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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 diagnosismean ± SD or n(%)p-value
 Age at PAH diagnosis, years63.4 ± 10.4 
 Disease duration at PAH diagnosisa, years14.1 ± 11.6 
WHO Functional Class at diagnosis
 Class I6 (4.5%) 
 Class II32 (2.4%) 
 Class III82 (62.5%) 
 Class IV13 (9.8%) 
Hemodynamic measurements at PAH diagnosis
 6MWD,m329.3 ±109.9 
 mRAP, mmHg7.9 ± 3.8 
 mPAP, mmHg33.9 ± 10.9 
 PAWP, mmHg10.9 ± 4.0 
 mCI, L/min/m22.9 ± 1.3 
 PVR, Wood Units4.7 ± 2.8 
 Pericardial effusion at PAH diagnosis18 (11.8%) 
 Mean DLCO48.9 ± 15.5 
 Mean DLCO/VA, % predicted mL/min/mmHg58.5 ± 19.6 
Severe PAHb101 (66.0%) 
Overall survival4.8 ± 3.00.04
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.