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Table 2 Baseline characteristics (Study 1; Conventional PH and borderline PH group, including post-capillary PH)

From: Characteristics of patients meeting the new definition of pre-capillary pulmonary hypertension (Nice 2018) in a single Japanese pulmonary hypertension center

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   
  1. mPAP, mean pulmonary arterial pressure; PVR, pulmonary vascular resistance; PAWP, pulmonary arterial wedge pressure; CO, cardiac output; 6MWD, 6-min walk distance; %VC, percent vital capacity; FEV1.0%, percent predicted forced expiratory volume in one second; %DLCO/VA, diffusing capacity of carbon monoxide by the alveolar volume; PaO2, partial pressure of arterial oxygen; PaCO2, partial pressure of arterial carbon dioxide; PvO2, mixed venous oxygen tension; AaDO2, alveolar-arterial oxygen difference; WHO-FC, World Health Organization Functional Class; IPAH, idiopathic pulmonary hypertension; HPAH, hereditary pulmonary hypertension; PVOD, pulmonary veno-occlusive disease; PCH, pulmonary capillary hemangiomatosis; CTD, connective tissue disease; ILD, interstitial lung disease; COPD, chronic obstructive pulmonary disease; BE, bronchiectasis; PEA, pulmonary endarterectomy; PE, pulmonary embolism