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Table 1 Characteristics of the 126 SSc patients with mPAP > 20 mmHg

From: Mild pulmonary hemodynamic alterations in patients with systemic sclerosis: relevance of the new 2022 ESC/ERS definition of pulmonary hypertension and impact on mortality

 

N

Whole population

(n = 126)

PVR

[0—2 WU]

(n = 12)

PVR

[2—3 WU]

(n = 18)

PVR

[3—6 WU]

(n = 50)

PVR

[6—20 WU]

(n = 46)

p

SSc characteristics

     

Age (yo)

Gender (female)

SSc subtype (lcSSc)

ACA

ATA

Time since SSc diagnosis (y)

Time since RP onset (y)

Time since FEFRPS onset (y)

ILD

NYHA

I—II

III-IV

6MWD (m)

BNP (ng/L)

126

126

116

107

111

120

98

98

115

101

101

87

75

66.5 ± 16.6

104 (82.5)

95 (81.9)

60 (56.1)

24 (21.6)

7 ± 12

14 ± 15.8

9.5 ± 12.8

54 (47)

41 (40.6)

60 (59.4)

321 ± 173.5

37 ± 36.5

61.2 ± 8

9 (75)

8 (66.7)

3 (27.3)

5 (41.7)

9 ± 11.3

9 ± 10

9 ± 9

8 (66.7)

5 (55.6)

4 (44.4)

310.5 ± 81.8

40 ± 12.5

63.6 ± 18.5

18 (88.9)

16 (100)

12 (70.6)

4 (23.5)

6 ± 9.5

17 ± 12.8

10.5 ± 7.8

5 (31.3)

11 (91.7)

1 (8.3)

388 ± 46.5

53.5 ± 30.8

68.8 ± 13.8

38 (76)

31 (70.5)

21 (55.3)

10 (23.8)

7 ± 13

16 ± 18.8

9 ± 14

23 (50)

19 (47.5)

21 (52.5)

348 ± 116.5

27 ± 27.8

67.1 ± 17.1

41 (89.1)

40 (90.9)

24 (58.5)

5 (12.5)

7 ± 12.5

10 ± 17.8

10 ± 12.5

18 (43.9)

6 (15)

34 (85)

240 ± 139

40 ± 35

0.33

0.27

0.005

0.16

0.17

0.67

0.26

1

0.3

 < 0.001

 < 0.001

 < 0.001

0.23

RHC parameters

     

mPAP (mmHg)

PVR (WU)

TPR (WU)

RAP (mmHg)

sPAP (mmHg)

dPAP (mmHg)

PAWP (mmHg)

CO (L/min)

CI (L/min/m2)

SvO2 (%)

126

126

125

121

124

124

126

125

126

82

34.5 ± 16.8

4.9 ± 4.7

7.3 ± 4.9

7 ± 4

54 ± 29.3

19 ± 12.3

10 ± 6

4.7 ± 1.6

2.8 ± 1

68 ± 13.4

26 ± 8.3

1.6 ± 0.2

4.4 ± 3

8 ± 3.5

39 ± 11.5

13 ± 7.3

15 ± 9.8

5.5 ± 1.5

3.1 ± 1.1

70 ± 11.8

26 ± 5.5

2.4 ± 0.4

4.2 ± 1.7

6 ± 4

41 ± 7.5

13 ± 5.8

12 ± 8

6.1 ± 2.3

3.3 ± 0.7

74 ± 3

32.5 ± 8.8

4.4 ± 1.6

6.5 ± 1.7

6 ± 4

50 ± 17

17 ± 7

10 ± 5.8

4.8 ± 1.4

2.8 ± 0.8

68.5 ± 10.8

47.5 ± 11

9.2 ± 3.4

11.9 ± 3.3

8 ± 7

75 ± 21

28 ± 8

8 ± 4

4.2 ± 1.2

2.4 ± 0.7

59.6 ± 11.5

 < 0.001

 < 0.001

 < 0.001

0.004

 < 0.001

 < 0.001

 < 0.001

 < 0.001

 < 0.001

 < 0.001

Hemodynamic classification

     

Pre-capillary PH

Isolated post-capillary PH

Combined PH

86

12

10

86 (68.3)

12 (9.5)

10 (7.9)

0

6 (50)

0

12 (66.6)

6 (33.3)

0

44 (88)

0

6 (12)

42 (91.3)

0

4 (8.7)

 < 0.001

 < 0.001

 < 0.001

Pulmonary function tests*

     

FVC (% predicted)

FEV1 (% predicted)

TLC (% predicted)

DLCO (% predicted)

KCO (% predicted)

