title: [Therapy of pulmonary arterial hypertension].
abstract: New drugs for pulmonary arterial hypertension have shown efficacy in randomized controlled trials. Endothelin receptor antagonists (ERA) and prostanoids are most important for clinical practice. Bosentan represents the first approved orally active therapy for PAH. Besides its hepatotoxicity it is mostly well tolerated. The first approved prostanoid, epoprostenol, is currently first choice only for decompensated right heart failure in PAH. It has to be delivered continuously intravenously and is prone to complications, side effects and very high costs. Alternatively, subcutaneous treprostinil can be applied. It is less risky and expensive but may cause local pain at the infusion site. Inhaled iloprost combines the features of a prostanoid with pulmonary and intrapulmonary selectivity. Alternatively, iloprost is being used as continuous intravenous infusion. The phosphodiesterase-5 inhibitor sildenafil was effective in two randomized controlled trials but has not been approved for PAH therapy.
authors.affiliation: Medizinische Klinik, Klinikum der Justus-Liebig-Universität Giessen.
authors.forename: H
authors.initials: H
authors.lastname: Olschewski
authors.forename: A
authors.initials: A
authors.lastname: Ghofrani
authors.forename: B
authors.initials: B
authors.lastname: Enke
authors.forename: F
authors.initials: F
authors.lastname: Reichenberger
authors.forename: R
authors.initials: R
authors.lastname: Voswinckel
authors.forename: A
authors.initials: A
authors.lastname: Kreckel
authors.forename: S
authors.initials: S
authors.lastname: Ghofrani
authors.forename: R
authors.initials: R
authors.lastname: Wiedemann
authors.forename: R
authors.initials: R
authors.lastname: Schulz
authors.forename: F
authors.initials: F
authors.lastname: Grimminger
authors.forename: W
authors.initials: W
authors.lastname: Seeger
mesh_terms: D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D002121:Calcium Channel Blockers / Q000008:administration & dosage / Q000009:adverse effects; D004333:Drug Administration Routes; D004347:Drug Interactions; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D007262:Infusions, Intravenous; D010726:Phosphodiesterase Inhibitors / Q000008:administration & dosage / Q000009:adverse effects; D011453:Prostaglandins / Q000008:administration & dosage / Q000009:adverse effects; D016032:Randomized Controlled Trials as Topic; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins