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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Answer

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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