95

95

92

91

80

88 ± 23.5

80.9 ± 22.8

84.1 ± 18

41.8 ± 15.8

41.5 ± 12.8

86.9 ± 27

79 ± 23.3

85.4 ± 25

59.1 ± 21.1

55 ± 13.8

91.6 ± 19.8

85.6 ± 17.4

84.1 ± 15.9

53.9 ± 14.9

54 ± 10.5

90.2 ± 25.8

85.3 ± 27.2

83.1 ± 18.9

38.5 ± 14.3

38.9 ± 11.1

84.2 ± 21.4

74.9 ± 18.1

84.9 ± 16.3

38.7 ± 13.2

36.6 ± 10.5

0.66

0.21

0.97

 < 0.001

 < 0.001

Echocardiography

     

TRV (m/s)

RV/LV ratio

PAAT (m/s)

RA area (cm2)

Expiration IVC diam. (mm)

TAPSE (mm)

LVEF (%)

LA area (cm2)

E/A ratio

84

69

87

94

80

93

101

87

79

3.4 ± 0.8

1 ± 0.4

75 ± 28

18 ± 9.1

15.2 ± 5.8

20.8 ± 5.9

65 ± 5

19.5 ± 8.1

0.8 ± 0.4

3 ± 0.6

0.8 ± 0.2

110 ± 32

16.5 ± 5.6

17.3 ± 5.4

21 ± 7.2

50 ± 15

21.6 ± 8.9

0.9 ± 0.5

3 ± 0.5

0.8 ± 0.2

86.5 ± 38.3

18 ± 4.5

13.5 ± 3

24 ± 6.6

65 ± 5

20.8 ± 5.5

1 ± 0.4

3.4 ± 0.6

0.9 ± 0.2

76 ± 24.5

16.7 ± 8.8

14.9 ± 4.4

21.5 ± 4.7

65 ± 10

19.6 ± 8.5

0.8 ± 0.3

4.1 ± 0.4

1.3 ± 0.2

64 ± 25.5

22.6 ± 9

18.8 ± 4.8

17.7 ± 5.4

65 ± 5

17.1 ± 5

0.7 ± 0.3

 < 0.001

 < 0.001

0.002

0.014

 < 0.001

 < 0.001

0.07

0.04

0.004

Therapeutic strategy *

     

Initial

No treatment

Monotherapy

Dual therapy

Triple therapy

Last follow-up

No treatment

Monotherapy

Dual therapy

Triple therapy

126

126

49 (38.9)

51 (40.5)

22 (17.5)

4 (3.2)

36 (28.6)

34 (27)

34 (27)

22 (17.5)

10 (83.3)

2 (16.7)

0

0

8 (66.7)

3 (25)

0

1 (8.3)

15 (83.3)

3 (16.7)

0

0

9 (50)

8 (44.4)

1 (5.6)

0

22 (44)

23 (46)

5 (10)

0

17 (34)

15 (30)

13 (26)

5 (10)

2 (4.4)

23 (50)

17 (37)

4 (8.7)

2 (4.4)

8 (17.4)

20 (43.5)

16 (34.8)

0.001

0.001

  1. 6MWD: 6-min walking distance, ACA: anti-centromere antibodies, ATA: anti-topoisomerase antibodies, BNP: brain natriuretic peptide, CI: cardiac index, CO: cardiac output, diam: diameter, DLCO: diffusing capacity of the lung for carbon monoxide, dPAP: diastolic pulmonary arterial pressure, FEFRPS: first except for RP symptom, FEV1: forced expired volume at 1st minute, FVC: forced vital capacity, ILD: interstitial lung disease, IVC: inferior vena cava, KCO: carbon monoxide transfer coefficient, LA: left atrial, lc-SSc: limited cutaneous systemic sclerosis, LVEF: left ventricular ejection fraction, mPAP: mean pulmonary arterial pressure, NYHA: New York Heart Association, PAAT: pulmonary artery acceleration time, PAWP: pulmonary arterial wedge pressure, PVR: pulmonary vascular resistance, RA: right atrium, RAP: right atrial pressure, RHC: right heart catheterization, RP: Raynaud phenomenon, RV/LV: right ventricle / left ventricle, sPAP: systolic pulmonary arterial pressure, SSc: systemic sclerosis, SvO2: venous saturation in oxygen, TAPSE: tricuspid annular plane systolic excursion, TLC: total lung capacity, TPR: total pulmonary resistance, TRV: tricuspid regurgitation velocity, y: year, yo: years old
  2. Data were expressed as median ± IQR, except for parameters notified with an asterisk * (mean ± SD)
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Respiratory Research

ISSN: 1465-993X

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