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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