title: [Update: Current clinical developments in pulmonary hypertension].
abstract: During the last years, therapeutic options for the treatment of pulmonary arterial hypertension (PAH) have significantly improved. However, the therapeutic concept depends on the etiology of the disease, so that an exact classification is mandatory. Currently, three substance classes are approved for the treatment of PAH (Group I of the Venice Classification): Endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, and prostanoids. After the World Conference in Dana Point (2008), recent changes in therapeutic strategies comprise the early treatment of the disease, as well as the increased importance of an early use of combination therapy if treatment goals are not met. Several new substances are currently evaluated in clinical trials. The soluble guanylate cyclase (sGC) stimulators achieve potent, NO-independent vasodilation. Another promising pathophysiological approach is currently evaluated by the use of tyrosine kinase inhibitors - anti-proliferative drugs which inhibit or even may reverse the pulmonary vascular remodeling process. Serotonin receptor antagonists are also reported to have anti-proliferative, anti-thrombotic and anti-fibrotic effects. Other forms of pulmonary hypertension (Groups II-V) are strictly separated from PAH. Evidence on treatment with PAH specific agents is strongly needed for these groups. Patients with non-PAH pulmonary hypertension should be referred to PAH expert centers, and preferably treated in controlled studies.
authors.affiliation: Klinik III für Innere Medizin, Herzzentrum der Universität zu Köln.
authors.forename: D
authors.initials: D
authors.lastname: Dumitrescu
authors.forename: H A
authors.initials: HA
authors.lastname: Ghofrani
authors.forename: F
authors.initials: F
authors.lastname: Grimminger
authors.forename: M M
authors.initials: MM
authors.lastname: Hoeper
authors.forename: S
authors.initials: S
authors.lastname: Rosenkranz
mesh_terms: D000959:Antihypertensive Agents / Q000145:classification / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D004691:Endarterectomy; D065128:Endothelin Receptor Antagonists; D006162:Guanylate Cyclase / Q000187:drug effects / Q000378:metabolism; D006333:Heart Failure / Q000150:complications; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000188:drug therapy* / Q000209:etiology; D058986:Phosphodiesterase 5 Inhibitors; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use*; D011453:Prostaglandins / Q000627:therapeutic use*; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications; D011655:Pulmonary Embolism / Q000150:complications / Q000601:surgery; D011658:Pulmonary Fibrosis / Q000150:complications; D012702:Serotonin Antagonists / Q000494:pharmacology / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins; D012702:Serotonin Antagonists; D014665:Vasodilator Agents; D011505:Protein-Tyrosine Kinases; D006162:Guanylate Cyclase