Pulmonary hypertension is a really interesting disease state with a lot of unique medications, and some not-so-unique medications used in unexpected ways. It is also one of the few disease states where a brief interruption of therapy (epoprostenol) can lead to a life threatening situation. As usual, there is something for everyone here as you may see aspects of this in any practice setting.
Learning objectives
1) Describe the basic pathophysiology of pulmonary arterial hypertension (PAH)
2) Recognize the signs and symptoms associated with PAH
3) Describe the goals of treatment of PAH
4) Compare and contrast the mechanism of action, pharmacokinetics, safety and efficacy of the prostacyclin analogs epoprostenol and treprostinil
5) Describe the role of endothelin receptor antagonists (bosentan, ambrisentan, and macitentan), phophodiesterase-5 inhibitors (sildenafil and tadalafil), and the guanylate cyclase stimulator (riociguat) in the management of PAH
6) Describe why certain PAH medications require enrollment in a risk evaluation and management strategy (REMS) program prior to dispensation