Table 1.
Major clinical characteristics of the three main RNA subgroups in the discovery cohort (n = 359) at the time of sampling.
| Low-risk subgroup II (high immunoglobulin) | Intermediate-risk subgroup V (intermediate immunoglobulin) | High-risk subgroup I (low immunoglobulin) | All patients | |
|---|---|---|---|---|
| n | 112 | 89 | 129 | 359 |
| Age, years | 46 [37–56] | 52 [41–62] | 57 [45–70] | 52 [42–64] |
| Age at diagnosis, years | 41 [31–51] | 46 [37–55] | 52 [42–67] | 47 [35–59] |
| Gender:Female | 82 (73%) | 69 (78%) | 80 (62%) | 253 (70%) |
| Vasoresponse | 10 (21.7%) | 6 (13.6%) | 6 (16.2%) | 23 (16.4%) |
| Treatments | ||||
| Phosphodiesterase 5 Inhibitors (PDE5i) | 12 (15.4%) | 16 (21.9%) | 22 (21.8%) | 53 (19.4%) |
| Endothelin receptor antagonist (ERA) | 6 (7.69%) | 13 (17.8%) | 8 (7.92%) | 33 (12.1%) |
| PDE5i & ERA combination | 42 (53.8%) | 30 (41.1%) | 53 (52.5%) | 134 (49.1%) |
| Prostacyclin therapy | 3 (3.85%) | 1 (1.37%) | 3 (2.97%) | 7 (2.56%) |
| Calcium channel blockers | 15 (19.2%) | 13 (17.8%) | 14 (13.9%) | 45 (16.5%) |
| WHO functional class | ||||
| I | 18 (16.5%) | 10 (11.2%) | 6 (4.7%) | 35 (9.8%) |
| II | 45 (41.3%) | 36 (40.4%) | 44 (34.1%) | 143 (40.2%) |
| III | 43 (39.4%) | 40 (44.9%) | 65 (50.4%) | 158 (44.4%) |
| IV | 3 (2.8%) | 3 (3.4%) | 14 (10.9%) | 20 (5.6%) |
| 6-minute walking distance, m | 397 [338–500] | 420 [367–464] | 327 [183–390] | 387 [300–449] |
| NT-proBNP, ng/l | 131.7 [54.5–362.0] | 185.5 [76.3–463.5] | 345.0 [91.0–1556.1] | 222.5 [78.9–1162.8] |
| Forced expiratory volume [% predicted] | 92 [82–101] | 84 [72–98] | 78 [66–98] | 85 [68–100] |
| Forced vital capacity [% predicted] | 101 (20) | 99 (24) | 93 (29) | 97 (24) |
| Transfer factor of lung for carbon monoxide [% predicted] | 93 [87–106] | 97 [92–101] | 88 [67–96] | 94 [87–103] |
| Diagnostic Right Heart Catheter Study | ||||
| Mean pulmonary artery pressure, mmHg | 47 [39–60] | 52 [37–65] | 56 [41–65] | 51 [39–63] |
| Mean right atrial pressure, mmHg | 8 [4–10] | 8 [4–11] | 11 [6–14] | 9 [4–12] |
| Mean pulmonary arterial wedge pressure, mmHg | 10 [7–12] | 10 [8–13] | 12 [10–14] | 11 [8–13] |
| Cardiac Index, l/min/m2 | 2.3 [1.6–2.8] | 2.2 [1.7–2.4] | 1.9 [1.5–2.5] | 2.2 [1.6–2.5] |
| Pulmonary vascular resistance, Wood Units | 8.1 [5.7–14.1] | 15.0 [5.9–16.1] | 8.4 [5.9–13.2] | 8.9 [5.7–15.0] |
Intervals describe first and third quartiles. Parentheses describe standard deviation (SD).