Table 1.
Baseline characteristics of patients with pulmonary arterial hypertension associated with congenital heart disease after defect correctiona
| Characteristic | Corrected CHD‐PAH population | Non‐CHD population (n = 1046) | ||
|---|---|---|---|---|
| Placebo (n = 50) | Selexipag (n = 60) | Overall (n = 110) | ||
| Female sex, n (%) | 42 (84.0) | 46 (76.7) | 88 (80.0) | 835 (79.8) |
| Age years, mean ± SD | 40.3 ± 14.8 | 40.2 ± 15.4 | 40.3 ± 15.1 | 48.9 ± 15.2 |
| Geographic region, n (%) | ||||
| Asia | 17 (34.0) | 15 (25.0) | 32 (29.1) | 196 (18.7) |
| Eastern Europe | 21 (42.0) | 24 (40.0) | 45 (40.9) | 259 (24.8) |
| Latin America | 4 (8.0) | 8 (13.3) | 12 (10.9) | 98 (9.4) |
| North America | 2 (4.0) | 8 (13.3) | 10 (9.1) | 183 (17.5) |
| Western Europe/Australia | 6 (12.0) | 5 (8.3) | 11 (10.0) | 310 (29.6) |
| Time since diagnosis of PAHb, years, mean ± SD | 3.5 ± 5.5 | 3.6 ± 6.1 | 3.6 ± 5.8 | 2.3 ± 3.3 |
| WHO FC, n (%) | ||||
| I | – | 1 (1.7) | 1 (0.9) | 8 (0.8) |
| II | 28 (56.0) | 38 (63.3) | 66 (60.0) | 463 (44.3) |
| III | 22 (44.0) | 21 (35.0) | 43 (39.1) | 564 (53.9) |
| IV | – | – | – | 11 (1.1) |
| 6MWD, m, mean ± SD | 358.7 ± 72.9 | 366.6 ± 71.4 | 363.0 ± 71.9 | 352.2 ± 80.8 |
| Use of medications for PAH, n (%) | 35 (70.0) | 40 (66.7) | 75 (68.2) | 845 (80.8) |
| None | 15 (30.0) | 20 (33.3) | 35 (31.8) | 201 (19.2) |
| ERA | 7 (14.0) | 11 (18.3) | 18 (16.4) | 152 (14.5) |
| PDE‐5i | 18 (36.0) | 19 (31.7) | 37 (33.6) | 337 (32.2) |
| ERA and PDE‐5i | 10 (20.0) | 10 (16.7) | 20 (18.2) | 356 (34.0) |
| NT‐proBNPc, ng/L, median (Q1, Q3) | 471 (186, 1390) | 286 (99, 752) | 336 (124, 986.5) | 593.5 (196, 1654) |
6MWD, 6‐minute walk distance; CHD, congenital heart disease; ERA, endothelin receptor antagonist; NT‐proBNP, N‐terminal pro‐brain natriuretic peptide; PAH, pulmonary arterial hypertension; PDE‐5i, phosphodiesterase‐5 inhibitor; SD, standard deviation; WHO FC, World Health Organization functional class.
Testing of baseline characteristics showed there were (i) no significant differences (P > 0.05) between placebo and selexipag at baseline in the corrected CHD‐PAH patients with the exception of NT‐proBNP (P < 0.05), and (ii) significant differences (P < 0.05) between the corrected CHD‐PAH and non‐CHD populations with the exception of sex and 6MWD (P > 0.05) [comparisons to placebo were conducted using Fisher's exact test (sex, geographic region, WHO FC and use of medications for PAH), unadjusted analysis of variance (age and time since diagnosis of PAH) and Wilcoxon‐Mann‐Whitney test (6MWD and NT‐proBNP)].
Confirmed by right heart catheterisation.
Includes all patients with a baseline assessment: for the corrected CHD‐PAH population, n = 49 for placebo and n = 59 for selexipag; for the overall non‐CHD population, n = 1034.