Table 2.
Registry | Switzerland20 | UK/Ireland21 | COMPERA23b | |||
---|---|---|---|---|---|---|
Cohort | Diagnosis before 2000 | Diagnosis 2009–2012 | ≤50 years | >50 years | 18–65 years | >65 years |
Number of patients | 24 | 171 | NP | NP | 209 | 378 |
Age (years), mean (SD) | 42 (16) | 60 (15) | 36.5 (9.3) | 65.1 (8.3) | 54 (16)c | 75 (8)c |
Female (%) | 66.7 | 55.6 | 73.2 | 66.5 | 69.4 | 55.3 |
Time from first symptoms to diagnosis (months), median (IQR) | NP | NP | 12 (6–24) | 24 (12–36) | NP | NP |
6MWD (m), mean (SD) | 341 (120) | 366 (139) | 330 (119) | 246 (112) | 340 (131) | 266 (114) |
Haemodynamics, mean (SD) | ||||||
Cardiac index (L/min−1/m2) | 2.6 (0.9) | 2.6 (0.8) | 2.1 (0.7) | 2.1 (0.7) | 2.2 (0.7) | 2.2 (0.7) |
RAP (mmHg) | 9 (4) | 9 (8) | 10.0 (5.8) | 10.2 (6.1) | 9 (5) | 8 (5) |
mPAP (mmHg) | 55 (18) | 46 (12) | 57.2 (14.9) | 51.0 (12.2) | 50 (13) | 41 (10) |
PVR (Wood units) | 10.1 (5.4) | 8.5 (5.2) | 13.9 (6.7) | 11.8 (5.8) | 12.0 (6.3) | 8.3 (4.5) |
6MWD, 6-min-walk distance; COMPERA, comparative, prospective registry of newly initiated therapies for pulmonary hypertension; IQR, interquartile range; mPAP, mean pulmonary arterial pressure; NP, not presented; PVR, pulmonary vascular resistance; RAP, right atrial pressure; SD, standard deviation.
PAH-only where reported, IPAH otherwise.
Austria, Belgium, Germany, Italy, the Netherlands, Switzerland, and UK.
Median (interquartile range).