Table 1.
Patient characteristics.
| All, n = 338 | IPAH/FPAH, n = 171 | CTD‐PAH, n = 91 | Other‐PAH, n = 76a | p Value | |
|---|---|---|---|---|---|
| Age at enrollment, years, mean | 52.6 ± 13.4 | 50.9 ± 14.6 | 59.6 ± 12.2 | 47.9 ± 13.3 | <0.001 |
| Female, n (%) | 249 (73.7) | 119 (69.6) | 74 (81.3) | 56 (73.7) | 0.12 |
| Race, White/AA/Others, n | 257/37/44 | 137/14/20 | 64/16/11 | 56/7/13 | 0.16 |
| Prevalent/incident | 291 (86)/47 (14) | 152 (89)/19 (11) | 72 (79)/19 (21) | 67 (88)/9 (12) | 0.07 |
| Years since PH diagnosis, median (min, max) | 3.2 (0.0, 35.9) | 3.7 (0.0, 35.9) | 2.5 (0.0, 21.0) | 4.0 (0.0, 27.2) | 0.19 |
| WHO FC at enrollment (I/II/III/IV), n | 43/137/141/17 | 32/76/58/5 | 6/32/46/7 | 6/28/37/5 | <0.001 |
| Vitals signs at enrollment | |||||
| Systolic BP, mmHg, mean | 114 ± 16 | 114 ± 15 | 117 ± 18 | 113 ± 16 | 0.27 |
| Diastolic BP, mmHg, mean | 69 ± 10 | 68 ± 10 | 69 ± 11 | 69 ± 10 | 0.77 |
| Heart rate, bpm, mean | 76 ± 13 | 77 ± 12 | 77 ± 13 | 75 ± 14 | 0.72 |
| Respiratory rate, rpm, mean | 18 ± 4 | 17 ± 2 | 19 ± 6 | 18 ± 3 | 0.06 |
| BMI, kg/m2, mean | 29.1 ± 7.5 | 30.0 ± 7.3 | 27.8 ± 7.1 | 28.5 ± 8.2 | 0.04 |
| Laboratory data at enrollment | |||||
| Creatinine, mg/dL, mean | 0.9 ± 0.4 | 1.0 ± 0.6 | 1.0 ± 0.5 | 1.0 ± 0.4 | 0.93 |
| eGFR, mL/min/1.73 m2, mean | 80.6 ± 24.4 | 82.5 ± 23.8 | 75.4 ± 25.0 | 82.2 ± 24.4 | 0.08 |
| Na, mEq/mL, mean | 139.4 ± 2.8 | 139.6 ± 2.7 | 139.5 ± 2.8 | 139.1 ± 3.1 | 0.47 |
| Bilirubin total, mg/dL, mean | 0.7 ± 0.6 | 0.7 ± 0.5 | 0.6 ± 0.3 | 1.0 ± 0.9 | <0.001 |
| Hemoglobin, g/dL, mean | 13.7 ± 2.1 | 13.9 ± 1.9 | 12.9 ± 1.9 | 14.3 ± 2.5 | <0.001 |
| ProBNP, pg/mL, median [P25, P75] | 258 [97, 997] | 185 [83, 785] | 602 [179, 2252] | 180 [91, 857] | <0.001 |
| Troponin, ng/mL, median (min, max) | 0.01 (0.009, 0.15) | 0.009 (0.009, 0.15) | 0.009 (0.009, 0.03) | 0.009 (0.009, 0.118) | 0.59 |
| Pulmonary function at enrollment | |||||
| 6MWD, m, median (min, max) | 405 (38, 732) | 420 (38, 732) | 364 (122, 615) | 394 (107, 615) | <0.001 |
| FEV1 percentage, mean | 78.1 ± 18.1 | 80.4 ± 17.7 | 74.7 ± 18.1 | 76.6 ± 18.7 | 0.05 |
| FVC percentage, mean | 84.4 ± 17.1 | 87.3 ± 15.2 | 78.9 ± 17.8 | 83.8 ± 19.2 | <0.001 |
| FEV1/FVC percentage, mean | 92.0 ± 10.0 | 91.2 ± 9.5 | 94.5 ± 10.7 | 91.1 ± 9.9 | 0.03 |
| TLC percentage, mean | 89.2 ± 15.9 | 92.9 ± 13.4 | 80.7 ± 14.7 | 91.0 ± 18.9 | <0.001 |
| DLCO percentage, mean | 57.4 ± 20.1 | 63.6 ± 19.0 | 41.9 ± 17.8 | 60.7 ± 19.5 | <0.001 |
| Therapies | |||||
| Diuretic, n (%) | 216 (63.9) | 113 (66.1) | 60 (65.9) | 43 (56.6) | 0.31 |
| PDE5 inhibitor, n (%) | 225 (66.6) | 117 (68.4) | 59 (64.8) | 49 (64.5) | 0.76 |
