title: [Therapy of Pulmonary Arterial Hypertension].
abstract: Pulmonary hypertension (PH) is classified into five distinct groups according to the fifth world conference in Nice 2013. Pulmonary arterial hypertension (PAH) comprises idiopathic PAH, hereditary PAH, drug-induced and associated PAH. Right heart catheterization is essential for the diagnosis of PH and should precede initiation of a targeted PAH therapy. Besides general measures and supportive therapy, four different classes of targeted drugs have been approved for the treatment of PAH. Combination therapy, either sequential or initial (up-front), is increasingly gaining recognition. Risk stratification and treatment goals have been defined to guide therapeutic decisions. However, cure is still far from reach and lung transplantation is an important treatment option for patients with end-stage disease under optimal supportive and targeted drug therapy.
authors.affiliation: Zentrum für pulmonal arterielle Hypertonie, Martin Zeitz Zentrum für seltene Erkrankungen, Universitätsklinikum Hamburg-Eppendorf, Hamburg.
authors.forename: H
authors.initials: H
authors.lastname: Klose
authors.affiliation: Zentrum für pulmonal arterielle Hypertonie, Martin Zeitz Zentrum für seltene Erkrankungen, Universitätsklinikum Hamburg-Eppendorf, Hamburg.
authors.forename: L
authors.initials: L
authors.lastname: Harbaum
authors.affiliation: Zentrum für pulmonal arterielle Hypertonie, Martin Zeitz Zentrum für seltene Erkrankungen, Universitätsklinikum Hamburg-Eppendorf, Hamburg.
authors.forename: T
authors.initials: T
authors.lastname: Oqueka
authors.affiliation: Zentrum für pulmonal arterielle Hypertonie, Martin Zeitz Zentrum für seltene Erkrankungen, Universitätsklinikum Hamburg-Eppendorf, Hamburg.
authors.forename: M
authors.initials: M
authors.lastname: Simon
authors.affiliation: Zentrum für pulmonale Hypertonie, Thoraxklinik am Universitätsklinikum Heidelberg.
authors.forename: E
authors.initials: E
authors.lastname: Grünig
mesh_terms: D000959:Antihypertensive Agents / Q000008:administration & dosage*; D004305:Dose-Response Relationship, Drug; D019317:Evidence-Based Medicine; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy*; D017410:Practice Guidelines as Topic; D015272:Pulmonary Medicine / Q000592:standards*; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents