Dtsch Med Wochenschr 2014; 139(S 04): S155-S165
DOI: 10.1055/s-0034-1370220
Konsensus | Review article
Pneumologie, Thoraxchirurgie
© Georg Thieme Verlag KG Stuttgart · New York

Chronisch thromboembolische pulmonale Hypertonie (CTEPH) 2014 (Langversion)

Ein PositionspapierChronic thromboembolic pulmonary hypertension – A position paper
H. Wilkens
1   Klinik für Innere Medizin V, Universitätsklinikum des Saarlandes, Homburg
,
I. Lang
2   Klinik für Innere Medizin II, Abt. Kardiologie, Medizinische Universität Wien
,
T. Blankenburg
3   Krankenhaus Martha-Maria Halle-Dölau, Klinik für Innere Medizin II, Halle
,
C. Grohé
4   Klinik für Pneumologie, Evangelische Lungenklinik Berlin Buch
,
S. Guth
5   Abteilung für Thoraxchirurgie, Kerckhoff Klinik, Bad Nauheim
,
M. Held
6   Missionsärztliche Klinik Würzburg, Abteilung Innere Medizin, Würzburg
,
W. Klepetko
8   Klinische Abteilung für Thoraxchirurgie, Medizinische Universität Wien
,
S. Konstantinides
7   Centrum für Thrombose und Hämostase (CTH), Universitätsmedizin Mainz
,
T. Kramm
5   Abteilung für Thoraxchirurgie, Kerckhoff Klinik, Bad Nauheim
,
U. Krüger
9   Klinik für Kardiologie und Angiologie, Herzzentrum Duisburg
,
M. Lankeit
7   Centrum für Thrombose und Hämostase (CTH), Universitätsmedizin Mainz
,
H. J. Schäfers
10   Klinik für Thorax-Herz-Gefäßchirurgie, Universitätsklinikum des Saarlandes, Homburg
,
H. J. Seyfarth
11   Abteilung Pneumologie (Department für Innere Medizin, Neurologie und Dermatologie), Universitätsklinikum Leipzig
,
E. Mayer
5   Abteilung für Thoraxchirurgie, Kerckhoff Klinik, Bad Nauheim
› Institutsangaben
Weitere Informationen

Publikationsverlauf

21. Mai 2014

23. Juni 2014

Publikationsdatum:
01. August 2014 (online)

Zusammenfassung

Dieses Positionspapier fasst die wichtigen Entwicklungen im Bereich der Diagnostik und Therapie der chronisch thromboembolischen pulmonalen Hypertonie (CTEPH) zusammen. Basierend auf den Empfehlungen der Task Force für CTEPH beim 5. Weltsymposium für Pulmonale Hypertonie (PH) in Nizza 2013 bezieht es Stellung zu offenen Fragen, die bei der Behandlung von Patienten mit CTEPH entstehen. Die Arbeitsgruppe wurde im Rahmen des jährlich stattfindenden DACH-Symposiums zur PH in Heidelberg 2013 berufen.

Bei Verdacht auf CTEPH sollten eine Echokardiographie und eine Spiroergometrie erfolgen. Bei positivem Befund ist die bildgebende Methode der Wahl die Ventilations-Perfusionsszintigraphie. Entscheidungen über die Therapie von Patienten mit CTEPH sollten in einem Expertenzentrum auf der Basis einer interdisziplinären Diskussion mit einem erfahrenen PEA-Chirurgen getroffen werden. Die pulmonale Endarteriektomie (PEA) ist Therapie der Wahl für Patienten mit CTEPH. Der Einsatz einer PH-spezifischen medikamentösen Therapie setzt eine umfangreiche Diagnostik entsprechend den Empfehlungen der Leitlinien voraus, sollte in PH-erfahrenen Zentren initiiert werden und sollte nur erfolgen, wenn ausgeschlossen wurde, dass ein Patient durch die potenziell kurative PEA behandelt werden kann.

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

 
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