Table 1.
Baseline population characteristics of patients with SAPH grouped by PH-specific therapy initiation. Categorical variables expressed as N (%), and normally distributed continuous variables given as mean (SD), otherwise median (Q1, Q3).
| No PH therapy (N = 21) | Started on PH therapy (N = 74) | Total (N = 95) | P-value | |
|---|---|---|---|---|
| Age at PH Diagnosis, years | 49.4 ± 9.8 | 53.0 ± 11.2 | 52.2 ± 11.0 | 0.1 |
| Women | 12 (57.14%) | 60 (81.08%) | 72 (75.79%) | 0.02 |
| Race | 1.0 | |||
| Caucasian | 2 (9.5%) | 10 (13.5%) | 12 (12.6%) | |
| African American | 19 (90.5%) | 63 (85.1%) | 82 (86.3%) | |
| Other | 0 (0%) | 1 (1.4%) | 1 (1.0%) | |
| BMI, kg/m2 | 28.4 (25.4, 36.1) | 29.3 (24.8, 34.3) | 28.6 (25.0, 34.3) | 0.9 |
| Smoker | 8 (38.1%) | 27 (36.5%) | 35 (36.8%) | 0.9 |
| WHO Functional Class | 0.1 | |||
| Class 1 | 0 (0.00%) | 1 (1.35%) | 1 (1.06%) | |
| Class 2 | 6 (30.00%) | 15 (20.27%) | 21 (22.34%) | |
| Class 3 | 4 (20.00%) | 34 (45.95%) | 38 (40.43%) | |
| Class 4 | 10 (50.00%) | 24 (32.43%) | 34 (36.17%) | |
| Sarcoidosis Stage | 0.2 | |||
| 0 | 2 (10.53%) | 3 (4.05%) | 5 (5.38%) | |
| 1 | 2 (10.53%) | 4 (5.41%) | 6 (6.45%) | |
| 2 | 4 (21.05%) | 8 (10.81%) | 12 (12.90%) | |
| 3 | 1 (5.26%) | 4 (5.41%) | 5 (5.38%) | |
| 4 | 10 (52.6%) | 55 (74.3%) | 65 (69.9%) | |
| DLCO, % | 44.0 (24.0, 57.0) | 32.0 (26.0, 45.0) | 33.0 (26.0, 47.0) | 0.2 |
| Mean FVC, % | 53.4 ± 20.2 | 56.5 ± 18.4 | 55.8 ± 18.7 | 0.6 |
| Immunosuppressants | ||||
| Steroids | 11 (52.4%) | 47 (63.5%) | 58 (61.0%) | 0.4 |
| Methotrexate | 0 (0.0%) | 6 (8.1%) | 6 (6.3%) | 0.3 |
| Mycophenolate | 0 (0.0%) | 2 (2.7%) | 2 (2.1%) | 1.0 |
| Hydroxychloroquine | 2 (9.5%) | 7 (9.5%) | 9 (9.5%) | 1.0 |
| Systolic BP, mmHg | 125.5 ± 22.3 | 126.7 ± 20.1 | 126.4 ± 20.5 | 0.8 |
| Biomarkers | ||||
| NT-proBNP, pg/mL (N = 37) | 510.5 (169.0, 5062.0) | 1028.5 (254.0, 2311.0) | 909.5 (225.0, 2807.0) | 0.9 |
| Echocardiography (N = 95) | ||||
| RV size | 0.9 | |||
| Normal | 3 (14.3%) | 16 (21.6%) | 19 (20.0%) | |
| Mildly enlarged | 5 (23.8%) | 15 (20.3%) | 20 (21.1%) | |
| Moderately enlarged | 4 (19.1%) | 16 (21.6%) | 20 (21.1%) | |
| Severely enlarged | 9 (42.9%) | 27 (36.5%) | 36 (37.9%) | |
| RV function | 0.3 | |||
| Normal | 10 (47.6%) | 21 (28.4%) | 31 (32.6%) | |
| Mild dysfunction | 1 (4.8%) | 11 (14.9%) | 12 (12.6%) | |
| Moderate dysfunction | 5 (23.8%) | 25 (33.8%) | 30 (31.6%) | |
| Severe dysfunction | 5 (23.8%) | 17 (23.0%) | 22 (23.2%) | |
| TAPSE, cm | 1.9 ± 0.7 | 1.9 ± 0.8 | 1.9 ± 0.7 | 0.9 |
| Pericardial effusion | 0.2 | |||
| None/trace | 18 (85.7%) | 69 (93.2%) | 87 (91.6%) | |
| Mild | 1 (4.8%) | 4 (5.4%) | 5 (5.3%) | |
| Moderate | 2 (9.5%) | 1 (1.4%) | 3 (3.2%) | |
| Hemodynamics (N = 75) | ||||
| RA pressure, mmHg | 10.0 (8.0, 15.0) | 8.0 (5.0, 13.0) | 8.0 (5.0, 14.0) | 0.3 |
| Mean PA pressure, mmHg | 47.6±15.3 | 49.1 ± 12.0 | 48.8 (12.5) | 0.7 |
| Cardiac index, L/min/m2 | 2.2 (1.9, 2.8) | 2.3 (1.7, 2.7) | 2.2 (1.7, 2.7) | 0.8 |
| PVR, Wood units | 6.1 (4.1, 12.3) | 8.6 (6.3, 12.7) | 8.5 (5.5, 12.7) | 0.1 |
Abbreviations: PH (pulmonary arterial hypertension), BMI (body mass index), WHO (World Health Organization), DLCO (diffusing capacity of the lung for carbon monoxide), FVC (forced vital capacity), 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).