Chronisch thromboembolische pulmonale Hypertonie (CTEPH) 2014 (Langversion)

 

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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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