Selection of Infusion Prostacyclin Therapy in Pulmonary Arterial Hypertension: Not Just a Last Resort

Pulmonary arterial hypertension (PAH) is a progressive, fatal vasculopathy that clinically manifests as increased pulmonary vascular resistance and elevation of pulmonary arterial pressures, ultimately leading to right heart failure and death. Median untreated survival period is 2.8 years, with a survival rate of 34% at 5 years before the availability of epoprostenol.1 Parenteral prostacyclin therapy is arguably the most effective and longest Food and Drug Administration-approved class of drugs for PAH and has been included in guidelines for treatment of PAH for almost 20 years. Intravenous epoprostenol as Flolan® remains the only drug that has demonstrated a survival advantage (Figure 1).2 Despite this demonstration of survival advantage and early evidence in its ability to improve a majority (70%) of patients to a point where they no longer required active listing for lung transplantation,3 epoprostenol or other infusion agents have consistently been shown to be withheld or underutilized in patients with advanced PAH.45 This apparent paradox as well as significant successful prostacyclin therapy in a variety of PAH patients6–8 forms the basis of our discussion on the role of infusion prostacyclins in modern management of PAH.