| ERA# | PDE-5 inhibitor¶ | Prostacyclin analogue | |||
| Intravenous epoprostenol | Inhaled iloprost | Treprostinil+ | |||
| Overall use§ | 1147 (47.0) | 1194 (49.0) | 480 (19.7) | 237 (9.7) | 307 (12.6) |
| Monotherapy | 452 (18.5) | 417 (17.1) | 188 (7.7) | 23 (0.9) | 84 (3.4) |
| Combination with one oral therapyƒ | 291 (11.9) | 290 (11.9) | 243 (10.0) | 138 (5.7) | 148 (6.1) |
| Combination with one prostacyclin analogue | 224 (9.2) | 305 (12.5) | 2 (0.1) | 3 (0.1) | 5 (0.2) |
| Combination with >1 other therapy | 180 (7.4) | 182 (7.5) | 47 (1.9) | 73 (3.0) | 70 (2.9) |
| NYHA/WHO functional class I/II | |||||
| Overall use | 468 (47.1) | 474 (47.7) | 187 (18.8) | 71 (7.1) | 111 (11.2) |
| Monotherapy | 216 (21.7) | 187 (18.8) | 83 (8.4) | 4 (0.4) | 26 (2.6) |
| Combination with one oral therapyƒ | 110 (11.1) | 109 (11.0) | 85 (8.6) | 41 (4.1) | 60 (6.0) |
| Combination with one prostacyclin analogue | 76 (7.6) | 110 (11.1) | |||
| Combination with >1 other therapy | 66 (6.6) | 686 (6.8) | 19 (1.9) | 26 (2.6) | 25 (2.5) |
| NYHA/WHO functional class III | |||||
| Overall use | 525 (47.7) | 567 (51.5) | 218 (19.8) | 130 (11.8) | 161 (14.6) |
| Monotherapy | 181 (16.4) | 177 (16.1) | 80 (7.3) | 16 (1.5) | 43 (3.9) |
| Combination with one oral therapyƒ | 147 (13.4) | 147 (13.4) | 117 (10.6) | 78 (7.1) | 79 (7.2) |
| Combination with one prostacyclin analogue | 114 (10.4) | 160 (14.5) | 2 (0.2) | 2 (0.2) | 4 (0.4) |
| Combination with >1 other therapy | 83 (7.5) | 83 (7.5) | 19 (1.7) | 34 (3.1) | 35 (3.2) |
| NYHA/WHO functional class IV | |||||
| Overall use | 55 (44.4) | 61 (49.2) | 44 (35.5) | 14 (11.3) | 15 (12.1) |
| Monotherapy | 5 (4.0) | 14 (11.3) | 17 (13.7) | 2 (16) | 5 (4.0) |
| Combination with one oral therapyƒ | 16 (12.9) | 16 (12.9) | 21 (16.9) | 8 (6.5) | 4 (3.2) |
| Combination with one prostacyclin analogue | 18 (14.5) | 15 (12.1) | |||
| Combination with >1 other therapy | 16 (12.9) | 16 (12.9) | 6 (4.8) | 4 (3.2) | 6 (4.8) |
Data are presented as n (%). Combinations with one oral therapy, with one prostacyclin analogue, and with more than one oral therapy are mutually exclusive and exclude calcium channel blockers. Blinded clinical trial patients are excluded from this presentation (n=87). ERA: endothelin receptor antagonist; PDE-5: phosphodiesterase type-5; NYHA/WHO: New York Heart Association/World Health Organization. #: 953 on bosentan, 106 on sitaxsentan and 89 on ambrisentan; ¶: 1,147 on sildenafil and 47 on tadalafil; +: treprostinil use includes 159 on intravenous, 112 on subcutaneous, 28 on inhaled and nine on oral treprostinil; §: n=2,435; ƒ: oral therapy is defined as bosentan, sildenafil, ambrisentan, sitaxsentan and tadalafil. Reproduced from [15] with permission from the publisher.