PT  - JOURNAL ARTICLE
AU  - Olivier Sitbon
AU  - NicholasW. Morrell
TI  - Pathways in pulmonary arterial hypertension: the future is here
AID  - 10.1183/09059180.00004812
DP  - 2012 Dec 01
TA  - European Respiratory Review
PG  - 321--327
VI  - 21
IP  - 126
4099  - http://err.ersjournals.com/content/21/126/321.short
4100  - http://err.ersjournals.com/content/21/126/321.full
SO  - EUROPEAN RESPIRATORY REVIEW2012 Dec 01; 21
AB  - It is well established that the endothelin, nitric oxide and prostacyclin pathways play an important role in the development of pulmonary arterial hypertension (PAH). Indeed, the therapeutic options currently available for the management of PAH all act on one of these mechanistic pathways. However, this is an exciting time for both clinicians and scientists, as increased understanding of the mechanisms involved in the pathogenesis and progression of PAH has resulted in the development of a number of novel therapeutic options. This article highlights how the introduction of new compounds such as macitentan, riociguat and selexipag, which act on the endothelin, nitric oxide and prostacyclin pathways, respectively, have the potential to further improve the prognosis for patients with PAH.