Medical Therapies for PAH
Drug Class
Table 5. Medical Therapies for PAH Dosage Form
Generic (Brand)
Prostanoids Epoprostenol (Flolan®
)b
Continuous, chronic IV infusion (2 ng/kg/min every 15 min or longer until dose-limiting pharmacologic effects are elicited or until a tolerance limit is established. Changes in infusion rate based on persistence, recurrence, or worsening of symptoms and adverse events due to excessive doses).
Comments
Indicated for the long-term intravenous treatment of primary pulmonary hypertension and pulmonary hypertension associated with the scleroderma spectrum of disease in NYHA Class III and Class IV patients who do not respond adequately to conventional therapy. Epoprostenol is administered by continuous intravenous infusion via a central venous catheter using an ambulatory infusion pump. The pump should be small and lightweight and be able to adjust infusion rates in 2 ng/kg/min increments.
Common Adverse Effects:a Flushing, headache, nausea/vomiting, hypotension, jaw pain; pain and local infection at injection site, sepsis (rare)
Caution: Patients should be referred to a center with experience in managing patients with PAH for proper dosing of this agent
Iloprost (Ventavis®
)
Aerosol (inhalation solution, 10 or 20 μg/mL in a 1 or 2 mL ampule, delivered through either of two pulmonary drug delivery devices: the I-neb® AAD® AAD®
Treprostinil (Remodulin®
Indicated for the treatment of pulmonary arterial hypertension (WHO Group I) in patients with NYHA Class III or IV symptoms.
(initial dose 2.5 μg, 6-9 times/ day—titrate to 5 μg, 6-9 times/ day, up to 45 μg/day max).
System or the Prodose® System)
)b
IV or subcut infusion (1, 2.5, 5, 10 mg/mL) (Initial dose: 1.25 ng/kg min; increments of 1.25 ng/kg min per wk for the first 4 wks of treatment, later 2.5 ng/kg/ min per wk). Because of the risks associated with chronic indwelling central venous catheters, including serious blood stream infections, continuous intravenous infusion should be reserved for patients who are intolerant of the subcutaneous route, or in whom these risks are considered warranted.
Common Adverse Effects:a Flushing, increased cough, headache, nausea, flu syndrome
Indicated for the treatment of pulmonary arterial hypertension in patients with NYHA Class II-IV symptoms to diminish symptoms associated with exercise.
Common Adverse Effects:a subcut infusion site pain, erythema, induration or rash; headache, diarrhea, nausea Other Adverse Effects:a Catheter-related line infections leading to blood stream infections and sepsis, which may be fatal (rare)
Caution: Therapy should be referred to a center with experience in managing patients with PAH for proper dosing of this agent.
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