What are the types of advanced vasodilatory therapies used in the treatment of pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Therefore, for the majority of PAH patients, treatment requires initiation of one or more of the advanced vasodilatory therapies. At present, three main classes of therapy exist, with each targeting a separate pathophysiologic arm, as follows:

  • Endothelin receptor antagonists (ambrisentan, bosentan, and macitentan): Activation of the endothelin system causes increased levels of the vasoconstrictor endothelin-1 in PAH patients. These agents act to antagonize this pathway.
  • Phosphodiesterase-5 (PDE-5) inhibitors (sildenafil, tadalafil, vardenafil) and guanylate cyclase stimulators (riociguat): Cyclic guanosine monophosphate (cGMP) results in vasodilation through the nitric oxide/cGMP pathway, and PDE-5 is responsible for degrading cGMP. PDE-5 inhibitors thus prevent this degradation
  • Prostacyclin analogues (beraprost, epoprostenol, iloprost, treprostinil) and prostacyclin receptor agonists (selexipag): Prostacyclin is a potent vasodilator, and dysregulation of prostacyclin synthesis metabolism has been identified in PAH patients.

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