Table 1.
Baseline demographics.
| Combined group (n = 56) | No lung disease (n = 35) | Lung disease (n = 21) | p Values | |
|---|---|---|---|---|
| Age (year) | 61 (50–70) | 59 (48–67) | 68 (55–75) | 0.02 |
| Male (n, %) | 24 (43%) | 11 (32%) | 13 (62%) | 0.05 |
| Deceased (n, %) | 6 (11%) | 1 (3%) | 5 (24%) | 0.024 |
| Race (n, %) | ||||
| Caucasian | 48 (86%) | 32 (91%) | 16 (76%) | 0.16 |
| African-American | 5 (9%) | 2 (6%) | 3 (14%) | |
| Asian | 1 (2%) | 1 (3%) | 0 (0%) | |
| Not reported | 2 (4%) | 0 (0%) | 2 (10%) | |
| Parenchymal lung disease (n, %) | 35 (63%) | 35 (100%) | 0 (0%) | <0.001 |
| None | 12 (21%) | 0 (0%) | 12 (57%) | |
| COPD | 6 (11%) | 0 (0%) | 6 (29%) | |
| ILD | 3 (5%) | 0 (0%) | 3 (14%) | |
| CPFE | ||||
| Lung transplant (n, %) | 2 (4%) | 0 (0%) | 2 (10%) | 0.13 |
| Scleroderma (n, %) | 7 (13%) | 4 (12%) | 3 (14%) | >0.9 |
| Smoker (n, %) | 30 (54%) | 13 (37%) | 17 (81%) | 0.002 |
| Total pack yearsa | 13.8 (21.4) | 0 (0–2.5) | 18.0 (9.3–45.0) | <0.001 |
| Pulmonary vasodilator (n, %) | 27 (49%) | 16 (46%) | 11 (55%) | 0.51 |
| Ambrisentan | 5 (19%) | 4 (25%) | 1 (9%) | |
| Bosentan | 1 (4%) | 1 (6%) | 0 (0%) | |
| Riociguat | 5 (19%) | 3 (19%) | 2 (18%) | |
| Selexipag | 1 (4%) | 0 (0%) | 1 (9%) | |
| Sildenafil | 11 (41%) | 10 (63%) | 1 (9%) | |
| Tadalafil | 17 (63%) | 9 (56%) | 8 (73%) | |
| 6MWT (m) | 329 (259–396) | 363 (317–415) | 293 (210–366) | 0.10 |
| WHO Functional Class (n, %)a | 0.20 | |||
| I | 2 (6%) | 0 (0%) | 2 (14%) | |
| II | 21 (62%) | 16 (80%) | 5 (36%) | |
| III | 10 (29%) | 4 (20%) | 6 (43%) | |
| IV | 1 (3%) | 0 (0%) | 1 (7%) | |
| Pulmonary function testing | ||||
| FEV1 (%) | 82 (65–101) | 93 (69–112) | 70 (48–81) | 0.006 |
| FVC (%) | 86 (70–99) | 88 (71–106) | 77 (69–93) | 0.33 |
| DLco (%) | 63 (45–72) | 70 (56–80) | 44 (34–55) | <0.001 |
| Transthoracic echocardiogram | ||||
| TR Jet (m/s) | 2.6 (2.3–3.0) | 2.4 (2.2–2.7) | 3.0 (2.8–3.2) | <0.001 |
| LAVI (mL/m2) | 27 (19–32) | 22.0 (19.0–32.0) | 29.5 (21.0–33.5) | 0.22 |
Values are indicated as median with interquartile range (IQR) unless otherwise specified by “a”.
a
Mean with standard deviation (SD).
COPD: chronic obstructive pulmonary disease; ILD: interstitial lung disease; CPFE: combined pulmonary fibrosis and emphysema; 6MWT: 6-min walk test; WHO: World Health Organization; TR: tricuspid regurgitant. FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity; DLco: diffusing capacity of lungs for carbon monoxide; LAVI: left atrial volume index. Patients were not treated with PH-specific therapies at the time of exRHC; medications were later added at the discretion of the treating physician.