title: [Chronic thromboembolic pulmonary hypertension--a position paper].
abstract: This position paper summarises current developments in chronic thromboembolic pulmonary hypertension (CTEPH) including diagnostic approaches and treatment options. Based on the guidelines of the task force of CTEPH experts at the 5th World Symposium on Pulmonary Hypertension in Nice 2013. Open questions arising during the treatment of patients with CTEPH are addressed. Patients with suspected CTEPH should undergo echocardiography and cardiopulmonary exercise testing. A ventilation/perfusion scan is the recommended imaging test for screening in the diagnostic algorithm for the evaluation of CTEPH. CTEPH-patients should be discussed in an expert center with an interdisciplinary team and an experienced PEA surgeon to decide the further treatment. Pulmonary endarterectomy (PEA) is the treatment of choice for patients with CTEPH. Medical therapy with PH-targeted medications for inoperable CTEPH and residual disease after PEA should only be initiated if evaluation reveals that the patient is no candidate for a PEA. Current data suggest that CTEPH patients treated with PEA have a better long-term survival rate and quality of life than patients treated with medical therapy.
authors.affiliation: Klinik für Innere Medizin V, Universitätsklinikum des Saarlandes, Homburg.
authors.forename: H
authors.initials: H
authors.lastname: Wilkens
authors.affiliation: Klinik für Innere Medizin II, Abt. Kardiologie, Medizinische Universität Wien.
authors.forename: I
authors.initials: I
authors.lastname: Lang
authors.affiliation: Krankenhaus Martha-Maria Halle-Dölau, Klinik für Innere Medizin II, Halle.
authors.forename: T
authors.initials: T
authors.lastname: Blankenburg
authors.affiliation: Klinik für Pneumologie, Evangelische Lungenklinik Berlin Buch.
authors.forename: C
authors.initials: C
authors.lastname: Grohé
authors.affiliation: Abteilung für Thoraxchirurgie, Kerckhoff Klinik, Bad Nauheim.
authors.forename: S
authors.initials: S
authors.lastname: Guth
authors.affiliation: Missionsärztliche Klinik Würzburg, Abteilung Innere Medizin, Würzburg.
authors.forename: M
authors.initials: M
authors.lastname: Held
authors.affiliation: Klinische Abteilung für Thoraxchirurgie, Medizinische Universität Wien.
authors.forename: W
authors.initials: W
authors.lastname: Klepetko
authors.affiliation: Centrum für Thrombose und Hämostase (CTH), Universitätsmedizin Mainz.
authors.forename: S
authors.initials: S
authors.lastname: Konstantinides
authors.affiliation: Abteilung für Thoraxchirurgie, Kerckhoff Klinik, Bad Nauheim.
authors.forename: T
authors.initials: T
authors.lastname: Kramm
authors.affiliation: Klinik für Kardiologie und Angiologie, Herzzentrum Duisburg.
authors.forename: U
authors.initials: U
authors.lastname: Krüger
authors.affiliation: Centrum für Thrombose und Hämostase (CTH), Universitätsmedizin Mainz.
authors.forename: M
authors.initials: M
authors.lastname: Lankeit
authors.affiliation: Klinik für Thorax-Herz-Gefäßchirurgie, Universitätsklinikum des Saarlandes, Homburg.
authors.forename: H J
authors.initials: HJ
authors.lastname: Schäfers
authors.affiliation: Abteilung Pneumologie (Department für Innere Medizin, Neurologie und Dermatologie), Universitätsklinikum Leipzig.
authors.forename: H J
authors.initials: HJ
authors.lastname: Seyfarth
authors.affiliation: Abteilung für Thoraxchirurgie, Kerckhoff Klinik, Bad Nauheim.
authors.forename: E
authors.initials: E
authors.lastname: Mayer
mesh_terms: D000465:Algorithms; D002908:Chronic Disease; D003131:Combined Modality Therapy; D003299:Cooperative Behavior; D004452:Echocardiography; D004691:Endarterectomy; D005080:Exercise Test; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D033183:Interdisciplinary Communication; D008403:Mass Screening; D058990:Molecular Targeted Therapy; D017410:Practice Guidelines as Topic; D011379:Prognosis; D011651:Pulmonary Artery; D011655:Pulmonary Embolism / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D015996:Survival Rate; D062606:Tertiary Care Centers; D014692:Ventilation-Perfusion Ratio / Q000502:physiology