IVI epoprostenol as salvage therapy in pulmonary arterial hypertension: an Australian perspective

Abstract

Background: IVI epoprostenol is the only therapy for pulmonary arterial hypertension (PAH) with a randomized controlled trial demonstrating improved survival, when used as first-line monotherapy. In Australia it is used as salvage therapy for those failing treatment with other targeted therapies or presenting in World Health Organization functional class (FC) IV.

Aims: Report experience with IVI epoprostenol, administered as salvage therapy for the treatment of adults with PAH in a single Australian PAH centre.

Methods: Retrospective case series of all patients commenced on IVI epoprostenol for PAH, between 2002 and 2010. Review of case notes with collection of data at baseline and after treatment, including FC, 6-min walk test (6MWT), right ventricular systolic pressure (RVSP) on echocardiogram, patient survival and treatment complications. Change in indices was assessed using the Wilcoxon Sign Rank Test and is expressed as median (inter-quartile range).

Results: A total of 23 patients was included. Treatment was generally well tolerated with few major complications. At the end of the study period, nine patients were successfully bridged to transplant, five had a sustained response to IVI epoprostenol, six had an incomplete response but were clinically stabilized, two died awaiting transplant and one died who was not a candidate for transplantation. Overall, when measured at best level post initiation of IVI epoprostenol, there were significant improvements in FC -1 [0 to -1] (P < 0.0001), 6MWT (m) +117 [70-264] (P= 0.002) and RVSP (mmHg) -7.0 [4.0 to -45] (P= 0.03).

Conclusion: Findings support efficacy of epoprostenol as salvage therapy for patients with PAH.