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Table 2 Characteristics at PAH diagnosis

From: The economic burden of systemic sclerosis related pulmonary arterial hypertension in Australia

Patient characteristics at PAH diagnosis

mean ± SD or n(%)

p-value

Demographics

 Age at PAH diagnosis, years

63.4 ± 10.4

 

 Disease duration at PAH diagnosisa, years

14.1 ± 11.6

 

WHO Functional Class at diagnosis

 Class I

6 (4.5%)

 

 Class II

32 (2.4%)

 

 Class III

82 (62.5%)

 

 Class IV

13 (9.8%)

 

Hemodynamic measurements at PAH diagnosis

 6MWD,m

329.3 ±109.9

 

 mRAP, mmHg

7.9 ± 3.8

 

 mPAP, mmHg

33.9 ± 10.9

 

 PAWP, mmHg

10.9 ± 4.0

 

 mCI, L/min/m2

2.9 ± 1.3

 

 PVR, Wood Units

4.7 ± 2.8

 

 Pericardial effusion at PAH diagnosis

18 (11.8%)

 

 Mean DLCO

48.9 ± 15.5

 

 Mean DLCO/VA, % predicted mL/min/mmHg

58.5 ± 19.6

 

Severe PAHb

101 (66.0%)

 

Overall survival

4.8 ± 3.0

0.04

Time to death from PAH diagnosis, years(n=77)

4.3 ± 2.7

Survival from PAH diagnosis, years (n=68)

5.3 ± 3.3

  1. Abbreviations: pulmonary arterial hypertension (PAH), six minute walk distance (6MWD), mean pulmonary arterial pressure (mPAP), mean right atrial pressure (mRAP), pulmonary vascular resistance (PVR), cardiac index (CI), pulmonary arterial wedge pressure (PAWP), diffusing capacity of the lungs for carbon monoxide (DLCO), DLCO divided by the alveolar volume (DLCO/VA), world health organization (WHO)
  2. adisease duration defined as from first non-Raynaud’s disease manifestation,
  3. bsevere PAH defined by the presence of WHO Functional Class IV, presence of a pericardial effusion, 6MWD <300m, right atrial pressure on right heart catheter of >15 and cardiac index of <2.