Transitioning Between Oral And Parenteral Prostacyclin Therapy In Two Patients With Idiopathic Pulmonary Arterial Hypertension

Title: Transitioning Between Oral And Parenteral Prostacyclin Therapy In Two Patients With Idiopathic Pulmonary Arterial Hypertension
Author(s): S Devlin (first author) S Cullivan B McCullagh SP Gaine
Institution: National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital
Poster: Click to view poster
Category: Miscellaneous
Abstract: Current therapies for pulmonary arterial hypertension (PAH) target the nitric oxide, endothelin and prostacyclin pathways to promote pulmonary vasodilation and reduced right ventricular afterload and failure. Agents that utilise the latter pathway include the prostacyclin analogue epoprostenol, treprostinil, iloprost and selexipag, a selective prostacyclin (IP) receptor agonist. As more medications become available, transitioning between agents is increasingly performed. While clear guidelines exist for initiation of individual drugs, equivalent guidelines for transition between medications are often lacking.

We describe two cases of transition from oral selexipag to subcutaneous treprostinil in patients with idiopathic PAH. Following an average of 34.5 months on selexipag, the decision was made to transition to parenteral prostacyclin due to NYHA functional class III symptoms and intermediate risk features. The patients were admitted for in-hospital transitions, whereby treprostinil uptitrations could be guided by clinical response and prostacyclin-related side effects. Overall, these transitions were well tolerated, taking place over an average of 11 days. Regarding long-term outcomes, Patient 1 experienced a progressive decline in symptoms and was listed for double lung transplantation, but died 2 years following this transition. Patient 2 remains stable with NYHA FC III symptoms, 4 years later.

Our patient-centred approach, guided by prostacyclin side-effects, was both safe and well-tolerated, providing an example of a safe method of transitioning from oral selexipag to subcutaneous treprostinil.