Patient characteristics
| IPAH (n=13)* | CTD-PAH (n=9)† | CHD-PAH (n=7)‡ | |
| Gender (male) | 3 (23%) | 1 (11%) | 5 (71%) |
| Age (years) | 50 (33–60) | 71 (50–74) | 41 (26–40) |
| Time to diagnosis (months) | 40 (12–80) | 6 (3–15) | 1 (1–12) |
| Right heart catheterisation | |||
| mPAP (mm Hg) | 58 (47–78) | 37 (31–52) | 43 (38–61) |
| RAP (mm Hg) | 13 (8–19) | 8 (6–11) | 8 (6–9) |
| PAWP (mm Hg) | 13 (11–17) | 9 (5–13) | 12 (10–16) |
| CI (L/min/m2) | 2.4 (1.6–2.8) | 3.1 (2.4–3.6) | 3.3 (2.9–3.5) |
| CO (L/min) | 4.2 (2.7–5.1) | 5.6 (4.8–6.9) | 6.0 (4.1–7.4) |
| PVR (dynes s/cm5) | 1025 (628–1430) | 371 (189–830) | 426 (293–577) |
| SvO2 (%) | 60 (56–73) | 63 (61–75) | 70 (60–79) |
| Functional parameters | |||
| NYHA functional class 1 (n) | 0 (0%) | 0 (0%) | 1 (14%) |
| NYHA functional class 2 (n) | 5 (38%) | 4 (44%) | 2 (28%) |
| NYHA functional class 3 (n) | 7 (54%) | 4 (44%) | 4 (57%) |
| NYHA functional class 4 (n) | 1 (8%) | 1 (11%) | 0 (0%) |
| 6MWD (m) | 320 (226–365) | 289 (267–432) | 452 (263–592) |
| NT proBNP (μmol/L) | 69 (32–203) | 69 (15–79) | 34 (11–43) |
| Vasoactive medication (n)§ | 10 (77%) | 5 (56%) | 0 (0%) |
Healthy subject characteristics: n=19, gender (male) = 6 (32%), age (years) = 48 (30–52). Continuous variables are presented as median and IQR in parentheses and categorical variables as counts and percentages in parentheses.
*Genetic testing revealed that two patients had a BMPR2 gene mutation.
†Seven patients with rheumatoid arthritis, one patient with systemic sclerosis, one patient with systemic lupus erythematosus.
‡Five patients with atrial septal defect, one patient with Eisenmenger’s syndrome and ventricular septal defect, one patient with major aortopulmonary collateral arteries.
§Vasoactive medication at the time when blood sample was taken.
CHD-PAH, congenital heart disease associated pulmonary arterial hypertension; CI, cardiac index; CO, cardiac output; CTD-PAH, connective tissue disease associated pulmonary arterial hypertension; IPAH, idiopathic pulmonary arterial hypertension; mPAP, mean pulmonary arterial pressure; 6MWD, 6 min walk distance; NYHA, New York Heart Association; PAWP, pulmonary arterial wedge pressure; PVR, pulmonary vascular resistance; RAP, right atrial pressure; SvO2, central mixed venous oxygen saturation.