TABLE 2 ] .
Patient Demographics and Disease Characteristics at Enrollment
| Parameter | Patients With ≥ 1 Hospitalization | Patients With No Hospitalizations (D) | P Value (C vs D) | P Value (A vs B) | ||
| PAH Related (A) | PAH Unrelated (B) | Alla (C) | ||||
| No. of patientsb | 257 | 214 | 471 | 372 | … | … |
| Mean age (SD), y | 51.9 (16.0) | 52.4 (17.0) | 52.1 (16.4) | 52.6 (17.6) | .70c | .73c |
| Age < 18 y, No. (%) | 3 (1.2) | 9 (4.2) | 12 (2.5) | 13 (3.5) | .42d | .037d |
| Age ≥ 65 y, No. (%) | 63 (24.5) | 46 (21.5) | 109 (23.1) | 95 (25.5) | .42d | .44d |
| Female, No. (%) | 201 (78.2) | 159 (74.3) | 360 (76.4) | 283 (76.1) | .90d | .32d |
| PAH cause | ||||||
| Idiopathic PAH | 128 (49.8) | 94 (43.9) | 222 (47.1) | 181 (48.7) | .37d | .38d |
| Familial PAH | 9 (3.5) | 5 (2.3) | 14 (3.0) | 7 (1.9) | … | … |
| Associated PAH | … | … | ||||
| CTD | 68 (26.5) | 74 (34.6) | 142 (30.1) | 108 (29.0) | ||
| CHD | 13 (5.1) | 10 (4.7) | 23 (4.9) | 21 (5.6) | ||
| PoPH | 17 (6.6) | 19 (8.9) | 36 (7.6) | 18 (4.8) | ||
| HIV | 5 (1.9) | 2 (0.9) | 7 (1.5) | 6 (1.6) | ||
| Drugs/toxins | 13 (5.1) | 7 (3.3) | 20 (4.2) | 20 (5.4) | ||
| Other | 2 (0.8) | 3 (1.4) | 5 (1.1) | 4 (1.1) | ||
| PVOD | 2 (0.8) | 0 (0.0) | 2 (0.4) | 7 (1.9) | ||
| Comorbidities, No. (%) | ||||||
| Diabetes | 42 (16.9) | 31 (14.8) | 73 (16.0) | 38 (10.6) | .025d | .54d |
| Hypertension | 105 (42.3) | 104 (49.8) | 209 (45.7) | 151 (41.9) | .28d | .11d |
| COPD | 36 (14.5) | 34 (16.3) | 70 (15.3) | 41 (11.4) | .10d | .60d |
| Depression | 51 (20.6) | 48 (23.0) | 99 (21.7) | 52 (14.4) | .008d | .54d |
| Pre-enrollment parenteral therapy | ||||||
| No. % | 57 (22.2) | 37 (17.3) | 94 (20.0) | 31 (8.3) | < .001 | .19 |
| Median days pre-enrollment | 17 | 33 | 24 | 28 | .75 | .088 |
| NYHA functional class, No. (%) | ||||||
| I | 9 (4.0) | 5 (2.7) | 14 (3.4) | 15 (4.8) | .014e | .034e |
| II | 41 (18.2) | 46 (25.1) | 87 (21.3) | 80 (25.8) | … | … |
| III | 128 (56.9) | 113 (61.7) | 241 (59.1) | 183 (59.0) | … | … |
| IV | 47 (20.9) | 19 (10.4) | 66 (16.2) | 32 (10.3) | … | … |
| 6MWD, m | ||||||
| No. of patients | 157 | 148 | 305 | 226 | .12c | .34c |
| Mean (SD) | 311.5 (124.9) | 298.5 (112.4) | 305.2 (119.0) | 322.5 (137.2) | … | … |
| BNP, pg/mL | ||||||
| No. of patients | 106 | 103 | 209 | 147 | .18f | .36f |
| Mean (SD) | 463.5 (583.4) | 457.0 (742.7) | 460.3 (665.1) | 491.6 (1070.2) | … | … |
| Median (IQR) | 262.5 (87.0-592.0) | 203.0 (81.0-530.0) | 231.0 (86.0-551.0) | 176.0 (64.0-502.0) | … | … |
| Pericardial effusion, No. (%) | 70 (27.2) | 52 (24.3) | 122 (25.9) | 63 (16.9) | .002d | .47d |
| Hemodynamics | ||||||
| Cardiac index, L/min/m2 | ||||||
| No. of patients | 203 | 168 | 371 | 290 | .018c | .026c |
| Mean (SD) | 2.1 (0.8) | 2.3 (0.8) | 2.2 (0.8) | 2.4 (0.9) | … | … |
| PVR, Wood units | ||||||
| No. of patients | 242 | 202 | 444 | 350 | .20c | .15c |
| Mean (SD) | 12.0 (6.0) | 10.5 (6.3) | 11.3 (6.1) | 10.8 (6.2) | … | … |
| mPAP at rest | ||||||
| No. of patients | 245 | 205 | 450 | 354 | .16c | .076c |
| Mean (SD) | 51.1 (12.9) | 48.9 (13.1) | 50.1 (13.0) | 48.8 (13.5) | … | … |
| mRAP, mm Hg | ||||||
| No. of patients | 225 | 186 | 411 | 323 | < .001c | .34c |
| Mean (SD) | 10.6 (6.5) | 10.0 (5.7) | 10.3 (6.2) | 8.8 (5.5) | … | … |
| REVEAL Registry risk score | ||||||
| No. of patients | 257 | 214 | 471 | 372 | < .001c | .84c |
| Mean (SD) | 8.6 (2.1) | 8.6 (2.1) | 8.6 (2.1) | 7.9 (2.2) | … | … |
| Length of follow-up from enrollment, mo | ||||||
| No. of patients | 257 | 214 | 471 | 372 | < .001c | .004c |
| Mean (SD) | 39.6 (22.6) | 45.3 (19.3) | 42.2 (21.3) | 35.6 (22.1) | … | … |
All patients with ≥ 1 hospitalization (C) includes patients with PAH-related (A) and PAH-unrelated (B) hospitalizations. Nineteen patients with undetermined causes of hospitalization were not included in this cohort. 6MWD = 6-min walk distance; BNP = brain natriuretic peptide; CHD = congenital heart disease; CTD = connective tissue disease; IQR = interquartile range; mPAP = mean pulmonary arterial pressure; mRAP = mean right atrial pressure; NYHA = New York Heart Association; PoPH = portopulmonary hypertension; PVOD = pulmonary veno-occlusive disease; PVR = pulmonary vascular resistance. See Table 1 legend for expansion of other abbreviation.
The total number of patients in each cohort with data at enrollment for each parameter is assumed to be 257 (A), 214 (B), 471 (C), and 372 (D), unless otherwise noted.
P value is computed using t test.
P value is computed using χ2 test.
P value is computed using Mantel-Haenszel test.
P value is computed using Wilcoxon signed rank sum test.