. 2020 Nov 19;21:304. doi: 10.1186/s12931-020-01566-y

Table 1.

Baseline patient characteristics and biomarker values

	Overall N = 58	On therapy with ERA N = 16	On therapy with PDE5i or sGC^a N = 17	On therapy with ERA + PDE5i^b N = 25
Age (years)	61.1 (46.1, 68.5)	61.6 (51.0, 68.1)	69.1 (61.1, 76.4)	51.2 (37.7, 63.9)
Female	52 (89.7%)	16 (100.0%)	14 (82.4%)	22 (88.0%)
Years since PAH diagnosis	6.7 (2.3, 10.7)	7.5 (2.8, 12.0)	5.9 (1.2, 10.9)	6.8 (2.7, 10.0)
6MWD (m)	427.9 (362.1, 487.4) (N = 54)	413.8 (359.1, 461.0)	427.9 (351.1, 460.9) (N = 15)	457.2 (369.0, 526.7) (N = 23)
mPAP (mmHg)	40.3 (27.0, 49.3) (N = 35)	39.8 (31.8, 48.3) (N = 12)	29.2 (22.0, 37.0) (N = 10)	46.7 (40.3, 50.0) (N = 13)
PVR (WU)	5.1 (3.6, 6.3) (N = 31)	5.1 (3.9, 6.2) (N = 11)	3.6 (2.4, 3.7) (N = 9)	6.0 (5.6, 6.9) (N = 11)
DLCO (% predicted)	65.5 (53.0, 77.0) (N = 30)	53.0 (46.0, 72.0) (N = 10)	62.0 (53.0, 95.0) (N = 9)	77.0 (53.0, 94.0) (N = 11)
RVSP (mmHg)	50.0 (36.0, 61.0) (N = 31)	57.0 (31.0, 73.0) (N = 7)	37.0 (22.0, 49.0) (N = 11)	60.0 (47.0, 62.0) (N = 13)
ET-1 (pg/mL)	2.7 (2.3, 5.1) (N = 50)	3.1 (2.3, 3.9) (N = 14)	2.2 (1.8, 2.7) (N = 15)	4.4 (2.5, 6.0) (N = 21)
NT-proBNP (pg/mL)	289.0 (119.0, 592.0) (N = 53)	386.0 (119.0, 661.0) (N = 15)	264.0 (89.5, 541.0) (N = 16)	297.0 (125.0, 592.0) (N = 22)
RDW (%)	14.6 (13.9, 16.5) (N = 51)	15.1 (14.2, 16.4) (N = 15)	14.4 (13.5, 15.0) (N = 15)	15.4 (14.2, 16.8) (N = 21)
cGMP (pmoles/mL)	51.0 (36.0, 120.3) (N = 48)	41.3 (33.1, 109.8) (N = 13)	60.7 (33.3, 121.3) (N = 15)	51.0 (39.4, 115.7) (N = 20)
SNO-Hb (moles SNO/mole Hb)	0.0011 (0.0008, 0.0019) (N = 36)	0.0008 (0.0005, 0.0011) (N = 10)	0.0010 (0.0008, 0.0014) (N = 12)	0.0017 (0.0011, 0.0021) (N = 14)
cGMP to NTproBNP ratio (pmoles/pg)	0.25 (0.10, 0.63) (N = 43)	0.23 (0.10, 0.66) (N = 12)	0.29 (0.05, 0.69) (N = 14)	0.27 (0.11, 0.41) (N = 17)
ADMA (nM)	520.0 (446.0, 563.0) (N = 53)	550.5 (500.0, 556.0) (N = 14)	526.5 (442.5, 594.0) (N = 16)	515.0 (432.0, 565.0) (N = 23)
SDMA (nM)	413.0 (352.7, 493.5) (N = 50)	352.7 (327.0, 402.2) (N = 13)	453.6 (411.2, 521.0) (N = 14)	424.9 (353.6, 500.0) (N = 23)
NO2- (nM)	51.2 (34.1, 98.2) (N = 30)	72.6 (41.0, 163.4) (N = 10)	39.0 (26.5, 53.2) (N = 8)	46.9 (35.0, 93.0) (N = 12)

Overall
N = 58

On therapy with ERA
N = 16

On therapy with PDE5i or sGC^a
N = 17

On therapy with ERA + PDE5i^b
N = 25

Age (years)

61.1 (46.1, 68.5)

61.6 (51.0, 68.1)

69.1 (61.1, 76.4)

51.2 (37.7, 63.9)

