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Table 5 Comparison of demographic, clinical and haemodynamic characteristics of patients with PAH diagnosed at first screen and PAH diagnosed at subsequent screens

From: Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme

Characteristic

PAH detected on first screening

PAH detected on subsequent screening

P value

mean ± SD or %

mean ± SD or %

Number of patients

122

38

 

Age at PAH diagnosis

63.9 ± 11.0

62.7 ± 9.5

0.56

Disease duration at PAH diagnosis

13.4 ± 12.8

14.6 ± 8.7

0.62

Female

96 (85.7%)

32 (84.2%)

0.82

Disease subtype

 Limited

81 (75.7%)

23 (62.2%)

0.03

 Diffuse

17 (15.9%)

13 (35.1%)

 MCTD

9 (8.4%)

1 (2.7%)

Status

 Alive

54 (48.2%)

26 (68.4%)

0.03

 Dead

55 (49.1%)

10 (26.3%)

 Withdrawn

3 (2.7%)

1 (2.6%)

 Unable to contact

0 (0%)

1 (2.6%)

Follow up, years

3.3 ± 2.4

5.9 ± 1.9

<0.001

WHO class at PAH diagnosis

 Class I

4 (4.0%)

0 (0%)

0.01

 Class II

16 (16.2%)

15 (40.5%)

 Class III

63 (75.9%)

20 (54.1%)

 Class IV

16 (16.2%)

2 (5.4%)

6MWD at PAH diagnosis

295.9 ± 118.3

340.6 ± 115.6

0.05

mPAP on RHC

38.2 ± 11.4

32.5 ± 8.3

0.005

mRAP on RHC

9.7 ± 8.5

8.4 ± 3.9

0.40

PVR on RHC

5.5 ± 3.1

3.8 ± 1.7

0.005

CI on RHC

2.9 ± 1.6

2.9 ± 0.7

0.87

Pericardial effusion

16 (16.0%)

1 (2.7%)

0.03

  1. Probability of PAH
  2. PAH pulmonary arterial hypertension, MCTD mixed connective tissue disease, WHO World Health Organisation, 6MWD six-minute walk distance, mPAP mean pulmonary arterial pressure, RHC right-heart catheterization, mRAP mean right atrial pressure, PVR pulmonary vascular resistance, CI cardiac index, NT-pro-BNP N-terminal pro b-type natriuretic peptide