Table 2.
Death or hospitalization (N = 64) | No clinical event (N = 31) | Total | P-value | |
---|---|---|---|---|
Baseline | ||||
Age at PH diagnosis, years | 50.9 (11.3) | 55.1 (9.9) | 52.2 (11.0) | 0.08 |
Women | 51 (79.7%) | 21 (67.7) | 72 (75.8%) | 0.2 |
Race | 1.0 | |||
Caucasian | 8 (12.5%) | 4 (12.9%) | 12 (12.6%) | |
African American |
55 (85.9%) | 27 (87.1%) | 82 (86.3%) | |
Other | 1 (1.6%) | 0 (0%) | 1 (1.1%) | |
BMI, kg/m2 | 28.5 (25.7, 36.1) | 28.9 (21.3, 33.8) | 28.6 (25.0, 34.3) | 0.4 |
WHO functional class | 0.6 | |||
Class 1 | 1 (1.6%) | 0 (0%) | 1 (1.1%) | |
Class 2 | 12 (18.8%) | 9 (30.0%) | 21 (22.3%) | |
Class 3 | 26 (40.6%) | 12 (40.0%) | 38 (40.4%) | |
Class 4 | 25 (39.1%) | 9 (30.0%) | 34 (36.2%) | |
Sarcoidosis stage | 0.7 | |||
0 | 3 (4.8%) | 2 (6.7%) | 5 (5.4%) | |
1 | 4 (6.4%) | 2 (6.7%) | 6 (6.5%) | |
2 | 8 (12.7%) | 4 (13.3%) | 12 (12.9%) | |
3 | 2 (3.2%) | 3 (10.0%) | 5 (5.4%) | |
4 | 46 (73.2%) | 19 (63.3%) | 65 (69.9%) | |
DLCO, % | 33.0 (25.0, 47.0) | 32.0 (27.0, 45.0) | 33.0 (26.0, 47.0) | 0.7 |
Immunosuppresants | ||||
Steroids | 39 (60.9%) | 19 (61.3%) | 58 (61.1%) | 1.0 |
Methotrexate | 5 (7.8%) | 1 (3.2%) | 6 (6.3%) | 0.7 |
Mycophenelate | 2 (3.1% | 0 (0%) | 2 (2.1%) | 1.0 |
Hydroxychloroquine | 5 (7.8%) | 4 (12.9%) | 9 (9.5%) | 0.5 |
Parameters at follow-up | ||||
NT-proBNP, pg/mL | 1258.0 (365.0, 3286.0) | 262.0 (150.5, 1810.0) | 785.5 (232.0, 2828.5) | 0.007 |
RV size | 0.2 | |||
Normal | 41 (64.1%) | 26 (83.9%) | 67 (70.5%) | |
Mildly enlarged | 6 (9.4%) | 2 (6.5%) | 8 (8.4%) | |
Moderately enlarged | 10 (15.6%) | 1 (3.2%) | 11 (11.6%) | |
Severely enlarged | 7 (10.9%) | 2 (6.5%) | 9 (9.5%) | |
RV function | 0.4 | |||
Normal | 46 (71.9%) | 25 (80.7%) | 71 (74.7%) | |
Mild dysfunction | 3 (4.7%) | 3 (9.7%) | 6 (6.3%) | |
Moderate dysfunction | 11 (17.2%) | 2 (6.5%) | 13 (13.7%) | |
Severe dysfunction | 4 (6.3%) | 1 (3.2%) | 5 (5.3%) | |
TAPSE, cm | 1.7 (0.5) | 1.8 (0.6) | 1.8 (0.5) | 0.7 |
RA pressure, mmHg | 10 (5, 14) | 7 (4, 13) | 9 (4, 14) | 0.3 |
Mean PA pressure, mmHg | 48 (13) | 45 (12) | 47 (13) | 0.4 |
Cardiac index, L/min/m2 | 2.4 (1.9, 3.0) | 2.5 (2.2, 3.0) | 2.5 (2.0, 3.0) | 0.4 |
PVR, Wood units | 8.4 (5.3, 11.5) | 7.4 (4.1, 8.9) | 8.1 (4.9, 10.5) | 0.09 |
Abbreviations: PH (pulmonary arterial hypertension), BMI (body mass index), WHO (World Health Organization), DLCO (diffusing capacity of the lung for carbon monoxide), NT-proBNP (N-terminal pro-brain natriuretic peptide), RV (right ventricular), TAPSE (tricuspid annular plane systolic excursion), RA (right atrial), PA (pulmonary artery), PVR (pulmonary vascular resistance).