | 10 | 4.3 | ± | 1.1 | 0.0002 |
PAWP(mmHg) | 301 | 7.5 | ± | 3.1 | 10 | 5.3 | ± | 2.4 | 0.0269 |
CO(L/min) | 301 | 4.2 | ± | 1 | 10 | 4.2 | ± | 0.6 | 0.8073 |
6MWD(m) | 254 | 357.8 | ± | 99.4 | 4 | 458.5 | ± | 80.4 | 0.0449 |
VC,% predicted | 278 | 92.2 | ± | 18.1 | 7 | 102.7 | ± | 16 | 0.1311 |
FEV1.0,% predicted | 278 | 75 | ± | 9.3 | 7 | 78 | ± | 10.2 | 0.4101 |
DLCO/VA,% predicted | 267 | 82.3 | ± | 24 | 7 | 85.2 | ± | 15.6 | 0.7473 |
PaO2(mmHg) | 296 | 60.7 | ± | 18.2 | 10 | 80.1 | ± | 11.1 | 0.0009 |
PaCO2(mmHg) | 296 | 37.5 | ± | 4.4 | 10 | 37 | ± | 4.2 | 0.7296 |
PVO2(mmHg) | 296 | 33.1 | ± | 1.2 | 10 | 38.2 | ± | 3.9 | 0.0002 |
O2 administration( +) | 34 (11.3%) | 0(0%) | 0.1248 | ||||||
AaDo2(mmHg) | 296 | 44.8 | ± | 19.1 | 10 | 25.9 | ± | 8.1 | 0.002 |
WHO-FC (I/II/III/IV) | (3/138/153/7) | (0/9/1/0) | 0.0345 | ||||||
Vasodilators( ±) | 301 | 168 | / | 133 | 10 | 3 | / | 7 | 0.1049 |
PEA | 301 | 156 | / | 145 | 10 | 0 | / | 14 | 0.0002 |
BPA | 301 | 52 | / | 249 | 10 | 0 | / | 14 | 0.0536 |
Underlying diseases | (%) | (%) | |||||||
PE | 279 | 92.7 | 9 | 90.0 | |||||
Pulmonary stenosis | 20 | 6.6 | 1 | 10.0 | |||||
Others | 2 | 0.7 | 0 | 0.0 |
Factors | Univariate crude hazard ratio (95% CI) | p-value | Multivariate hazard ratio (95% CI) | p-value |
---|---|---|---|---|
Age | 0.973 (0.949–1.000) | 0.052 | 0.992 (0.947–1.000) | 0.051 |
Hemodynaics classification (Conventional pre-capillary/Borderline pre-capillary) | 4.690 (0.623–35.284) | 0.055 | 4.265 (0.547–33.238) | 0.09 |
Time of diagnosis (~ 2010/2010 ~) | 0.846 (0.317–2.259) | 0.073 | 1.216 (0.416–3.551) | 0.716 |
Follow-up RHC data of patients with borderline PH and those with borderline pre-capillary PH
Group | Group of PH | Background disease | Age at diagnosis | RHC at diagnosis | Desition after first RHC | RHC at final follow up | Desition after final RHC | Outcome | ||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
mPAP (mmHg) | PVR (WU) | PCWP (mmHg) | mPAP (mmHg) | PVR (WU) | PCWP (mmHg) | |||||||
Boderline PH | 1 | SSc | 37 | 22 | 2.6 | 5 | Follow up | 37 | 2.6 | 9 | Ambrisentan | Deceased because of lung cancer in 14 month |
Boderline PH | 3 | COPD | 65 | 23 | 2.6 | 5 | Follow up | 35 | 3.7 | 4 | Treatment of COPD | Became feasible because of COPD |
Boderline PH | 3 | SSc + Fibrosis | 35 | 20 | 1.2 | 11 | Follow up | 40 | 2.9 | 16 | Treatment of left heart failure | Deceased because of left heart failure in 11 month |
Boderline PH | 4 | CTEPH | 46 | 24 | 2.4 | 7 | Follow up | 19 | 1.1 | 10 | Follow up | Stable |
Boderline pre-capillary PH | 4 | Aortitis | 61 | 24 | 5.8 | 4 | Follow up | 29 | 7.7 | 1 | Riociguat | Improved after the treatment of riociguat |
Discussion
Country | n | % of borderline pre-capillary PH | % of borderline PH | of total PH patients | Associated conditions | ||
---|---|---|---|---|---|---|---|
of total patients (including Non-PH) | of total pre-capillary PH patients | of total patients (including Non-PH) | Total | ||||
Assad et. al.(2017)[5] | U.S.A | 4343 | – | – | 18.0% | 22.6% | CTD:0.9% CAD:71.5% COPD + ILD:18.7% |
Douschan et.al.(2018)[6] | Austria | 547 | – | – | 11.7% | 18.1% | ※Conventional group only Group1:25.5% Group2:20.3% Group3:26.6% Group4:18.3% Group5:9.3% |
Gustavo et.al. (2013)[7] | the U.S | 1491 | – | – | 4.2% | 4.5% | ※Borderline + borerline precapillary only None:30.4% CTD:16.2% Heart disease:16.2% Respiratory disease:29.7 |
Umit et.al. (2019)[9] | Turkey | 58 | 12.1% | 14.0% | Unknown | Unknown | IPAH suspected:43.1% Congenital PH suspected:34.5% Systemic sclerosis: 3.4% Left heart disease and valvular disease:6.9% |
Our study | Japan | 683 | 3.2% | 4.3% | 7.3% | 8.6% | Group1:22.8% Group2:3.1% Group3:14.6% Group4:55.6% Group5:1.2% |