Die chronisch thromboembolische pulmonale Hypertonie (CTEPH)

Zusammenfassung

Die chronisch thromboembolische pulmonale Hypertonie (CTEPH) bildet die Kategorie 4 der ESC/ERS-Leitlinien zur pulmonalen Hypertonie (PH). Es handelt sich um eine seltene, aber auch unterdiagnostizierte Erkrankung, deren Inzidenz auf 0,1 – 9,1% bei Patienten nach einer akuten Lungenembolie (Mittel 4%) und auf bis zu 10% nach wiederholten Lungenembolien geschätzt wird. Sie ist die einzige Erkrankung aus dem Formenkreis der PH, die mittels einer Operation kurativ behandelt werden kann.

Patienten mit vermuteter CTEPH müssen diagnostisch abgeklärt werden hinsichtlich des Nachweises einer CTEPH und ihrer differenzialdiagnostischen Abgrenzung insbesondere zur idiopathischen pulmonalarteriellen Hypertonie. Zudem müssen chirurgisch therapierbare Patienten erkannt und die pulmonale Hämodynamik (mittlerer pulmonalarterieller Druck und Lungengefäßwiderstand) sicher bestimmt werden. Wenn eine Ventilations-Perfusions-Szintigrafie den Verdacht auf eine CTEPH liefert, sollte die weitere Diagnostik mittels CT-Angiografie und Rechtsherzkatheteruntersuchung erfolgen [1].

Die chronisch thromboembolische pulmonale Hypertonie (CTEPH) ist eine seltene, aber auch unterdiagnostizierte Erkrankung. Bei einem CTEPH-Verdacht muss die Erkrankung nachgewiesen und insbesondere gegen eine idiopathische pulmonalarterielle Hypertonie abgegrenzt werden. Chirurgisch therapierbare Patienten müssen erkannt und die pulmonale Hämodynamik sicher bestimmt werden.

Abstract

The chronic-thromboembolic pulmonary hypertension (CTEPH) resembles the category 4 of the ESC/ERS guidelines of pulmonary hypertension (PH). It is a rare, but also underdiagnosed, disease with an incidence between 0.1% – 9.1% in patients with acute pulmonary embolism (mean 4%) and up to 10% in recurrent pulmonary embolism. It is the only disease from the pulmonary hypertension group that is curable by surgery. Patients with suspected CTEPH have to be evaluated regarding the proof of this specific form of PH, especially to the differential diagnosis of primary pulmonary hypertension. Surgically treatable patients have to be selected correctly and the pulmonary hemodynamics (mean pulmonary artery pressure and resistance) needs to be quantified. The screening tool for patients with suspected CTEPH is the ventilation/perfusion scintigraphy. If there is suspicion of CTEPH further work-up should be done using CT-angiography and right heart catheter.

Schlüsselwörter

chronisch thromboembolische pulmonale Hypertonie - Lungenembolie - Bildgebung

Key words

chronic thromboembolic pulmonary hypertension - pulmonary embolism - imaging

• Literatur

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