Table 1

Cohort demographics and therapies

Congenital (n=44)Non-congenital (n=26)p Value
Age (years)
 Mean (SD)39 (8.7)64 (14.2)<0.001
 Median (IQR)39 (32.0–44.8)65 (56.5–74.0)
Sex (% men)39150.040
Mortality (% dead)30310.914
Diuretics (%)24500.038
Anticoagulants (%)26270.90
Treated (%)61100<0.001
Heart–lung transplant (%)740.605
Peak SPAP (mm Hg)
 Mean (SD)117 (14.2)110 (25.2)0.010
 Median (IQR)115 (110–124)103 (93–120)
6-min walk distance (m)
 Mean (SD)425 (105)370 (114)0.50
 Median (IQR)446 (351–485)369 (293–437)
WHO functional class (%)
 I90<0.001
 II5412
 III3583
 IV20
  • WHO functional class measured at time of initial referral to centre; 6-min walk distance represents average distance on most recent test. Data represented as percentages (%), mean with SD and/or median with IQR.