Prostacyclin analogues targeting the prostacyclin pathway are used to treat patients with pulmonary arterial hypertension (PAH), and are given either by an intravenous, subcutaneous or inhaled route [1]. Continuous i.v.… Click to show full abstract
Prostacyclin analogues targeting the prostacyclin pathway are used to treat patients with pulmonary arterial hypertension (PAH), and are given either by an intravenous, subcutaneous or inhaled route [1]. Continuous i.v. infusion of epoprostenol has received the strongest recommendation for treatment of the most severe forms of PAH [2], mainly because it is the only PAH-specific drug that was demonstrated to give an improvement in survival in a randomised controlled study [3]. Due to drawbacks associated with its i.v. administration, epoprostenol is preferentially offered to young, high-risk patients [2]. For PAH patients in New York Heart Association (NYHA) functional class (FC) III/IV with severe haemodynamic impairment, epoprostenol can be combined with an endothelin receptor antagonist (ERA) and a phosphodiesterase type-5 (PDE-5) inhibitor to achieve sustained clinical and haemodynamic efficacy [4, 5]. Even for patients who are very stable while receiving i.v. epoprostenol over a long period of time, any transition to selexipag warrants careful long-term clinical and haemodynamic follow-up because worsening is likely to occur http://bit.ly/32wR9eX
               
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