The Selective Prostacyclin (Ip) Receptor Agonist Selexipag: A Promising Drug in Pediatric Pulmonary Artery Hypertension
Journal Title: Journal of Intensive and Critical Care - Year 2017, Vol 3, Issue 4
Abstract
Pulmonary artery hypertension (PAH) is still an important cause of morbidity and mortality in children. Despite recent developments in PH-specific therapies, survival of patients with idiopathic PAH (IPAH) remains poor and appears to be worse in children compared with adults. During the past few years, treatment of PAH has undergone a real evolution, which has led to the current approval by regulatory agencies of 10 drugs for adult patients from three main pharmacological groups and four different routes of administration (oral, inhaled, subcutaneous and intravenous). Additional drugs such as the selective prostacyclin (IP) receptor agonist selexipag are expected to be approved in the near future. However, emerging therapeutic strategies for adult PH, such as a triple oral combination therapy, have not been sufficiently studied in children. The use of selexipag in children with IPAH, or pulmonary artery hypertension (PAH) associated with congenital heart disease (PAH-CHD) is of interest. Selexipag is the first orally administered prostacyclin receptor agonist with a non-prostanoid structure [1]. Selexipag leads to a vasodilation and a decrease in proliferation. An active metabolite of selexipag, ACT-333679, shows a high selectivity to the IP receptor, and its long half-life enables a twice-a-day oral dosing regimen [2,3]. As of December 2015 selexipag (Uptravi) was FDA approved in the US, making it the first oral prostacyclin agonist to be used in adults with PAH.
Authors and Affiliations
Martin Koestenberger, Hannes Sallmon, Georg Hansmann
Keywords
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- EP ID EP301151
- DOI 10.21767/2471-8505.100101
- Views 50
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