Baseline characteristics and outcomes
| PHRHC | No PHRHC | No PHecho | p-value | ||
| PHRHC versus no PHRHC | PHRHC versus no PHRHC or PHecho | ||||
| Demographics | |||||
| Patients n | 25 | 12 | 52 | ||
| Males n (%) | 16 (64.0) | 9 (75.0) | 35 (67.4) | 0.790 | 0.810 |
| Age years | 55.8±9.0 | 48.3±10.0 | 50.5±12.0 | 0.131 | 0.770 |
| BSA m2 | 1.9±0.2 | 2.0±0.3 | 2.1±0.2 | 0.194 | 0.020 |
| NYHA functional class I/II/III/IV | 0/6/15/4 | 1/2/8/0 | 12/29/9/2 | 0.518 | <0.001 |
| NT-pro-BNP log | 5.5±1.8 | 3.9±1.1 | 4.0±0.8 | 0.001 | <0.001 |
| Sarcoidosis | |||||
| Sarcoidosis-related fibrosis stage 4 | 25 (100) | 7 (58.3) | 30 (57.7) | 0.020 | 0.001 |
| Sarcoidosis treatment | 18 (72.0) | 10 (83.3) | 34 (65.4) | 0.766 | 0.460 |
| Oxygen therapy | 7 (28.0) | 0 | 1 (1.9) | 0.009 | <0.001 |
| Pulmonary function | |||||
| FVC L | 2.29±0.89 | 3.19±1.32 | 3.63±1.10 | 0.054 | <0.001 |
| FVC % predicted | 57.6±18.1 | 73.71±24.00 | 83.83±18.00 | 0.050 | <0.001 |
| FEV1 L | 1.45±0.79 | 2.35±1.13 | 2.54±0.83 | 0.011 | <0.001 |
| FEV1 % predicted | 46.4±17.2 | 66.94±31.02 | 73.08±19.72 | 0.018 | <0.001 |
| FVC % predicted/FEV1 % predicted | 1.78±0.81 | 1.45±0.49 | 1.47±0.39 | 0.213 | 0.800 |
| DLCO % predicted | 41.4±15.4 | 53.38±22.34 | 58.15±18.21 | 0.216 | 0.010 |
| FVC % predicted/DLCO % predicted | 1.73±0.72 | 1.63±0.59 | 1.54±0.44 | 0.896 | 0.348 |
| Haemodynamics | |||||
| RVSP mmHg | 62.6±21.4 | 31.9±5.3 | 29.6±4.2 | <0.001 | <0.001 |
| Systolic PAP mmHg | 61.8±16.9 | 28.9±5.5 | <0.001 | ||
| Mean PAP mmHg | 39.2±9.9 | 17.5±3.4 | <0.001 | ||
| Diastolic PAP mmHg | 26.0±7.4 | 11.7±2.8 | <0.001 | ||
| PCWP mmHg | 12.9±6.8 | 8.8±2.6 | 0.012 | ||
| TPG mmHg | 26.1±10.9 | 8.6±3.4 | <0.001 | ||
| DPG mmHg | 13.0±2.8 | 8.7±2.9 | <0.001 | ||
| PVR WU | 5.6±2.9 | 1.3±0.7 | <0.001 | ||
| Chest CT parameters | |||||
| PA diameter mm | 33.1±4.1 | 29.2±3.5 | 28.8±3.8 | 0.001 | <0.001 |
| PA diameter indexed to BSA mm·m−2 | 17.7±2.3 | 14.5±1.8 | 14.2±1.8 | <0.001 | <0.001 |
| PA diameter/AAD ratio | 1.1±0.2 | 0.9±0.1 | 1.0±0.2 | <0.001 | <0.001 |
| Fibrosis extent % | 0.130 | 0.001 | |||
| <5 | 3 (12.0) | 5 (41.7) | 23 (44.2) | ||
| 5–20 | 2 (8.0) | 1 (8.3) | 9 (17.3) | ||
| >20 | 20 (80.0) | 6 (50.0) | 20 (38.5) | ||
Data are presented as n, n (%) or mean±sd, unless otherwise stated. PHRHC: pulmonary hypertension confirmed by right heart catheterisation; PHecho: pulmonary hypertension confirmed by echocardiography; BSA: body surface area; NYHA: New York Heart Association; NT-proBNP: N-terminal pro-brain natriuretic peptide; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide; RVSP: right ventricular systolic pressure; PAP: pulmonary artery pressure; PCWP: pulmonary capillary wedge pressure; TPG: transpulmonary pressure gradient; DPG: diastolic pulmonary gradient; PVR: pulmonary vascular resistance; WU: Wood units; CT: computed tomography; PA: pulmonary artery; AAD: ascending aorta diameter.