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Table 1 Demographic and patient characteristics

From: Plasma markers in pulmonary hypertension subgroups correlate with patient survival

 

IPAH

(n = 16)

CTD-PAH

(n = 24)

CHD-PAH

(n = 10)

CTEPH

(n = 37)

HC

(n = 18)

p value

Baseline clinical characteristics

      

 Gender, female (%)

12 (75%)

21 (88%)

4 (40%)

20 (54%)

9 (50%)

 

 Age, y

54.3 ± 17.2

63.6 ± 11.8

41.0 ± 17.7

61.4 ± 14.2

31.6 ± 9.9

 < 0.0001

 BMI, kg/m2

27.7 ± 8.0

27.1 ± 4.4

23.7 ± 4.8

28.8 ± 6.0

 

0.16

 NYHA class 3–4, n (%)

12 (75%)

15 (63%)

3 (30%)

17 (46%)

  

 6MWT, m

350 ± 135

333 ± 122

426 ± 173

379 ± 129

 

0.35

 NT-pro BNP, pmol/L

317 ± 467

519 ± 1037

65 ± 88

127 ± 199

 

0.07

 Underlying CTD

      

  SSc, n (%)

 

20/24 (83%)

    

  SLE, n (%)

 

4/24 (17%)

    

Baseline right heart catheterization

      

 mPAP, mmHg

58.9 ± 16.5

41.5 ± 12.5

43.11 ± 14.9

40.1 ± 12.6

 

0.0001

 mRAP, mmHg

11.9 ± 6.7

10.4 ± 6.0

10.8 ± 6.2

9.6 ± 7.1

 

0.72

 Capillary wedge pressure, mmHg

9.2 ± 4.2

12.9 ± 8.1

14.5 ± 6.6

12.3 ± 4.5

 

0.21

 PVR, wood units

10.6 ± 3.9

6.0 ± 3.5

4.3 ± 2.9

5.3 ± 3.4

 

0.0002

PH-Medication

      

 At baseline, n (%)

0/16 (0%)

0/24 (0%)

0/10 (0%)

0/37 (0%)

  

 At 1 year follow up

      

  No PH-medication

0/13 (0%)

0/11 (0%)

2/6 (33%)3

3/19 (16%)5

  

  Mono therapy, n (%)

1/13 (8%)1

1/11 (9%)2

1/6 (17%)4

11/19 (58%)

  

  Duo therapy, n (%)

6/13 (46%)

9/11 (82%)

3/6 (50%)

5/19 (26%)

  

  Triple therapy, n (%)

6/13 (46%)

1/11 (9%)

0/6 (0%)

0/19 (0%)

  

Immunomodulatory drugs

      

 At baseline, n (%)

0/16 (0%)

3/24 (13%)

0/10 (0%)

0/37 (0%)

  

 At 1 year follow up, n (%)

0/13 (0%)

3/11 (27%)

0/6 (0%)

0/19 (0%)

  

Survival

      

 Death/lung transplant < 3 years

2 (12.5%)

8 (33.3%)

0 (0%)

6 (16.2%)

  

 Death/lung transplant > 3 years

2 (12.5%)

0 (0%)

3 (30%)

2 (5.4%)

  
  1. Data given as ‘mean, ± SD’, unless otherwise indicated
  2. BMI body mass index, CTEPH chronic thromboembolic pulmonary hypertension, PAH pulmonary arterial hypertension, IPAH idiopathic pulmonary arterial hypertension, CHD congenital heart disease, CTD connective tissue disease, 6MWT 6-min walk test, NT-pro BNP The N-terminal prohormone of brain natriuretic peptide, SSc systemic sclerosis, , SLE systemic lupus erythematosus, mPAP mean pulmonary arterial pressure, mRAP mean right atrium pressure, PVR pulmonary vascular resistance, RV right ventricle, RA right atrium, RVSP right ventricular systolic pressure
  3. 1This IPAH patient was on ERA monotherapy, due to severe side-effects on PDE5 therapy
  4. 2This CTD-PAH patient was on PDE5 monotherapy due to severe side-effects on ERA therapy
  5. 3These CHD-PAH patients were not started on PAH-medication immediately, since in one patient a possible effect of a surgical correction was awaited, and the other patient was started on medication after one-year since she had persistent PAH
  6. 4This CHD-PAH patient was on PDE5 monotherapy since the PAH was mild
  7. 5These CTEPH patients were not started on PH-medication since they underwent a pulmonary endarterectomy