Characteristic of the overall population and of the four pathophysiologically defined echocardiographic risk groups
| Overall population | Group 1 | Group 2 | Group 3 | Group 4 | p-value | |
| Subjects | 517 | 129 | 155 | 101 | 132 | |
| Age years | 52±15 | 52±14 | 53±14 | 51±17 | 51±17 | 0.598 |
| Female | 316 (64.8) | 67 (57) | 102 (70) | 68 (68) | 79 (64) | 0.140 |
| Heart rate bpm | 78±14 | 74±13 | 76±14 | 80±15 | 80±13 | 0.004 |
| SBP mmHg | 123±19 | 124±17 | 121±15 | 123±26 | 125±18 | 0.832 |
| DBP mmHg | 72±11 | 74±11 | 68±9 | 71±11 | 74±13 | 0.109 |
| PAH aetiology | ||||||
| Idiopathic | 285 (64.8) | 75 (65) | 85 (59) | 50 (70) | 75 (68) | 0.057 |
| Congenital heart disease | 37 (8.4) | 7 (6) | 12 (8) | 10 (14) | 8 (7) | |
| Portal hypertension | 22 (5.0) | 9 (8) | 10 (7) | 1 (1) | 2 (2) | |
| HIV | 12 (2.7) | 3 (3) | 7 (5) | 1 (1) | 1 (1) | |
| PVOD | 11 (2.5) | 2 (2) | 4 (3) | 3 (4) | 2 (2) | |
| Scleroderma | 39 (8.9) | 5 (4) | 18 (13) | 5 (7) | 11 (10) | |
| Connective tissue disease | 23 (5.2) | 10 (9) | 4 (3) | 1 (2) | 8 (7) | |
| Other | 11 (2.5) | 4 (4) | 4 (3) | 0 (0) | 3 (3) | |
| Prevalent patients | 421 (81.4) | 118 (92) | 137 (88) | 75 (74) | 91 (69) | <0.001 |
| WHO FC III-IV | 339 (66.1) | 63 (49) | 103 (67) | 66 (66) | 107 (81) | <0.001 |
| 6MWD m | 358±131 | 405±107 | 353±128 | 351±137 | 317±144 | 0.007 |
| Vasoactive therapy | ||||||
| PDE5i | 287 (58.2) | 74 (58) | 91 (61) | 51 (56) | 71(57) | 0.896 |
| sGC | 10 (2.7) | 3 (4) | 2 (2) | 5 (5) | 0 (0) | 0.121 |
| ERA | 240 (48.7) | 64 (50) | 83 (55) | 36 (40) | 47 (46) | 0.105 |
| Prostanoids | 122 (23.6) | 31 (24) | 41 (27) | 20 (20) | 30 (23) | 0.670 |
| Monotherapy | 160 (32.4) | 45 (35) | 52 (34) | 32 (35) | 31 (25) | 0.246 |
| Combination therapy | 215 (43.5) | 55 (43) | 71 (47) | 34 (37) | 55 (44) | 0.531 |
| Centre location | ||||||
| Europe | 375 (72.7) | 86 (67) | 100 (64) | 92 (91) | 98 (74) | <0.001 |
| USA | 141 (27.3) | 43 (33) | 55 (36) | 9 (9) | 34 (26) | |
| Echocardiographic data | ||||||
| RVEDD mm | 38 (30–45) | 32 (5–40) | 37 (28–47) | 38 (34–46) | 42 (34–49) | <0.001 |
| RVEDA cm2 | 29±9 | 25±7 | 28±10 | 28±9 | 33±8 | <0.001 |
| FAC % | 27.1±12.2 | 30±10 | 30±12 | 26±10 | 23±14 | 0.001 |
| TAPSE mm | 18±5 | 22±4 | 22±4 | 14±2 | 13±3 | <0.001 |
| TR moderate to severe | 333 (65.0) | 0 (0) | 155 (100) | 66 (67) | 112 (87) | <0.001 |
| Dilated IVC | 196 (44.0) | 25 (26) | 39 (34) | 0 (0) | 132 (100) | <0.001 |
| PASP mmHg | 68±26 | 50±22 | 71±28 | 73±23 | 77±21 | <0.001 |
| AcT ms | 78±22 | 88±23 | 81±20 | 71±19 | 69±18 | <0.001 |
| RA area# cm2 | 21 (17–26) | 19 (15–23) | 20 (17–24) | 20 (16–25) | 29 (24–36) | <0.001 |
| Pericardial effusion¶ % | 32 (27.6) | 7 (35) | 9 (24) | 4 (25) | 12 (29) | 0.822 |
| LVEDV mL | 65 (50–81) | 80 (63–95) | 70 (62–90) | 64 (47–80) | 54 (43–67) | <0.001 |
| LVEF % | 68±12 | 69±12 | 68±11 | 68±13 | 65±12 | 0.016 |
| Year of study publication | ||||||
| 2006–2011 % | 71 (13.7) | 18 (14) | 19 (12) | 9 (9) | 25 (19) | 0.152 |
| 2016–2018 % | 446 (86.3) | 111 (86) | 136 (88) | 92 (91) | 107 (81) | |
| Patient enrolment | ||||||
| Prospective % | 187 (36.2) | 50 (39) | 60 (39) | 18 (18) | 59 (45) | <0.001 |
| Retrospective % | 330 (63.8) | 79 (61) | 95 (61) | 83 (82) | 73 (55) |
Data are presented as n, mean±sd, n (%) or median (interquartile range), unless otherwise stated. All percentages are calculated based on data availability for each parameter. SBP: systolic blood pressure; DBP: diastolic blood pressure; PAH: pulmonary arterial hypertension; PVOD: pulmonary veno-occlusive disease; WHO FC: World Health Organization functional class; 6MWD: 6-min walk distance; PDE5i: phosphodiesterase-5 inhibitor; sGC: soluble guanylate cyclase stimulator; ERA: endothelin receptor antagonist; RVEDD: right ventricular end-diastolic diameter; RVEDA: right ventricular end-diastolic area; FAC: fractional area change; TAPSE: tricuspid annular plane systolic excursion; TR: tricuspid regurgitation; IVC: inferior vena cava; PASP: pulmonary arterial systolic pressure; AcT: acceleration time; RA: right atrial; LVEDV: left ventricular end-diastolic volume; LVEF: left ventricular ejection fraction. #: data available for 314 patients; ¶: data available for 116 patients.