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 drug monographs included within UpToDate.
¶ Choice of calcium channel blocker is based upon the patient's heart rate. Refer to text.Do you want to add Medilib to your home screen?