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Table 2 Disease characteristics of WHO group 1 PH, WHO group 2 PH, and hyperdynamic PH

From: Diversity of hemodynamic types in connective tissue disease associated pulmonary hypertension: more than a subgroup of pulmonary arterial hypertension

 

WHO group 1 PH

n = 138

WHO group 2 PH

n = 33

Hyperdynamic PH

n = 31

p value

IgG (g/L)

17.1 ± 8.4

18.8 ± 10.9

17.7 ± 7.4

0.735

Hypocomplementemia, No. (%)

40 (31.7)

12 (42.9)

9 (29.0)

0.454

Elevated hsCRP or ESR, No. (%)

70 (56.0)

15 (60.0)

14 (50.0)

0.773

Autoantibodies, No. (%)

 ANA

130 (97.7)

30 (100.0)

30 (96.8)

0.782

 Anti-dsDNA

35 (25.4)

5 (15.2)

7 (22.6)

0.268

 Anti-Sm

25 (18.1)

1 (3.0)

5 (16.1)

0.147

 Anti-RNP

76 (55.1)

11 (33.3)

24 (77.4)

0.005

 Anti-SSA

76 (55.1)

22 (66.7)

17 (54.8)

0.414

 Anti-SSB

22 (15.9)

10 (30.3)

5 (16.1)

0.161

 Anti-Scl-70

1 (0.7)

0 (0)

2 (6.5)

0.186

 Anti-Ro-52

72 (52.2)

17 (51.5)

12 (38.7)

0.554

 Anti-β2GP1

10 (7.2)

1 (3.0)

3 (9.7)

0.391

 ACL

3 (2.2)

2 (6.1)

1 (3.2)

0.505

 Elevated LA

3 (2.2)

1 (3.0)

2 (6.5)

0.181

BNP, ng/L

137.0 ± 275.0

221.4 ± 322.5

36.2 ± 45.8

0.047

NT-proBNP, pg/ml

1236.2 ± 2777.8

1290.0 ± 1882.2

112.4 ± 118.4

 < 0.001

WHO function class III–IV, No. (%)

23 (16.7)

5 (15.2)

0 (0)

0.028

Hemodynamics

 mABP, mmHg

90 ± 10

90 ± 13

92 ± 9

0.447

 mPAP, mmHg

45 ± 11

49 ± 11

25 ± 4

 < 0.001

 PAWP, mmHg

10 ± 3

19 ± 3

12 ± 2

 < 0.001

 RAP, mmHg

7 ± 3

12 ± 4

7 ± 2

 < 0.001

 CO, L/min

5.2 ± 1.6

5.3 ± 1.6

6.8 ± 1.3

 < 0.001

 CI, L/min × m2

3.3 ± 0.8

3.4 ± 1.1

4.2 ± 0.8

 < 0.001

 PVR, WU

7.5 ± 3.8

6.7 ± 4.4

1.9 ± 0.6

 < 0.001

Echocardiography

 IVC diameter, mm

14.2 ± 3.0

14.6 ± 2.0

13.0 ± 2.0

0.041

 RV diameter, mm

27.7 ± 7.2

27.9 ± 6.0

21.8 ± 4.4

 < 0.001

 RV/LVEDD ratio

0.70 ± 0.27

0.64 ± 0.16

0.47 ± 0.10

 < 0.001

 LVEF %

69.2 ± 6.2

65.7 ± 8.6

67.0 ± 7.6

0.110

 TAPSE, mm

17.2 ± 3.5

18.1 ± 3.6

19.0 ± 3.7

0.456

 Mitral valve regurgitation, No. (%)

14 (13.0)

9 (40.9)

5 (17.9)

0.028

 Pericardial effusion, No. (%)

36 (33.6)

10 (34.5)

4 (13.8)

0.104

  1. p-values <0.05 are shown in bold, which means statistically significant
  2. WHO World Health Organization, PH Pulmonary hypertension, IgG Immunoglobulin G, hsCRP Hypersensitive C-reaction protein, ESR Erythrocyte dissemination rate, ANA Anti-nuclear antibodies, anti-dsDNA anti-double-stranded DNA, anti-Sm Anti-Smith, ACL Anticardiolipin, Anti-β2GP1 Anti-beta2 glycoprotein 1, anti‐RNP Antiribonucleoprotein, BNP Brain natriuretic peptide, NT-proBNP N-terminal brain natriuretic peptide, mABP Mean arterial blood pressure, mPAP Mean pulmonary pressure, PAWP Pulmonary arterial wedge pressure, RAP Right atrium pressure, CO Cardiac output, CI Cardiac index, PVR Pulmonary arterial resistance, IVC Inferior vena cava, RV Right ventricle, LVEDD Left ventricular end-diastolic diameter, LVEF Left ventricular ejection fraction, TAPSE Tricuspid annular plane systolic excursion
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BMC Pulmonary Medicine

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