| ERA, n (%) | 189 (55.9) | 99 (57.9) | 45 (49.5) | 45 (59.2) | 0.34 |
| Prostacyclin, n (%) | 147 (43.5) | 85 (49.7) | 29 (31.9) | 33 (43.4) | 0.02 |
| SGC, n (%) | 17 (5.1) | 8 (4.7) | 3 (3.3) | 6 (7.9) | 0.38 |
| CCB, n (%) | 16 (4.8) | 11 (6.4) | 3 (3.3) | 2 (2.6) | 0.32 |
| 0/1/2/>2 PH drugs, n | 55/62/135/86 | 26/25/68/52 | 19/19/39/14 | 10/18/28/20 | 0.11 |
| Echocardiographic data | |||||
| RV dilation (normal/mild/moderate/severe), n | 66/101/89/70 | 34/55/42/35 | 22/18/30/15 | 10/28/17/20 | 0.07 |
| TR (none/mild/moderate/severe), n | 87/145/75/17 | 53/72/32/7 | 18/34/27/6 | 16/39/16/4 | 0.14 |
| RVSP, mmHg, median [P25, P75] | 64.7 [47.7, 84.3] | 61.6 [44.9, 80.3] | 65.3 [49.3, 77.8] | 69.9 [57.2, 90.8] | 0.05 |
| TAPSE, cm, median [P25, P75] | 1.8 [1.5, 2.2] | 1.9 [1.6, 2.2] | 1.8 [1.5, 2.2] | 1.8 [1.5, 2.0] | 0.91 |
| Pericardial effusion, n (%) | 135 (41.7) | 65 (40.0) | 51 (60.0) | 19 (25.7) | <0.001 |
| Hemodynamics at rest | |||||
| RAP, mmHg, mean | 7 ± 5 | 7 ± 4 | 7 ± 5 | 8 ± 6 | 0.67 |
| mPAP, mmHg, mean | 44 ± 15 | 43 ± 15 | 42 ± 13 | 42 ± 13 | 0.81 |
| PCWP, mmHg, mean | 11 ± 5 | 11 ± 5 | 10 ± 5 | 11 ± 6 | 0.34 |
| CO, L/min, median (min, max) | 5.0 (1.96, 13.7) | 5.2 (2.53, 11.73) | 4.7 (2.37, 13.7) | 5.0 (1.96, 9.05) | 0.18 |
| CI, L/min/m2, mean | 2.79 ± 0.88 | 2.79 ± 0.86 | 2.77 ± 0.97 | 2.79 ± 0.83 | 0.97 |
| PVR, Wood units, median [P25, P75] | 6.2 (0.5, 33.7) | 6.1 (0.5, 19.2) | 6.2 (1.2, 31.7) | 6.7 (2.0, 33.7) | 0.09 |
| SVR, mL/beat, mean | 71.7 ± 26.6 | 75.0 ± 26.6 | 66.7 ± 27.2 | 70.2 ± 24.7 | 0.04 |
| Mortality, n (%) | 42 (12.4) | 17 (9.9) | 17 (18.7) | 8 (10.5) | 0.10 |
Note: Statistics presented as mean ± SD, median [P25, P75], median (min, max) or N (%).
Abbreviations: 6MWD, 6 minute walk distance; AVR, acute vasodilator response; BMI, body mass index; BP, blood pressure; CCB, calcium‐channel blockers; CI, cardiac index; CO, cardiac output; DLCO, diffusion capacity of the lung to carbon monoxide; eGFR, estimated glomerular filtration rate; ERA, endothelin receptor antagonist; FEV1, force expiratory volume in 1 second; FVC, force vital capacity; IPAH, idiopathic pulmonary arterial hypertension; mPAP, mean pulmonary artery pressure; PAH, pulmonary arterial hypertension; PCWP, pulmonary capillary wedge pressure; PDE5, phosphodiesterase‐5; PVR, pulmonary vascular resistance; RAP, right atrial pressure; RV, right ventricle; RVSP, Right Ventricular Systolic Pressure; SCG, soluble guanylate cyclase; SVR, systemic vascular resistance; TAPSE, tricuspid annular plane systolic excursion; TLC, total lung capacity; TR, tricuspid regurgitation.
Other‐PAH includes: DI‐PAH, HIV‐PAH, PH‐PAH, CHD‐PAH, PVOD‐PAH, PCH‐PAH.