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Characteristics of medications used in the treatment of pulmonary hypertension

Characteristics of medications used in the treatment of pulmonary hypertension
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 one to two 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 one to two weeks until therapeutic response or dose limiting toxicity occurs
4 hours
Inhaled

One to three inhalations (ie, 6 to 18 micrograms), four times daily initially

Maintenance dose may be gradually titrated up to nine inhalations (ie, 54 micrograms), four times daily
4 hours
Iloprost Inhaled 2.5 to 5 micrograms, six to nine 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 one to two 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, two 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 three times daily, titrated up by 0.5 mg three times per day every two weeks until therapeutic response or dose limiting toxicity occurs (maximum dose 2.5 mg three times daily) 12 hours
Phosphodiesterase type 5 inhibitors
Sildenafil Oral 20 mg, three times daily 4 hours
IV

10 mg, three 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
Drugs used to treat pulmonary hypertension (PH) should only be administered by PH experts. For patients on medications for other conditions, specific drug interactions and management suggestions may be determined by using Lexi-Interact, the drug interactions program included with UpToDate.
* 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.
Data from:
  1. Montani D, Gunther S, Dorfmuller P, et al. Pulmonary arterial hypertension. ORJD 2013; 8:97.
  2. Coghlan G, Geaine S, Gatzoulis M, et al. Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. Thorax 2008; 63:ii1.
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