title: Standard PAH therapy improves long term survival in CTEPH patients.
abstract: BACKGROUND
  Chronic thromboembolic pulmonary hypertension (CTEPH), subsequent to pulmonary embolism is a relatively frequent cause of pulmonary hypertension. Similar to patients with pulmonary arterial hypertension (PAH), CTEPH carries a poor prognosis. There is no hard evidence for any other therapy except pulmonary endarterectomy and none for those patients that are not eligible for this procedure.
  
  
  METHODS
  Fifty patients with confirmed, inoperable CTEPH receiving specific vasodilative therapy (prostanoids, endothelin receptor antagonists, PDE 5-inhibitors or combination) were included in this retrospective study (mean age 55 years, range 16-76 years; 36 female, 14 male). Kaplan-Meier plots of these patients were compared with Kaplan-Meier plots of two historical CTEPH patient groups without any specific vasodilative treatment by log rank tests.
  
  
  RESULTS
  CTEPH patients treated with specific vasodilative compounds as used for therapy of PAH were followed up for 52 +/- 30 months and had a significantly improved survival compared with patients treated without PAH type vasodilators (p < or = 0.0002).
  
  
  CONCLUSIONS
  Our data may generate the hypothesis that specific vasodilative treatment improves outcome in patients with inoperable CTEPH.
authors.affiliation: Department of Respiratory Medicine, University of Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany. hans-juergen.seyfarth@uniklinik-leipzig.de
authors.forename: Hans-Juergen
authors.initials: HJ
authors.lastname: Seyfarth
authors.forename: Michael
authors.initials: M
authors.lastname: Halank
authors.forename: Heinrike
authors.initials: H
authors.lastname: Wilkens
authors.forename: Hans-Joachim
authors.initials: HJ
authors.lastname: Schäfers
authors.forename: Ralf
authors.initials: R
authors.lastname: Ewert
authors.forename: Martin
authors.initials: M
authors.lastname: Riedel
authors.forename: Ernst
authors.initials: E
authors.lastname: Schuster
authors.forename: Hans
authors.initials: H
authors.lastname: Pankau
authors.forename: Stefan
authors.initials: S
authors.lastname: Hammerschmidt
authors.forename: Hubert
authors.initials: H
authors.lastname: Wirtz
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D011655:Pulmonary Embolism / Q000150:complications* / Q000401:mortality; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents