Baseline characteristics and haemodynamics of patients with PAH–CHD
| Variable | Overall group | Eisenmenger syndrome with pre-tricuspid defect | Eisenmenger syndrome with post-tricuspid defect | PAH with systemic-to-pulmonary shunt | PAH with small defects | PAH after defect correction |
| Patients, n (%) | 32 (100) | 7 (22) | 15 (47) | 1 (3) | 2 (6) | 7 (22) |
| Age | 40±10 | 36±10 | 42±10 | 44 | 36±3 | 40±11 |
| BNP (pg/mL) | 3044±6332 | 6983±13 750 | 2020±2484 | 1124 | 204±107 | 2643±2849 |
| 6-MWD (m) | 321±116 | 344±143 | 305±107 | 300 | 427±23 | 310±135 |
| WHO FC IV | 17(53) | 3 (43) | 10(67) | 0 | 1 (50) | 3 (42) |
| mRAP (mm Hg) | 11±6 | 7±3 | 13±6 | 9 | 9±3 | 12±6 |
| mPAP (mm Hg) | 73±21 | 68±12 | 80±19 | 45 | 50±3 | 72±34 |
| PVR (WU) | 19±10 | 16±6 | 22±10 | 30 | 11±4 | 20±4 |
| CI (L/min/m2) | 2.1±0.6 | 2.4±0.7 | 2.1±0.6 | 2.4 | 2±0.2 | 1.9±0.2 |
| SpO2 (%) | 86±8 | 88±7 | 81±7 | 94 | 95±0.7 | 93±2 |
All data are presented as means±SD.
6-MWD, 6 min walking distance; BNP, B-type brain natriuretic peptide; CHD, congenital heart disease; CI, cardiac index; mPAP, mean pulmonary artery pressure; mRAP, mean right atrial pressure; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance; SpO2, systemic oxygen saturation; WHO FC, WHO functional class; WU, Wood units.