Female

52 (89.7%)

16 (100.0%)

14 (82.4%)

22 (88.0%)

Years since PAH diagnosis

6.7 (2.3, 10.7)

7.5 (2.8, 12.0)

5.9 (1.2, 10.9)

6.8 (2.7, 10.0)

6MWD (m)

427.9 (362.1, 487.4)

(N = 54)

413.8 (359.1, 461.0)

427.9 (351.1, 460.9)

(N = 15)

457.2 (369.0, 526.7)

(N = 23)

mPAP (mmHg)

40.3 (27.0, 49.3)

(N = 35)

39.8 (31.8, 48.3)

(N = 12)

29.2 (22.0, 37.0)

(N = 10)

46.7 (40.3, 50.0)

(N = 13)

PVR (WU)

5.1 (3.6, 6.3)

(N = 31)

5.1 (3.9, 6.2)

(N = 11)

3.6 (2.4, 3.7)

(N = 9)

6.0 (5.6, 6.9)

(N = 11)

DLCO (% predicted)

65.5 (53.0, 77.0)

(N = 30)

53.0 (46.0, 72.0)

(N = 10)

62.0 (53.0, 95.0)

(N = 9)

77.0 (53.0, 94.0)

(N = 11)

RVSP (mmHg)

50.0 (36.0, 61.0)

(N = 31)

57.0 (31.0, 73.0)

(N = 7)

37.0 (22.0, 49.0)

(N = 11)

60.0 (47.0, 62.0)

(N = 13)

ET-1 (pg/mL)

2.7 (2.3, 5.1)

(N = 50)

3.1 (2.3, 3.9)

(N = 14)

2.2 (1.8, 2.7)

(N = 15)

4.4 (2.5, 6.0)

(N = 21)

NT-proBNP (pg/mL)

289.0 (119.0, 592.0)

(N = 53)

386.0 (119.0, 661.0)

(N = 15)

264.0 (89.5, 541.0)

(N = 16)

297.0 (125.0, 592.0)

(N = 22)

RDW (%)

14.6 (13.9, 16.5)

(N = 51)

15.1 (14.2, 16.4)

(N = 15)

14.4 (13.5, 15.0)

(N = 15)

15.4 (14.2, 16.8)

(N = 21)

cGMP (pmoles/mL)

51.0 (36.0, 120.3)

(N = 48)

41.3 (33.1, 109.8)

(N = 13)

60.7 (33.3, 121.3)

(N = 15)

51.0 (39.4, 115.7)

(N = 20)

SNO-Hb (moles SNO/mole Hb)

0.0011 (0.0008, 0.0019)

(N = 36)

0.0008 (0.0005, 0.0011)

(N = 10)

0.0010 (0.0008, 0.0014)

(N = 12)

0.0017 (0.0011, 0.0021)

(N = 14)

cGMP to NTproBNP ratio (pmoles/pg)

0.25 (0.10, 0.63)

(N = 43)

0.23 (0.10, 0.66)

(N = 12)

0.29 (0.05, 0.69)

(N = 14)

0.27 (0.11, 0.41)

(N = 17)

ADMA (nM)

520.0 (446.0, 563.0)

(N = 53)

550.5 (500.0, 556.0)

(N = 14)

526.5 (442.5, 594.0)

(N = 16)

515.0 (432.0, 565.0)

(N = 23)

SDMA (nM)

413.0 (352.7, 493.5)

(N = 50)

352.7 (327.0, 402.2)

(N = 13)

453.6 (411.2, 521.0)

(N = 14)

424.9 (353.6, 500.0)

(N = 23)

NO2- (nM)

51.2 (34.1, 98.2)

(N = 30)

72.6 (41.0, 163.4)

(N = 10)

39.0 (26.5, 53.2)

(N = 8)

46.9 (35.0, 93.0)

(N = 12)

Continuous variables presented as median (25, 75); categorical variables presented as frequency (proportion)

ERA, endothelin receptor antagonist; PDE5i, phosphodiesterase 5 inhibitor; sGC, soluble guanylate cyclase stimulator; PAH, pulmonary arterial hypertension; 6MWD, six minute walk distance; m, meters; mPAP, mean pulmonary artery pressure; PVR, pulmonary vascular resistance; WU, woods units; RVSP, right ventricular systolic pressure

^a2/17 (11.8%) patients on therapy with soluble guanylate cyclase stimulator, the remainder on PDE5i

^b1/25 (4.0%) on therapy with soluble guanylate cyclase stimulator instead of PDE5i