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Chronisch thromboembolische pulmonale Hypertonie

Aktueller Stand der Diagnostik und Therapie

Chronic thromboembolic pulmonary hypertension

Current state of diagnostics and treatment

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Zeitschrift für Herz-,Thorax- und Gefäßchirurgie Aims and scope

Zusammenfassung

Die chronisch thromboembolische pulmonale Hypertonie (CTEPH) ist eine Langzeitkomplikation der akuten Lungenembolie, die zu chronischer pulmonaler Hypertonie (PH), Rechtsherzversagen und unbehandelt zum Tod führt. Die Ventilations‑/Perfusionsszintigraphie ist das Screeningverfahren der Wahl, und ein Normalbefund schließt eine CTEPH aus. Ein Missverhältnis mit keilförmigen Perfusionsdefekten bei normaler Ventilation in der Szintigraphie ist jedoch typisch für die CTEPH. Anhand der digitalen Subtraktionsangiographie der Pulmonalarterien zur Darstellung der exakten Lage der Obstruktionen werden die weiteren Therapieentscheidungen getroffen. Die Schwere der PH wird mithilfe der Rechtsherzkatheteruntersuchung beurteilt. Zusätzlich zur dauerhaften Antikoagulation sollte jeder Patient hinsichtlich der Möglichkeit einer pulmonalen Endarteriektomie als Therapie der Wahl mit kurativer Intention evaluiert werden. Für technisch inoperable Patienten wird die gezielte medikamentöse PH-Therapie mithilfe von Riociguat empfohlen; als zusätzliches interventionelles Verfahren sollte die pulmonale Ballonangioplastie erwogen werden. Die Behandlung sollte stets in einem entsprechend erfahrenen Expertenzentrum erfolgen.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a long-term complication of acute pulmonary embolism leading to chronic pulmonary hypertension (PH), right heart failure and death if left untreated. The screening procedure of choice is ventilation/perfusion scintigraphy and normal findings rule out CTEPH; however, a disparity with wedge-shaped perfusion defects in scintigraphy during normal ventilation is typical for CTEPH. Further treatment decisions are made based on digital subtraction angiography of pulmonary arteries to visualize the exact location of the obstructions. The severity of PH is assessed using right heart catheterization. In addition to lifelong anticoagulation, every patient has to be evaluated with respect to pulmonary endarterectomy as the recommended treatment with potentially curative intention. For technically inoperable patients targeted drug treatment of PH with riociguat is recommended and the possibility of balloon pulmonary angioplasty should be considered as an additional interventional procedure. The treatment should always be carried out in expert centers with appropriate experience in CTEPH.

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Abb. 1

Abbreviations

BPA:

pulmonale Ballonangioplastie

CTEPH:

chronisch thromboembolische pulmonale Hypertonie

DSA:

digitale Subtraktionsangiographie

HZV:

Herzzeitvolumen

LE:

Lungenembolie

mPAP:

„mean pulmonary arterial pressure“ (mittlerer pulmonalarterieller Druck)

MRT:

Magnetresonanztomographie

NYHA-FK:

Funktionsklasse der New York Heart Association

PA:

A. pulmonalis

PAOP:

„pulmonary artery occlusion pressure“ (pulmonalarterieller Okklusionsdruck, Wedge-Druck)

PEA:

pulmonale Endarteriektomie

PVR:

„pulmonary vascular resistance“ (pulmonalvaskulärer Widerstand)

TVT:

tiefe Venenthrombose

WSPH:

„World Symposium on Pulmonary Hypertension“ (Weltsymposium zur pulmonalen Hypertonie)

6‑MGS:

6‑Minuten-Gehstrecke

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Authors and Affiliations

  1. Abteilung für Thoraxchirurgie, Kerckhoff Klinik GmbH, Benekestr. 2–8, 61231, Bad Nauheim, Deutschland

    Stefan Guth,  Christoph B. Wiedenroth,  Diethard Prüfer &  Eckhard Mayer

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  1. Stefan Guth
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Correspondence to Stefan Guth.

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Interessenkonflikt

S. Guth hat Vortragshonorare der Firmen Actelion, Bayer, MSD und Pfizer erhalten. C.B. Wiedenroth hat Vortragshonorare der Firmen Actelion, AOP, Bayer, MSD and Pfizer erhalten. D. Prüfer hat Vortragshonorare von der Fa. MSD sowie Reisekostenzuschüsse von den Firmen Ethicon und Covidien erhalten. E. Mayer hat Vortragshonorare der Firmen Actelion, Bayer, MSD and BMS erhalten.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Guth, S., Wiedenroth, C.B., Prüfer, D. et al. Chronisch thromboembolische pulmonale Hypertonie. Z Herz- Thorax- Gefäßchir 34, 79–86 (2020). https://doi.org/10.1007/s00398-019-00356-z

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