The current treatment strategies for pulmonary arterial hypertension, outlined in the ERS/ESC guidelines published in 2009, have led to improvements in life expectancy and patient quality of life. However, a cure for the disease remains elusive. In order to improve the prognosis for patients with PAH, future research will focus on development of new therapeutic agents, identification of novel targets and studies involving combinations of existing treatments An important goal is to refine the efficacy and tolerability of agents that target NO, endothelin and prostacyclin signaling pathways. Novel targeted therapies, such as tyrosine kinase inhibitors that directly act on the abnormal proliferation of pulmonary vascular cells, are under active investigation. Finally, clinical trials evaluating combinations of treatments have shown encouraging results.