Table 1 Major clinical characteristics of the three main RNA subgroups in the discovery cohort (n = 359) at the time of sampling.

From: Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood

 

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]

  1. Intervals describe first and third quartiles. Parentheses describe standard deviation (SD).
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