Intravenous epoprostenol therapy in the treatment of pulmonary arterial hypertension: the Singapore experience

Abstract Funding Acknowledgements Type of funding sources: None. Background Pulmonary arterial hypertension (PAH) is a progressive disease with significant morbidity and mortality. While intravenous (IV) Epoprostenol, a prostacyclin analogue, has been shown to improve exercise tolerance, symptoms, hemodynamics and survival, there are challenges with initiation and maintenance of this IV therapy. Purpose We aim to describe our local experience of the use of IV Epoprostenol in the treatment of PAH patients in Singapore, highlighting various issues and challenges. Methods From 2016, patients at a tertiary cardiac institution diagnosed with Group 1 PAH and remaining in intermediate to high risk class with progressive symptoms (despite being on maximum tolerable doses of PhosphoDiEsterase-5 inhibitors and Endothelin-1 receptor antagonists) were assessed and counselled for initiation of Epoprostenol therapy. With a fixed set of local protocols, comprehensive assessment and support of a multi-disciplinary team including physicians, specialist nurses and pharmacists, suitable patients were started on this treatment. Results A total of 12 patients (11 female, mean age 42.8 +/- 11.0 years) were included. The average New York Heart Association class of the patients initiated on Epoprostenol was II-III. The pulmonary artery pressures and pulmonary vascular resistance on right heart catheterization prior to initiation was 52.5 (IQR 47.0-54.0) mmHg and 12.6 (IQR 10.0-14.2) Woods respectively. The duration from diagnosis to time of initiating Epoprostenol was 89 (IQR 62-140) months. Epoprostenol was generally well tolerated. The most common side effect experienced was diarrhea (5/12 patients) followed by headache and musculoskeletal complains (3/12 patients each). Of the 12 patients, 6 passed away after 12 (IQR 10-16) months of Epoprostenol therapy. Of the remaining 6, PAH was diagnosed 110 (IQR 104-136) months ago and Epoprostenol therapy has been continued for 17 (IQR 14-27) months. Of those who survived, right ventricle size and PA pressures on echocardiography remained relatively stable as compared to those who passed on. 2 patients had line related infections requiring a line change 1 and 2 times respectively. Conclusion While not without its challenges, the establishment of concrete protocols with the support of a multidisciplinary team allows for the introduction of IV Epoprostenol as an additional potential line of effective therapy for PAH patients in Singapore.