Therapy at diagnosis and last follow-up
| Strategy | n (%) |
| Initial treatment | |
| Medication class | |
| ERA | 66 (80) |
| PDE5i | 16 (20) |
| Specific medications | |
| Bosentan | 50 (61) |
| Sitaxsentan | 7 (9) |
| Macitentan | 5 (6) |
| Ambrisentan | 4 (5) |
| Sildenafil | 15 (18) |
| Tadalafil | 1(1) |
| Treatment at last follow-up | |
| Monotherapy | 68 (83) |
| Bosentan | 38 (46) |
| Sitaxsentan | 1 (1) |
| Macitentan | 8 (10) |
| Ambrisentan | 4 (5) |
| Sildenafil | 14 (17) |
| Tadalafil | 3 (4) |
| Combination therapy | 14 (17) |
| Ambrisentan+sildenafil | 8 (10) |
| Ambrisentan+tadalafil | 1 (1) |
| Macitentan+sildenafil | 4 (5) |
| Sitaxsentan+sildenafil | 1 (1) |
ERA: endothelin receptor antagonist; PDE5i: phosphodiesterase type-5 inhibitor.