TABLE 1

Demographics and clinical characteristics of the PAH Biobank cohort

DemographicsOverallCTD-PAHIPAH
Subjects n2017623870
Age years55±1559±1455±15
Sex, n female (%)1611 (80)565 (91)698 (80)
Genetic ancestry, n EA (%)1662 (82)564 (91)780 (90)
Aetiology n FPAH/PVOD/PortoPulm/Congenital/Drug/HIV/Other81/8/111/171 /93/42/18
NYHA FC, n I/II/III/IV (% III/IV)90/451/789/118 (45)24/140/266/34 (65)38/188/340/56 (64)
6MWD m347±141327±160351±136
Deaths n (%)324 (16)125 (20)112 (13)
Biomarkers
 Endostatin pg·mL−1 median (IQR)37 515 (27 946–50 901)41 504 (31 639–55 487)37 087 (27 856–50 028)
 NTproBNP pg·mL−1 median (IQR)672 (217–2164)907 (331–3077)520 (183–1621)
Haemodynamics
 RAP mmHg9±59±59±6
 mPAP mmHg50±1544±1151±14
 PAWP mmHg10±410±410±4
 PVR Wood units10±68±510±6
 Cardiac output L·min−14.7±1.74.7±1.64.6±1.6
 Cardiac index L·min−1·m−22.7±1.22.8±0.92.6±1.1
Therapies n (%)
 PDE5 inhibitor1546 (77)470 (75)641 (74)
 ERA1205 (60)370 (59)515 (59)
 IV/SC prostacyclin699 (35)161 (26)355 (41)
 CCB199 (10)51 (8)99 (11)

Data are presented as mean±sd, unless otherwise indicated. PAH: pulmonary arterial hypertension; CTD-PAH: connective tissue disease-associated PAH; IPAH: idiopathic PAH; EA: European ancestry; FPAH: familial PAH; PVOD: pulmonary veno-occlusive disease; Portopulm: portopulmonary hypertension; NYHA FC: New York Heart Association Functional Class; 6MWD: 6-min walking distance; IQR: interquartile range; NTproBNP: N-terminal pro-brain natriuretic peptide; RAP: right atrial pressure; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; PDE5: phosphodiesterase-5; ERA: endothelin receptor antagonist; IV: intravenous; SC: subcutaneous; CCB: calcium channel blocker.