TABLE 1.
Baseline demographics, haemodynamics, and cardiac MRI metrics.
| Baseline (n = 311) | IPAH/HPAH (n = 101) | PAH-CTD (n = 154) | PAH-CHD (n = 24) | PoPH (n = 22) |
Other PAH (n = 10) | |
| Demographics | ||||||
| Age, year | 56.7 ± 16.1 | 50.9 ± 17.6 | 63.2 ± 13.0 | 48.0 ± 18.1 | 53.5 ± 11.0 | 43.3 ± 6.1 |
| Sex, F/M (F %) | 223/88 (72) | 70/31 (69) | 119/35 (77) | 20/4 (83) | 12/10 (55) | 2/8 (20) |
| WHO FC I, n (%) | 1 (1) | 0 (0) | 0 (0) | 1 (4) | 0 (0) | 0 (0) |
| WHO FC II, n (%) | 14 (4) | 5 (5) | 4 (3) | 2 (8) | 2 (9) | 1 (10) |
| WHO FC III, n (%) | 261 (84) | 76 (75) | 137 (89) | 20 (87) | 19 (86) | 9 (90) |
| WHO FC IV, n (%) | 34 (11) | 20 (20) | 13 (8) | 0 (0) | 1 (5) | 0 (0) |
| PAH subtype, n (%) | ||||||
| IPAH/HPAH | 101 (33) | |||||
| PAH-CTD | 154 (49) | |||||
| PAH-CHD | 24 (8) | |||||
| PoPH | 22 (7) | |||||
| Other | 10 (3) | |||||
| Haemodynamics | ||||||
| mRAP, mm Hg | 10 ± 6 | 12 ± 6 | 9 ± 6 | 11 ± 5 | 11 ± 7 | 15 ± 8 |
| mPAP, mm Hg | 48 ± 14 | 57 ± 12 | 41 ± 12 | 55 ± 8 | 46 ± 8 | 52 ± 6 |
| PAWP, mm Hg | 10 ± 3 | 11 ± 3 | 10 ± 3 | 11 ± 3 | 11 ± 3 | 13 ± 2 |
| Cardiac output L/min | 4.8 ± 1.5 | 4.3 ± 1.3 | 5.0 ± 1.5 | 6.0 ± 1.4 | 5.6 ± 1.7 | 5.5 ± 0.6 |
| Cardiac index, L/min/m2 | 2.7 ± 0.9 | 2.4 ± 0.8 | 2.9 ± 0.8 | 3.0 ± 0.6 | 3.1 ± 1.6 | 2.8 ± 0.5 |
| PVR, dynes/m2 | 728 ± 420 | 976 ± 404 | 596 ± 393 | 640 ± 214 | 540 ± 189 | 593 ± 87 |
| MvO2, % | 63.4 ± 9.2 | 59.8 ± 8.0 | 65.1 ± 8.9 | 73.3 ± 10.9 | 67.8 ± 7.0 | 56.8 ± 12.8 |
| Right atrial measurement | ||||||
| RA area, cm2 | 25.6 ± 9.5 | 27.2 ± 10.2 | 24.2 ± 8.4 | 28.3 ± 10.7 | 24.3 ± 9.7 | 28.4 ± 11.4 |
| Right ventricle measurements | ||||||
| RVESVi, ml/m2 | 73.6 ± 34.0 | 84.9 ± 31.7 | 63.6 ± 31.4 | 85.7 ± 43.9 | 66.7 ± 28.4 | 100.2 ± 27.5 |
| RVESVi %pred | 301.8 ± 141.5 | 69.5 ± 22.4 | 75.7 ± 21.6 | 103.2 ± 63.6 | 81.0 ± 28.3 | 76.8 ± 31.3 |
| RVEDVi, ml/m2 | 110.8 ± 37.1 | 119.4 ± 34.7 | 99.9 ± 33.6 | 135.9 ± 43.5 | 107.4 ± 35.1 | 141.3 ± 38.8 |
| RVSVi, ml/m2 | 37.2 ± 13.8 | 34.5 ± 10.6 | 36.3 ± 10.2 | 50.2 ± 28.8 | 40.8 ± 14.3 | 41.0 ± 16.8 |
| RVEF, % | 35.8 ± 12.8 | 30.1 ± 9.6 | 39.2 ± 12.7 | 38.1 ± 17.6 | 39.2 ± 12.2 | 28.8 ± 7.5 |
| RVEF %pred | 53.3 ± 18.5 | 45.6 ± 14.6 | 57.3 ± 18.3 | 57.8 ± 25.3 | 59.0 ± 18.0 | 44.7 ± 11.4 |
MRI, magnetic resonance imaging; WHO FC, World Health Organisation functional class; PAH, pulmonary arterial hypertension; IPAH, idiopathic pulmonary arterial hypertension; HPAH, heritable pulmonary arterial hypertension; PAH-CTD, pulmonary arterial hypertension associated with connective tissue disease; PAH-CHD, pulmonary arterial hypertension associated with congenital heart disease; PoPH, portopulmonary hypertension; mRAP, mean right atrial pressure; mPAP, mean pulmonary arterial pressure; PAWP, pulmonary arterial wedge pressure; PVR, pulmonary vascular resistance; MvO2, mixed venous oxygen saturation; RA, right atrial; %pred, percentage predicted for age and sex; RVESVi, right ventricular end-systolic volume index; RVEDVi, right ventricular end-diastolic volume index; RVSVi, right ventricular stroke volume index; RVEF, right ventricular ejection fraction. Data are shown as mean ± SD unless otherwise stated.