eTable 1. Drugs licensed for the treatment of pulmonary arterial hypertension in Germany.
| Substance group | Substance | Approved dosage and route of administration | Reference(s) |
|
Endothelin receptor antagonists |
Ambrisentan | 1 × 5 mg/day or 1 × 10 mg/day orally | (12, 28) |
| Bosentan | Initial dosage 2 × 62.5 mg/day, Target dosage 2 × 125 mg/day orally |
(29– 31) | |
| Macitentan | 1 × 10 mg/day orally | (13) | |
| PDE5 inhibitors | Sildenafil | 3 × 20 mg/day orally | (32) |
| Tadalafil | 1 × 40 mg/day orally | (12, 33) | |
|
Stimulator of soluble guanylate cyclase |
Riociguat | Initial dosage 3 × 1.0 mg/day, Target dosage 3 × 2.5 mg/day orally |
(34, 35) |
| Prostacyclin analogs | Epoprostenol (classic form and thermostable variant) |
Individual dosing, target dosage usually 20–50 ng/kg/min IV |
(36) |
| Iloprost | 2.5 µg or 5 µg 6–9 ×/day by inhalation |
(37) | |
| Treprostinil | Individual dosing, target dosage usually 20–50 ng/kg/min IV or SC |
(38) | |
|
Prostacyclin receptor agonist |
Selexipag | Individual dosing, initial dosage 2 × 200 µg/day, maximum dosage 2 × 1600 µg/day |
(39) |