4CPS-223 Real world experience of selexipag for pulmonary arterial hypertension

Background and importance Pulmonary arterial hypertension (PAH) is a life threatening disease causing an increment in pulmonary vascular resistance which leads to right ventricular failure and death. Selexipag is an oral selective IP prostacyclin receptor agonist, shown to be beneficial in the treatment of PAH, reducing morbidity and mortality in these patients. It was approved by AEMPS in 2015. Aim and objectives The aim of this study was to evaluate medication adherence and the evolution of baseline characteristics, NT-proBNP, functional class (WHO-FC) and non-invasive studies, such as the 6 min walking test, after starting treatment with selexipag. The risk stratification before and after starting treatment with selexipag was also analysed. Material and methods Since the inclusion of selexipag in our therapeutic guide in November 2017 up until April 2020, seven patients (85.7% women) have received the drug. Adherence was calculated based on dispensation records, and the other parameters of the study were monitored at every visit. Results All patients had PAH, three of them associated with a congenital heart defect (two with Eisenmenger’s syndrome due to an uncorrected heart defect and one with a corrected heart defect). All had been treated with phosphodiesterase-5 inhibitors (PDE5-I) and endothelin receptor antagonists (ERAs) before starting selexipag. Two patients were in a low risk situation (switch from inhaled treprostinil to selexipag), four patients were in an intermediate risk situation and one patient was in a high risk situation, who after the titration phase was changed to epoprostenol because of persistence of the high risk situation. After the titration phase, one patient in the intermediate risk group changed to a low risk situation. We found that treatment with selexipag reduced the WHO-FC, the 6 min walking test, NT-proBNP and right heart failure symptoms. Regarding adherence, median medication adherence was 97%: five patients had 100% adherence to the treatment and the others showed 98% and 80% adherence. 50% of patients received a high dose of selexipag, and the most common side effects were diarrhoea and muscle pain. Conclusion and relevance The use of selexipag in our clinical practice in patients with PAH improved risk parameters (functional class and the 6 min walking test) and right ventricle systolic function. Patients showed high rates of adherence and good tolerance to treatment with a low incidence of side effects. References and/or acknowledgements Conflict of interest No conflict of interest