Transition From Inhaled Treprostinil to Selexipag in Pulmonary Arterial Hypertension

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To the Editor:
Pulmonary arterial hypertension (PAH) continues to be a life-threatening disease with high morbidity and mortality despite advances in therapy. In the modern era of pulmonary arterial vasodilator therapy, new medications have recently emerged, and protocols are required to facilitate safe and timely transition to alternate agents as desired.
Selexipag is an oral selective prostacyclin receptor agonist, recently approved for treatment of PAH. Selexipag is initiated at a dose of 200 mcg twice daily and then uptitrated weekly to the highest tolerated dose, with a maximum dose of 1600 mcg twice daily.1 Inhaled treprostinil is a prostacyclin vasodilator indicated for treatment of PAH. The initial inhaled treprostinil dose is 3 breaths (18 mcg of treprostinil) 4 times daily, uptitrated weekly by 3 breaths, as tolerated, to a target dose of 9 breaths (54 mcg of treprostinil).2
There is no published data on transitioning patients from inhaled treprostinil to selexipag. Here, we report our experience and protocol in one patient who was successfully converted.
The patient is a 58-year-old man with idiopathic PAH (World Health Organization Group I) and New York Heart Association Functional Classification (NYHA FC) II symptoms, maintained on a pulmonary arterial vasodilator regimen consisting of tadalafil 40 mg daily, ambrisentan 10 mg daily, and inhaled treprostinil 12 breaths 4 times daily.
Because of patient's request, he was transitioned from inhaled treprostinil to selexipag. The inhaled treprostinil was downtitrated with simultaneous uptitration of selexipag as an outpatient over a period of 8 weeks. The patient was instructed to reduce his inhaled treprostinil to 10 breaths 4 times per day and start selexipag at 200 mcg twice daily, on day 1. The selexipag was then uptitrated every week to the maximum dose of 1600 mcg twice daily with simultaneous downtitration of the inhaled treprostinil (Figure 1). The patient tolerated the transition well with no reported complications. On follow up, the patient's functional class remained stable (NYHA FC II) with normal plasma brain natriuretic peptide level and normal right ventricular systolic pressure by echocardiogram. In conclusion, we present the first report of successful transition from inhaled treprostinil to selexipag.

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