Chronisch thromboembolische pulmonale Hypertonie

Zusammenfassung

In der 2009 veröffentlichten Europäischen Leitlinie zur Diagnostik und Therapie der Pulmonalen Hypertonie (PH) wird unter anderem auf Aspekte der Pathophysiologie, Diagnostik und Therapie der chronisch thromboembolischen pulmonalen Hypertonie (CTEPH) eingegangen. Die korrekte Umsetzung der Leitlinie ist für das behandelte Krankheitsbild CTEPH von ganz entscheidender Bedeutung, da es sich um eine Form der PH handelt, die durch eine pulmonale Endarteriektomie (PEA) kurativ behandelbar sein kann, aber immer noch zu spät diagnostiziert und oft falsch therapiert wird. In der Leitlinie wird das Krankheitsbild relativ knapp behandelt, obwohl es eine häufige Form der pulmonalen Hypertonie ist. Jeder Patient mit PH unklarer Ursache sollte auf das Vorliegen einer CTEPH untersucht werden. Als Screening-Methode der Wahl wird eine Ventilations-Perfusionsszintigraphie empfohlen. Sobald eine Ventilations-Perfusionsszintigraphie oder CT-Angiographie Zeichen einer CTEPH zeigt, sollte der Patient in einem Zentrum vorgestellt werden, das Expertise in der medizinischen und chirurgischen Versorgung dieser Patienten hat. Nach Diagnosestellung einer CTEPH muss zur Beurteilung der Operabilität die Vorstellung bei einem erfahrenen PEA-Chirurgen erfolgen. Wesentliche Inhalte der Europäischen Leitlinie wurden kommentiert und bezüglich Diagnostik und Therapie ergänzt.

Abstract

In the 2009 European Guidelines on pulmonary hypertension one section covers aspects of pathophysiology, diagnosis and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The practical implementation of the guidelines for this disease is of crucial importance, because CTEPH is a form of pulmonary hypertension which can be surgically cured. It is, however, frequently diagnosed late in the course of disease and often treated not correctly. In the European Guidelines CTEPH is addressed relatively briefly, although it is a common form of PH which is often overlooked. Any patient with unexplained PH should be evaluated for the presence of CTEPH. A ventilation/perfusion lung scan is recommended as the first step to exclude CTEPH. If the ventilation/perfusion lung scan or multislice CT angiography reveals perfusions defects suggesting the diagnosis of CTEPH, the patient should be referred to a centre with expertise in the medical and surgical management of these patients. After diagnosis of CTEPH the case has to be reviewed by an experienced surgeon in a PEA centre for assessment of operability. The recommendations of the European guidelines are summarized in the current manuscript with additional comments regarding diagnosis and treatment according to most recent evidence.

Schlüsselwörter

chronisch thromboembolische pulmonale Hypertonie - Empfehlungen - Diagnostik - Therapie

Keywords

chronic thromboembolic pulmonary hypertension - guidelines - diagnosis - therapy

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