Version May 2024
| Drug | Route of administration | Dose range (adult) | Half-life |
| Prostacyclin pathway agonists | |||
| Epoprostenol | Continuous IV infusion via central venous catheter | 1 to 12 nanograms/kg/minute initially Dose titrated up every 1 to 2 weeks until therapeutic response or dose limiting toxicity occurs | 3 to 5 minutes (single dose) 15 minutes (continuous infusion) |
| Treprostinil | Continuous IV infusion via central venous catheter or continuous subcutaneous infusion | 0.625 to 1.25 nanograms/kg/minute initially Dose titrated up every 1 to 2 weeks until therapeutic response or dose limiting toxicity occurs | 4 hours |
| Inhaled | 1 to 3 inhalations (ie, 6 to 18 micrograms), 4 times daily initially Maintenance dose may be gradually titrated up to 9 inhalations (ie, 54 micrograms), 4 times daily | 4 hours | |
| Iloprost | Inhaled | 2.5 to 5 micrograms, 6 to 9 times daily | 20 to 30 minutes (half-life of pulmonary vasodilating effect) |
| Selexipag | Oral Intravenous formulation is available for temporary use when unable to take oral therapy | 200 to 1600 micrograms twice daily Dose titrated up every 1 to 2 weeks until therapeutic response or dose limiting toxicity occurs For intravenous formulation, see labeling for dosing | 0.8 to 2.5 hours (selexipag) 6.2 to 13.5 hours (active metabolite) |
| Endothelin receptor antagonists | |||
| Bosentan | Oral | 62.5 to 125 mg, 2 times daily Dose is adjusted for low body weight or drug interactions* | 5 hours |
| Ambrisentan | Oral | 5 to 10 mg daily | 9 hours |
| Macitentan | Oral | 10 mg per day | 14 to 18 hours (parent drug) |
| Nitric oxide-cyclic guanosine monophosphate enhancers | |||
| Soluble guanylate cyclase stimulant | |||
| Riociguat | Oral | Initial dose 0.5 to 1 mg 3 times daily, titrated up by 0.5 mg 3 times per day every 2 weeks until therapeutic response or dose limiting toxicity occurs (maximum dose 2.5 mg 3 times daily) | 12 hours |
| Phosphodiesterase type 5 inhibitors | |||
| Sildenafil | Oral | 20 mg, 3 times daily | 4 hours |
| IV | 10 mg, 3 times daily Dose is adjusted for drug interactions* | 4 hours | |
| Tadalafil | Oral | 40 mg daily Dose is adjusted for drug interactions* | 35 hours |
| Calcium channel blockers¶ | |||
| Nifedipine | Oral | Start 30 mg per day. Increase to the maximum tolerated dose over days to weeks. | 7 hours |
| Diltiazem extended-release | Oral | Start 120 mg per day. Increase to the maximum tolerated dose over days to weeks. | 6 to 9 hours |
| Amlodipine | Oral | Start 2.5 mg per day. Increase to the maximum tolerated dose over days to weeks. | 30 to 50 hours |
PH: pulmonary hypertension.
* Refer to individual Lexicomp drug monographs included with UpToDate.
¶ Choice of calcium channel blocker is based upon the patient's heart rate. Refer to text.آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟
