Epoprostenol Sodium and High Cholesterol (Hyperlipoproteinemia, Hypertriglyceridemia, Sitosterolemia)

• Generic Name: epoprostenol
• Dosage Forms: n.a.
• Other Brand Names: Flolan, Veletri

Management of pulmonary arterial hypertension (PAH; WHO group 1) to improve exercise capacity; efficacy established principally in patients with NYHA/WHO functional class III or IV PAH (idiopathic, heritable, or associated with connective tissue diseases). Has been designated an orphan drug by FDA for treatment of PAH.

Recommended as one of several treatment options for initial management of PAH in patients with NYHA/WHO functional class III or IV symptoms who are not candidates for calcium-channel blocker therapy or in whom such therapy failed. Generally considered treatment of choice for patients with NYHA/WHO class IV PAH because of demonstrated benefit on survival.

Individualize choice of PAH therapy; consider factors such as disease severity, route of administration, potential adverse effects and costs of treatment, clinician experience, and patient preference.

In patients with inadequate response to initial monotherapy, may consider combination therapy with an endothelin-receptor antagonist, phosphodiesterase (PDE) type 5 inhibitor, or soluble guanylate cyclase stimulator (added sequentially). Such combination therapy may provide additive and/or synergistic benefits by targeting different pathophysiologic pathways of disease.

Safety and efficacy not systematically evaluated in patients with pulmonary hypertension associated with diseases other than scleroderma.