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, female | 24 (72.7) | 39 (81.3) | 63 (77.8) |
WHO group I subgroup | |||
Idiopathic or heritable PAH | 17 (51.5) | 24 (50.0) | 41 (50.6) |
PAH associated with connective tissue disorders | 15 (45.5) | 14 (29.2) | 29 (35.8) |
PAH associated with corrected simple congenital heart disease | 1 (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 | |||
I | 1 (3.0) | 2 (4.2) | 3 (3.7) |
II | 5 (15.1) | 13 (27.1) | 18 (22.2) |
III | 21 (63.6) | 30 (62.5) | 51 (63.0) |
IV | 6 (18.2) | 3 (6.3) | 9 (11.1) |
Renal insufficiency, yes | 4 (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, yes | 4 (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 monotherapy | 22 (66.7) | 12 (25.0) | 34 (42.0) |
Macitentan + PDE5 inhibitor | 11 (33.3) | 25 (52.1) | 36 (44.4) |
Macitentan + prostacyclin / Macitentan + PDE5 inhibitor + prostacyclin | 0 (0) | 11 (22.9) | 11 (13.6) |