title: Riociguat for the treatment of pulmonary hypertension.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) is still an incurable disease with high mortality despite recent treatment advances. Chronic thromboembolic pulmonary hypertension (CTEPH) is a specific form of pulmonary hypertension due to thromboembolic occlusion of pulmonary arteries. Although 50 - 60% of the CTEPH patients can be cured via pulmonary endarterectomy (PEA), a significant portion is inoperable. For both diseases, therefore, new treatments are urgently needed.
  
  
  METHODS
  The review will explain the mechanism of action of the soluble guanylate cyclase stimulator riociguat (BAY 63-2521) and will give an overview regarding the current scientific and clinical data of riociguat in both indications PAH and CTEPH. The most relevant publications up to date were used as sources for this review.
  
  
  CONCLUSIONS
  Riociguat is a novel treatment option in PAH class 1, which, in contrast to phosphodiesterase-5 inhibitors, acts independently of endogenous nitric oxide and has shown efficacy in combination therapy with endothelin-1 receptor antagonists. Riociguat is the first approved drug for non-operable CTEPH and sustained CTEPH after PEA, thus introducing a proven pharmacologic treatment option for this group of patients. Long-term results in the real-life setting are still lacking and are needed to provide evidence for the true amount of progress riociguat adds to the field.
authors.affiliation: Ludwig-Maximilians University of Munich, Comprehensive Pneumology Center, Department of Internal Medicine V , Klinikum Grosshadern Marchioninistrasse 15, 81377 Munich , Germany +49 89 44007 5310 ; +49 89 44007 8608 ; juergen.behr@med.uni-muenchen.de.
authors.forename: Tobias
authors.initials: T
authors.lastname: Meis
authors.forename: Juergen
authors.initials: J
authors.lastname: Behr
mesh_terms: D002908:Chronic Disease; D002986:Clinical Trials as Topic; D020536:Enzyme Activators / Q000627:therapeutic use*; D006162:Guanylate Cyclase / Q000378:metabolism; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D011655:Pulmonary Embolism / Q000150:complications / Q000517:prevention & control; D011720:Pyrazoles / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D018160:Receptors, Cytoplasmic and Nuclear / Q000378:metabolism; D000071756:Soluble Guanylyl Cyclase
chemical_list: D020536:Enzyme Activators; D011720:Pyrazoles; D011743:Pyrimidines; D018160:Receptors, Cytoplasmic and Nuclear; D006162:Guanylate Cyclase; D000071756:Soluble Guanylyl Cyclase; C542595:riociguat
keywords: BAY 63-2521; chronic thromboembolic pulmonary hypertension; clinical study; nitric oxide; pulmonary arterial hypertension; riociguat; soluble guanylate cyclase