Characteristics | All patients (n = 45) |
Gender: | |
Male, n (%) | 9 (20) |
Female, n (%) | 36 (80) |
Age (years) | 32.5 (10.3) |
Weight (kg) | 53.1 (12.5) |
BSA (m2) | 1.5 (0.2) |
Aetiology of PAH: | |
IPAH, n (%) | 10 (22) |
CHD-PAH, n (%) | 23 (51) |
Repaired defects | 3 (7) |
Unrepaired defects* | 20 (44) |
CTD-PAH, n (%) | 11 (24) |
Porto-PAH, n (%) | 1 (2) |
WHO functional class: | |
II, n (%) | 11 (24) |
III, n (%) | 29 (64) |
IV, n (%) | 5 (12) |
mPAP (mm Hg) | 69.2 (14.9) |
RAP (mm Hg) | 5.6 (5.0) |
PCWP‡ (mm Hg) | 6.8 (3.6) |
PVR (Wood units) | 18.1 (9.3) |
CI (l/min/m2) | 2.7 (1.2) |
6MWD (m) | 409 (103) |
Borg dyspnoea score | 3.1 (1.6) |
Oral digoxin, n (%) | 21 (47) |
Oral warfarin, n (%) | 15 (33) |
Oral diuretics, n (%) | 31 (69) |
*Data are presented as mean (SD) unless stated otherwise; †ventricular septal defect (12), atrial septal defect (4) and patent ductus arteriosus (4); ‡the PCWP could not be successfully measured in eight patients at the first evaluation because of a markedly enlarged right atrium and severe tricuspid regurgitation, but these eight patients were identified as having a PCWP <15 mm Hg on repeated right heart catheterisation with the aid of a guidewire with a diameter of 0.025 inches (Cordis, Johnson & Johnson, USA) 14 (3) months later.
BSA, body surface area; CHD, congenital heart disease; CI, cardiac index; CTD, connective tissue disease; IPAH, idiopathic pulmonary arterial hypertension; mPAP, mean pulmonary artery pressure; 6MWD, 6 min walking distance; PAH, pulmonary arterial hypertension; PCWP, pulmonary capillary wedge pressure; Porto-PAH, portopulmonary hypertension; PVR, pulmonary vascular resistance; RAP, right atrial pressure; WHO, World Health Organization.