Safe and effective use of prostacyclins to treat pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a progressive, incurable disease of the small pulmonary arteries that results in right ventricular failure and, ultimately, death if the patient does not receive a lung transplant.1 Significant advances in the management of PAH over the past two decades have been made as a result of the increased availability of novel medications and delivery systems to treat this disease. There are currently nine Food and Drug Administration (FDA)-approved therapies for the management of PAH including phosphodiesterase type-5 inhibitors, endothelin-receptor antagonists, and prostacyclin agonists. The parenteral prostacyclins, including epoprostenol and treprostinil, are recommended as first-line therapies for patients at higher risk of death based on clinical assessment.1 Even patients who initially present as lower risk and are treated with oral or inhaled therapies often require parenteral prostacyclins as their disease progresses.1 Because of the acuity of this patient population, complexity of the medical regimens used, and significant medication costs (generally in excess of $100,000 annually for prostacyclin therapy), pharmacists should serve an integral role in ensuring successful outcomes for these patients.