Table 1.
Demographics and Clinical Characteristics of the Cohort at Enrollment
| Observations (No.) | Demographics/Clinical Characteristics | Overall (N = 2,017) | CTD-PAH (n = 623) | IPAH (n = 870) |
|---|---|---|---|---|
| Demographics | ||||
| 2,017 | Age, y | 55 (15) | 59 (14) | 55 (15) |
| 2,017 | Sex, female, No. (%) | 1,611 (80) | 565 (91) | 698 (80) |
| 2,017 | Race, white, No. (%) | 1,662 (82) | 564 (91) | 780 (90) |
| 1,448 | NYHA FC I/II/III/IV, No. (%III/IV) | 90/451/789/118 (63) | 24/140/266/34 (65) | 38/188/340/56 (64) |
| 1,023 | 6MWD, m | 347 (141) | 327 (160) | 351 (136) |
| 1,984 | Deaths during follow-up, No. (%) | 324 (16) | 125 (20) | 112 (13) |
| 2,017 | Etiology (FPAH/PVOD/PortoPulm/congenital/drug/HIV/other), No. | 81/8/111/171 /93/42/18 | … | … |
| 2,017 | REVEAL Registry 1.0 score | 7.3 (1.8) | 8.0 (1.6) | 6.7 (1.7) |
| 2,017 | REVEAL Registry 2.0 score | 7.3 (2.4) | 7.9 (2.1) | 6.5 (2.2) |
| Biomarker values | ||||
| 2,017 | NT-proBNP, median (P25-P75), pg/mL | 672 (217-2,164) | 907 (331-2,164) | 520 (183-1,621) |
| 2,017 | Gal3, median (P25-P75), pg/mL | 10,129 (6,925-14,073) | 10,444 (7,151-15,057) | 10,335 (7,010-14,270) |
| 2,017 | ST2, median (P25-P75), pg/mL | 5,593 (3,772-8,939) | 6,563 (4,398-11,249) | 5,005 (3,419-7,752) |
| Hemodynamics | ||||
| 1,972 | RAP, mm Hg | 9 (5) | 9 (5) | 9 (6) |
| 2,007 | mPAP, mm Hg | 50 (15) | 44 (11) | 51 (14) |
| 1,951 | PAWP, mm Hg | 10 (4) | 10 (4) | 10 (4) |
| 1,916 | PVR, Wood units | 10 (6) | 8 (5) | 10 (6) |
| 1,940 | Cardiac output, L/min | 4.7 (1.7) | 4.7 (1.6) | 4.6 (1.6) |
| 1,940 | Cardiac index, L/min/m2 | 2.7 (1.2) | 2.8 (0.9) | 2.6 (1.1) |
| 2,017 | Therapies, No. (%) | |||
| PDE5 inhibitor | 1,546 (77) | 470 (75) | 641 (74) | |
| ERA | 1,205 (60) | 370 (59) | 515 (59) | |
| IV/SC prostacyclin | 699 (35) | 161 (26) | 355 (41) | |
| CCB | 199 (10) | 51 (8) | 99 (11) |
All data are presented as mean (SD) unless otherwise specified. 6MWD = 6-min walk distance; CCB = calcium channel blocker; CTD-PAH = connective tissue disease-associated pulmonary arterial hypertension; ERA = endothelin receptor antagonist; FPAH = familial PAH; Gal3 = galectin 3; IPAH = idiopathic PAH; mPAP = mean pulmonary arterial pressure; NT-proBNP = N-terminal brain natriuretic peptide prohormone; NYHA FC = New York Heart Association functional class; P25, P75 = 25th and 75th percentiles; PAWP = pulmonary artery wedge pressure; PDE5 = phosphodiesterase-5; PortoPulm = portopulmonary hypertension; PVOD = pulmonary venoocclusive disease; PVR = pulmonary vascular resistance; RAP = right atrial pressure; REVEAL Registry = Registry to Evaluate Early and Long-Term PAH Disease Management; SC = subcutaneous; ST2 = soluble suppression of tumorigenicity 2.