Chronisch thromboembolische pulmonale Hypertonie (Gruppe 4)

Zusammenfassung

Die chronisch thromboembolische Lungengefäßerkrankung (CTEPD) ist eine wichtige Spätfolge der akuten Lungenembolie, bei der die Thromben in fibröses Gewebe transformiert werden, mit der Gefäßwand verwachsen und zu chronischen Obstruktionen führen. Man unterscheidet die CTEPD ohne pulmonale Hypertonie (PH) mit einem pulmonalarteriellen Mitteldruck bis 20 mmHg von der Form mit PH. Hierbei spricht man weiterhin von chronisch thromboembolischer pulmonaler Hypertonie (CTEPH).

Liegt der Verdacht auf eine CTEPH vor, sollten initial eine Echokardiografie und eine Ventilations-/Perfusions-Szintigrafie erfolgen, um Perfusionsdefekte nachzuweisen. Danach empfiehlt sich die Zuweisung zu einem CTEPH-Zentrum, wo weitere bildgebende Diagnostik und eine Rechtsherzkatheteruntersuchung erfolgen, um die Diagnose zu sichern und das Therapiekonzept festzulegen.

Drei Therapieoptionen stehen aktuell zur Verfügung. Therapie der Wahl ist die pulmonale Endarteriektomie (PEA). Für nichtoperable Patient*innen oder Patient*innen nach PEA und residueller PH, gibt es eine gezielte medikamentöse PH-Therapie sowie das interventionelle Verfahren der pulmonalen Ballonangioplastie (BPA). Zunehmend werden PEA, BPA und die medikamentöse Therapie im Rahmen multimodaler Konzepte kombiniert.

Alle Patient*innen müssen nachgesorgt werden, was vorzugsweise in (CTE)PH-Zentren erfolgen sollte. Für CTEPH-Zentren werden eine Mindestanzahl von PEA-Operationen (50/Jahr) und BPA-Interventionen (100/Jahr) gefordert.

Abstract

Chronic thromboembolic pulmonary disease (CTEPD) is an important late complication of acute pulmonary embolism, in which the thrombi transform into fibrous tissue, become integrated into the vessel wall, and lead to chronic obstructions. CTEPD is differentiated into cases without pulmonary hypertension (PH), characterized by a mean pulmonary arterial pressure up to 20 mmHg and a form with PH. Then, it is still referred to as chronic thromboembolic pulmonary hypertension (CTEPH).

When there is suspicion of CTEPH, initial diagnostic tests should include echocardiography and ventilation/perfusion scan to detect perfusion defects. Subsequently, referral to a CTEPH center is recommended, where further imaging diagnostics and right heart catheterization are performed to determine the appropriate treatment.

Currently, three treatment modalities are available. The treatment of choice is pulmonary endarterectomy (PEA). For non-operable patients or patients with residual PH after PEA, PH-targeted medical therapy, and the interventional procedure of balloon pulmonary angioplasty (BPA) are available. Increasingly, PEA, BPA, and pharmacological therapy are combined in multimodal concepts.

Patients require post-treatment follow-up, preferably at (CTE)PH centers. These centers are required to perform a minimum number of PEA surgeries (50/year) and BPA interventions (100/year).

Schlüsselwörter

chronisch thromboembolische pulmonale Hypertonie - CTEPH - chronisch thromboembolische pulmonale Erkrankung - CTEPD - pulmonale Endarteriektomie - PEA - pulmonale Ballonangioplastie - BPA

Keywords

chronic thromboembolic pulmonary hypertension - CTEPH - chronic thromboembolic disease - CTED - pulmonary endarterectomy - PEA - balloon pulmonary angioplasty - BPA

Publikationsverlauf

Artikel online veröffentlicht:
14. November 2023

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