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- PAH can be classified as primary (PPH) or secondary pulmonary hypertension (SPH)
- Epoprostenol a prostacyclin analog was the first primary drug for patients w/PAH
- Recent clinical trials describe combination therapy as superior in efficacy to traditional monotherapy
- Varied etiologies of PAH hampers the performance of RCTs for each combination therapy
- PAH is associated w/diminished endothelium factor & nitric oxide, increased phosphodiesterase enzyme leading to the development of the ET-1 receptor antagonist (ERA) bosentan and the PDE- V inhibitor sildenafil
- RCTs are currently investigating the efficacy of three news agents in tx of PAH: imatinib, riociguat, and selexipag
References
A. Yao. Journal of Cardiology 60 (2012) 344–349