Rapid transition from oral selexipag to parenteral treprostinil in a patient with mixed-etiology pulmonary hypertension

Purpose: Selexipag is an oral nonprostanoid IP prostacyclin receptor agonist that is indicated for treatment of pulmonary arterial hypertension (PAH). In patients with continued symptoms of PAH despite maximized oral therapy with selexipag and other oral therapies, a transition to parenteral prostacyclin may be warranted. There is a paucity of data regarding how to safely transition from oral selexipag to parenteral treprostinil. We describe rapid transition from oral selexipag to parenteral treprostinil in this case report.

Summary: A 65-year-old female with mixed-etiology PAH as result of pulmonary fibrosis related to polymyositis was admitted to the intensive care unit to be transitioned from selexipag to treprostinil due to dyspnea at rest despite therapy with selexipag 1,600 mg twice daily and macitentan 10 mg daily for 3 years. At baseline the patient required oxygen support (4 L/min) at rest to maintain oxygen saturation at or above 90%. Right heart catheterization performed 8 weeks prior to admission revealed severe PAH, with a pulmonary arterial pressure of 73/27 mm Hg and pulmonary vascular resistance of 10 Wood units. On the day of admission the patient was given selexipag 800 µg at 9 am and simultaneously started on intravenous (i.v.) treprostinil at a dose of 2 ng/kg/min. The treprostinil dose was increased by 2 ng/kg/min every 3 hours until a target dose of 22 ng/kg/min was achieved, at which point the patient had experienced dyspnea improvement. She experienced a mild headache and flushing during rapid treprostinil dose escalation. After 30 hours of i.v. treprostinil infusion, the patient was transitioned to subcutaneous treprostinil therapy and discharged.

Conclusion: In this case the patient was rapidly transitioned from oral selexipag to i.v. and then subcutaneous treprostinil therapy over a 30-hour period, with minimal adverse effects.

Keywords: pulmonary arterial hypertension; selexipag; treprostinil.