TABLE 1

Baseline demographics

Demographic variablePooled population
Sex M/F (n)10/19
Age years (mean±sd)54.9±11.4
IPAH/FPAH/CTD (n)15/4/10
PVR dyn·s·cm−5 (mean±sd)612.30±317.63
mPAP mmHg (mean±sd)43.57±11.07
NT-proBNP pg·mL−1 (median (IQR))329.50 (549)
Cardiac output L·min−1 (median (IQR))5.14 (2.18)
PAH medication (%)
 Ambrisentan/bosentan/macitentan17.2/6.9/3.4
 Sildenafil/tadalafil/riociguat58.6/13.8/11.1
6MWT m (mean±sd)425.9±112.1
WHO class II/III (%)48.3/51.7

M: male; F: female; IPAH: idiopathic pulmonary hypertension; FPAH: familial pulmonary hypertension; CTD: connective tissue disease; PVR: pulmonary vascular resistance; mPAP: mean pulmonary arterial pressure; NT-proBNP: N-terminal pro-brain natriuretic peptide; IQR: interquartile range; 6MWT: 6-min walk test; WHO: World Health Organization.