Pulmonary hypertension (PH) is a progressive disease that is accompanied by a 
poor prognosis. Pulmonary vasoconstriction is facilitated through multiple 
pathways and results in increased pulmonary vascular pressure leading to cell 
proliferation, vascular remodeling, right ventricular hypertrophy/failure, and 
ultimately death. Until recently, just six medications were approved -all for 
one subclass of PH. On October 8, 2013, riociguat (Adempas®) became the first 
medication approved for multiple etiologies of PH. Preclinical studies have 
demonstrated safety and efficacy with significant clinical trials supporting its 
advancement into phase IV trials. Although long-term safety and efficacy and 
place in therapy remain to be established, riociguat presents as an exciting new 
option for the treatment of PH and potentially has additional indications in the 
near future.