Effectiveness of epoprostenol in the management of pulmonary arterial hypertension: findings of an Australian retrospective chart review

Background: Pulmonary arterial hypertension (PAH) is a progressively fatal disease. Parenteral prostanoids, including intravenous (IV) epoprostenol, are the most effective therapies for PAH. As epoprostenol requires continuous infusion, therapy is challenging and use is managed by specialist units. Aims: To describe the clinical outcomes for the use of IV epoprostenol in an Australian pulmonary vascular disease unit and identify opportunities to improve its use. Method: We conducted a retrospective chart review of all adult patients who received IV epoprostenol for PAH at Prince Charles Hospital. Data were collected at baseline, initiation of epoprostenol and quarterly to the end of the audit period or the discontinuation of epoprostenol. Descriptive statistics were performed and comparisons made to epoprostenol initiation. Kaplan-Meier curves were used to estimate survival outcomes. Results: Thirty-nine patients received epoprostenol therapy. Most (87.2%) were female, had connective tissue disease (46.2%) or idiopathic PAH (35.9%) and had a World Health Organization functional Class of III or IV at initiation. The mean duration of epoprostenol therapy was 2.9 years. Intravenous epoprostenol was associated with favourable outcomes; 1, 3 and 5-year survival rates since initiation of 90.0, 75.8 and 68.2%; robust improvements in functional class; and improvements in 6-min walking distance of 160.4 m at 3 years; 94.9% patients experienced at least one drug-related adverse event and line complications were common. Conclusion: Epoprostenol therapy was associated with impressive survival rates and durable improvements in functional outcomes. Epoprostenol therapy is challenging; however, it is manageable by the majority of patients with most experiencing positive, sustainable outcomes.