Demographics and clinical characteristics of the overall cohort and connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) and idiopathic pulmonary arterial hypertension (IPAH) subgroups
| Overall | CTD-PAH | IPAH | p-value# | |
| Demographics | ||||
| Subjects | 2017 | 623 | 870 | |
| Age years | 55±15 | 59±14 | 55±15 | <0.01 |
| Female | 1611 (80) | 565 (91) | 698 (80) | <0.01 |
| Race white | 1662 (82) | 564 (91) | 780 (90) | ns |
| NYHA FC I/II/III/IV (III/IV) | 90/451/789/118 (45) | 24/140/266/34 (48) | 38/188/340/56 (45) | ns |
| 6MWD m | 347±141 | 327±160 | 351±136 | <0.01 |
| BMI kg·m−2 | 30±10 | 29±12 | 31±9 | <0.01 |
| Deaths | 338 (17) | 138 (22) | 110 (13) | <0.01 |
| Aetiology | ||||
| CTD-PAH | 623 | |||
| IPAH | 870 | |||
| FPAH | 81 | |||
| PVOD-PAH | 8 | |||
| PoPH-PAH | 111 | |||
| Congenital | 171 | |||
| Drug-PAH | 93 | |||
| HIV-PAH | 42 | |||
| Other | 18 | |||
| IL-6 pg·mL−1 | 1.82 (0.86–3.34) | 2.24 (1.09–4.33) | 1.62 (0.72–2.94) | <0.01 |
| Haemodynamics | ||||
| RAP mmHg | 9±5 | 9±5 | 9±6 | <0.01 |
| mPAP mmHg | 50±15 | 44±11 | 51±14 | <0.01 |
| PAWP mmHg | 10±4 | 10±4 | 10±4 | ns |
| PVR Wood units | 10±6 | 8±5 | 10±6 | <0.01 |
| Cardiac output L·min−1 | 4.7±1.7 | 4.7±1.6 | 4.6±1.6 | 0.01 |
| Cardiac index L·min−1·m−2 | 2.7±1.2 | 2.8±0.9 | 2.6±1.1 | <0.01 |
| Therapies | ||||
| PDE-5 inhibitor | 1546 (77) | 470 (75) | 641 (74) | ns |
| ERA | 1205 (60) | 370 (59) | 515 (59) | ns |
| Intravenous/subcutaneous prostacyclin | 699 (35) | 161 (26) | 355 (41) | <0.01 |
| CCB | 199 (10) | 51 (8) | 99 (11) | 0.05 |
Data are presented as n, mean±sd, n (%) or median (interquartile range), unless otherwise stated. NYHA FC: New York Heart Association functional class; 6MWD: 6-min walk distance; BMI: body mass index; FPAH: familial PAH; PVOD-PAH: pulmonary veno-occlusive disease-associated PAH; PoPH-PAH: portopulmonary hypertension-associated PAH; IL: interleukin; RAP: right atrial pressure; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary artery wedge pressure; PVR: pulmonary vascular resistance; PDE: phosphodiesterase; ERA: endothelin receptor antagonist; CCB: calcium channel blocker; ns: nonsignificant. #: p-values reflect comparisons between CTD-PAH and IPAH.