Table 1.
Baseline characteristics and SOD levels in patients with IPAH.
| Total (n = 133) | BMPR2 mut (n = 28) | BMPR2 wt (n = 105) | P-valuea | |
|---|---|---|---|---|
| Age, years | 36 (27, 51) | 28 (22, 34) | 39 (29, 53) | <0.001 |
| Female gender, n (%) | 94 (71) | 15 (54) | 79 (75) | <0.001 |
| BMI, kg/m2 | 22 (20, 24) | 21 (20, 23) | 22 (20, 24) | 0.163 |
| WHO FC, n (%) | 0.191 | |||
| Class II | 45 (34) | 9 (32) | 36 (34) | |
| Class III | 71 (53) | 17 (61) | 54 (51) | |
| Class IV | 17 (13) | 2 (7) | 15 (14) | |
| Onset to diagnosis, months | 24 (7, 48) | 18 (5, 43) | 24 (8, 48) | 0.293 |
| 6MWD, metersb | 367 (311, 439) | 407 (330, 248) | 360 (308, 440) | 0.376 |
| BNP, pg/ml | 355 (144, 515) | 307 (143, 533) | 391 (157, 475) | 0.812 |
| Cu/Zn-SOD, U/ml | 148 (114, 213) | 147 (125, 215) | 142 (110, 213) | 0.608 |
| Mn-SOD, U/ml | 40 (34, 65) | 40 (34, 80) | 40 (34, 163) | 0.863 |
| Ec-SOD, U/l | 85 (79, 91) | 82 (77, 86) | 86 (81, 93) | 0.010 |
| Hemodynamic variables | ||||
| HR, bpm | 84 (75, 94) | 86 (76, 92) | 84 (74, 98) | 0.787 |
| mRAP, mmHg | 7 (5, 12) | 5 (7, 12) | 7 (5, 12) | 0.862 |
| mPAP, mmHg | 62 (51, 69) | 68 (57, 82) | 59 (50, 66) | 0.003 |
| PAWP, mmHg | 9 (7, 11) | 9 (7, 11) | 9 (7, 11) | 0.959 |
| CO, L/min | 3.6 (3.0, 4.7) | 3.4 (2.8, 4.7) | 3.6 (3.0, 4.7) | 0.561 |
| CI, L/min/m2 | 2.2 (1.8, 32.8) | 2.0 (1.7, 2,7) | 1.9 (1.6, 2.5) | 0.362 |
| PVR, Wood units | 15 (10, 19) | 16 (13, 23) | 14 (9, 19) | 0.039 |
| SVO2, % | 59 (54, 67) | 58 (55, 67) | 59 (54, 67) | 0.654 |
| PAH-specific therapies, n (%)c | 0.848 | |||
| Bosentan (oral) | 16 (12) | 4 (14) | 12 (11) | |
| Iloprost (inhaled) | 3 (2) | 0 (0) | 3 (3) | |
| Sildenafil (oral) | 59 (44) | 14 (50) | 45 (43) | |
| Vardenafil (oral) | 30 (23) | 5 (18) | 25 (24) | |
| Combination therapy | 15 (11) | 2 (7) | 13 (12) | |
Values are expressed as medians (interquartile range), except for female gender and WHO functional class, which are numbers of patients (%).
Comparison between groups with BMPR2 mut and BMPR2 wt.
6MWD could be successfully measured in 124 patients.
Seven patients (5%) were enrolled in PATENT-1 (Riociguat) study.
BMI, body mass index; BMPR 2, bone morphogenetic protein receptor type 2; BNP, brain natriuretic peptide; CI, cardiac index; CO, cardiac output; Cu/Zn-SOD, copper–zinc superoxide dismutase; Ec-SOD, extracellular form of Cu/Zn superoxide dismutase; HR, heart rate; Mn-SOD, manganese superoxide dismutase; mPAP, mean pulmonary arterial pressure; mRAP, mean right atrial pressure; mut, mutation; 6MWD, 6-min walking distance; PAWP, pulmonary artery wedge pressure; PVR, pulmonary vascular resistance; SvO2, mixed venous oxygen saturation; WHO FC, World Health Organization functional class; wt, wild-type.