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Table 1 Patient demographics and disease characteristics at baseline

From: Changes in REVEAL risk score in patients with pulmonary arterial hypertension treated with macitentan in clinical practice: results from the PRACMA study

 Incident (n = 33)Prevalent (n = 48)Total (n = 81)
Age, years, mean (SD)57.9 (18.3)56.6 (14.9)57.2 (16.3)
Sex, female24 (72.7)39 (81.3)63 (77.8)
WHO group I subgroup
 Idiopathic or heritable PAH17 (51.5)24 (50.0)41 (50.6)
 PAH associated with connective tissue disorders15 (45.5)14 (29.2)29 (35.8)
 PAH associated with corrected simple congenital heart disease1 (3.0)9 (18.8)10 (12.4)
Time since PAH diagnosis, months, median (Q1-Q3)*1.0 (0.2–2.6)42.8 (14.5–97.7)11.1 (1.8–56.2)
WHO FC
 I1 (3.0)2 (4.2)3 (3.7)
 II5 (15.1)13 (27.1)18 (22.2)
 III21 (63.6)30 (62.5)51 (63.0)
 IV6 (18.2)3 (6.3)9 (11.1)
Renal insufficiency, yes4 (13.3)9 (19.6)13 (17.1)
SBP, mm Hg, mean (SD)117.9 (16.0)122.2 (17.9)120.4 (17.2)
Heart rate, beats/min, mean (SD)79.5 (11.8)77.9 (12.6)78.6 (12.3)
6-min walk test, m, mean (SD)*309.1 (153.2)401.6 (102.9)362.5 (133.7)
BNP, pg/mL, mean (SD)368.7 (433.2)247.5 (188.5)300.2 (316.1)
NT-proBNP, pg/mL mean (SD)1140.9 (1451.3)1452.4 (1995.5)1327.9 (1797.6)
Pericardial effusion, yes4 (12.9)4 (8.9)8 (10.5)
DLCO, % predicted, mean (SD)57.4 (19.3)57.9 (19.4)57.8 (19.2)
mRAP, mm Hg, mean (SD)11.7 (10.7)10.3 (8.1)10.9 (9.3)
PVR, WU, mean (SD)9.2 (4.5)8.8 (4.3)9.0 (4.3)
Therapy for PAH*
 Macitentan monotherapy22 (66.7)12 (25.0)34 (42.0)
 Macitentan + PDE5 inhibitor11 (33.3)25 (52.1)36 (44.4)
 Macitentan + prostacyclin / Macitentan + PDE5 inhibitor + prostacyclin0 (0)11 (22.9)11 (13.6)
  1. Data are number of patients (percentage) except when otherwise indicated
  2. BNP brain natriuretic peptide, DLCO diffusing capacity of the lungs for carbon monoxide, FC functional class, mRAP mean right atrial pressure, NT-proBNP N-terminal pro-BNP, PAH pulmonary arterial hypertension, PDE5 phosphodiesterase type 5, PVR pulmonary vascular resistance, Q1 25th percentile, Q3 75th percentile, SD standard deviation, SBP systolic blood pressure, WHO World Health Organization, WU Wood units
  3. *p < 0.01 between incident and prevalent patients