Table 1.
Demographic and Clinical Data
| Variable | Cohort A (N=48) |
Cohort B (N=161) |
|---|---|---|
| Age, years | 62 (47–71) | 59 (49–71) |
| Female, n (%) | 36 (75) | 111 (69) |
| Body Mass Index, kg/m2 | 26 (23–31) | 27 (22–31)† |
| Etiology, n (%) | ||
| Idiopathic | 16 (33) | 64 (40) |
| Heritable | 4 (8) | 2 (1) |
| Drug/toxin-induced | 3 (6) | 4 (2) |
| Connective tissue disease | 20 (42) | 50 (31) |
| HIV infection | ----- | 3 (2) |
| Portal hypertension | ----- | 26 (16) |
| Congenital heart disease | 5 (11) | 9 (6) |
| PVOD/PCH | ----- | 3 (2) |
| Medications, n (%) | ||
| PDE5 inhibitors | 31 (65) | 64 (40) |
| Endothelin receptor antagonists | 28 (58) | 47 (29) |
| IV/SQ PGI2 | 7 (15) | 26 (16) |
| Inhaled/PO PGI2 or PGI2 receptor agonist | 17 (35) | 6 (4) |
| Soluble guanylate cyclase stimulator | 3 (6) | 2 (1) |
| Calcium channel blockers | 3 (6) | 12 (7) |
| Treatment naïve | 9 (19) | 47 (29) |
| Comorbidities, n (%) | ||
| Hypertension | 21 (44) | 58 (36) |
| Obstructive sleep apnea | 17 (35) | 26 (16) |
| COPD | 6 (13) | 27 (17) |
| Interstitial lung disease | 3 (6) | 9 (6) |
| Cancer | 8 (17) | 15 (9) |
| Diabetes mellitus | 7 (15) | 21 (13) |
| REVEAL 2.0 | 6 (4–9) | 7 (6–9) |
| REVEAL Lite 2 | 5 (4–6) | 7 (5–8) |
| mPAP, mmHg | 36 (25–44) | 44 (34–52) |
| RAP, mmHg | 5 (3–7)* | 10 (7–13) |
| PAWP, mmHg | 9 (6–12)* | 8 (6–13) |
| PVR, WU | 5 (3–8) | 8 (5–10) |
| HR, bpm | 71 (62–84)* | 78 (67–90) |
| SBP, mmHg | 130 (113–141)* | 122 (112–136) |
| DLCO, % predicted | 55 (42–72)* | NR |
| 6MWD, meters | 333 (256–405)* | 358 (158–466)† |
| NYHA class | 2 (2–3)* | 3 (2–3)† |
| NT-proBNP, pg/ml | 288 (78–763)* | NR |
| BNP, pg/ml | NR | 145 (56–418)† |
| Hospitalization within the past 6 months, n (%) | 19 (40) | NR |
Continuous variables are displayed as median (interquartile range).
Indicates less than 48 values available: RAP=47; PAWP=47; HR=37; SBP=46; DLCO=46; 6MWD=45; NYHA=39; NT-proBNP=46
Indicates less than 161 values available: Body mass index=159; NYHA=160; 6MWD=136; BNP=158,
Abbreviations: PAH, pulmonary arterial hypertension; HIV, human immunodeficiency virus; PVOD/PCH, pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis; PDE5, phosphodiesterase 5; PGI2, prostacyclin; COPD, chronic obstructive pulmonary disease; REVEAL, Registry to Evaluate Early and Long-Term PAH Disease Management; mPAP, mean pulmonary artery pressure; RAP, right atrial pressure; PAWP, pulmonary artery wedge pressure; PVR, pulmonary vascular resistance; WU, Wood units; bpm, beats per minute; DLCO, diffusing capacity of the lungs for carbon monoxide; NR, not recorded; 6MWD, six minute walk distance; NYHA, New York Heart Association; NT-proBNP, N-terminal pro brain-type natriuretic peptide; BNP, brain-type natriuretic peptide.