Extract
Prostacyclin analogues targeting the prostacyclin pathway are used to treat patients with pulmonary arterial hypertension (PAH), and are given either by an intravenous (IV), subcutaneous (SC) or inhaled route [1]. Continuous IV 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 IV 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 hemodynamic impairment, epoprostenol can be combined with an endothelin receptor antagonist (ERA) and a phosphodiesterase type-5 (PDE-5) inhibitor to achieve sustained clinical and hemodynamic efficacy [4, 5].
Footnotes
This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.
Conflict of interest: Dr Yanaka has nothing to disclose.
Conflict of interest: Dr Guillien has nothing to disclose.
Conflict of interest: Dr Soumagne has nothing to disclose.
Conflict of interest: Dr Benet has nothing to disclose.
Conflict of interest: Dr Piliero has nothing to disclose.
Conflict of interest: Dr Picard has nothing to disclose.
Conflict of interest: Dr Pison reports grants and personal fees from GlaxoSmithKline, personal fees from Novartis Pharma, personal fees from Boehringer Ingelheim, personal fees from Astra Zeneca, outside the submitted work.
Conflict of interest: Dr Sitbon reports personal fees from Arena Pharmaceuticals, personal fees and non-financial support from Actelion Pharmaceuticals, personal fees from Acceleron Pharmaceuticals, grants and personal fees from Bayer HealthCare, non-financial support from GlaxoSmithKline, personal fees from Gossamer Bio, grants and personal fees from Merck, outside the submitted work.
Conflict of interest: Dr Bouvaist reports grants and non-financial support from GlaxoSmithKline, grants and non-financial support from Bayer HealthCare, personal fees and non-financial support from Actelion Pharmaceuticals, outside the submitted work.
Conflict of interest: Dr Degano reports personal fees and non-financial support from Actelion Pharmaceuticals, non-financial support from Bayer HealthCare, grants, personal fees and non-financial support from Novartis Pharma, personal fees from Chiesi, personal fees from GlaxoSmithKline, personal fees from Menarini, outside the submitted work.
- Received December 16, 2019.
- Accepted February 16, 2020.
- Copyright ©ERS 2020
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