Chronic Thromboembolic Pulmonary Hypertension (CTEPH) – Potential Role of Multidetector-Row CT (MD-CT) and MR Imaging in the Diagnosis and Differential Diagnosis of the Disease^[1]

 

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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) can be defined as pulmonary hypertension (resting mean pulmonary arterial pressure of 25 mm Hg or more determined at right heart catheterization) with persistent pulmonary perfusion defects. It is a rare, but underdiagnosed disease with estimated incidences ranging from 0.5 % to 3.8 % of patients after an acute pulmonary embolism (PE), and in up to 10 % of those with a history of recurrent PE. CTEPH is the only form of pulmonary hypertension that can be surgically treated leading to normalization of pulmonary hemodynamics and exercise capacity in the vast majority of patients. The challenges for imaging in patients with suspected CTEPH are fourfold: the imaging modality should have a high diagnostic accuracy with regard to the presence of CTEPH and allow for differential diagnosis. It should enable detection of patients suitable for PEA with great certainty, and allow for quantification of PH by measuring pulmonary hemodynamics (mPAP and PVR), and finally, it can be used for therapy monitoring. This overview tries to elucidate the potential role of ECG-gated multidetector CT pulmonary angiography (MD-CTPA) and MR imaging, and summarizes the most important results that have been achieved so far. Generally speaking, ECG-gated MD-CTPA is superior to MR in the assessment of parenchymal and vascular pathologies of the lung, and allows for the assessment of cardiac structures. The implementation of iodine maps as a surrogate for lung perfusion enables functional assessment of lung perfusion by CT. MR imaging is the reference standard for the assessment of right heart function and lung perfusion, the latter delineating typical wedge-shaped perfusion defects in patients with CTEPH. New developments show that with MR techniques, an estimation of hemodynamic parameters like mean pulmonary arterial pressure and pulmonary vascular resistance will be possible. CT and MR imaging should be considered as complementary investigations providing comprehensive information in patients with CTEPH.

Citation Format:

• Wirth G, Brüggemann K, Bostel T et al. Chronic Thromboembolic Pulmonary Hypertension (CTEPH) – Potential Role of Multidetector-Row CT (MD-CT) and MR Imaging in the Diagnosis and Differential Diagnosis of the Disease. Fortschr Röntgenstr 2014; 186: 751 – 761

Zusammenfassung

Die chronisch-thromboembolische pulmonale Hypertonie (CTEPH) kann als pulmonalarterielle Hypertonie (mittlerer pulmonalarterieller Ruhedruck > 25 mm Hg, bestimmt im Rahmen einer Rechtsherzkatheteruntersuchung) mit persistierenden Perfusionsdefekten nach einer einmaligen oder wiederholten Lungenembolie definiert werden. Es handelt sich um eine seltene, aber eher unterdiagnostizierte Erkrankung, deren Inzidenz zwischen 0,5 und 3,8 % nach einer akuten, und bis zu 10 % nach wiederholten Lungenembolien geschätzt wird. Die CTEPH ist die einzige chirurgisch therapierbare Form einer pulmonalen Hypertonie; durch eine erfolgreiche Operation kommt es zur Normalisierung der pulmonalen Hämodynamik und der körperlichen Belastbarkeit. Die Herausforderungen für die Bildgebung bei Patienten mit vermuteter CTEPH sind: hohe diagnostische Genauigkeit bezüglich dem Nachweis einer CTEPH und ihrer differentialdiagnostischen Abgrenzung, Erkennung chirurgisch therapierbarer Patienten, sichere Bestimmung der pulmonalen Hämodynamik (mittlerer pulmonalarterieller Druck und Lungengefäßwiderstand) und Eignung zur Therapieverlaufskontrolle. Diese Übersicht versucht, die potenzielle Rolle der EKG-getriggerten Mehrschicht-CT und der MRT in der Diagnostik der CTEPH zu beleuchten, und fasst die bis dahin erzielten Resultate zusammen. Allgemein zeichnen sich Vorteile für die MS-CT hinsichtlich der Detektion vaskulärer und parenchymatöser Veränderungen ab; mit EKG-Triggerung ermöglicht sie auch eine Beurteilung der kardialen Strukturen. Die Implementierung der Dual-Energy-Technik ermöglicht ein Jod-Mapping des Lungenparenchyms und damit eine Beurteilung der Lungenperfusion. Die MRT gilt nach wie vor als Referenzverfahren für die Beurteilung von Herzfunktion und Lungenperfusion. Neuere Entwicklungen zeigen, dass das Verfahren auch eine Abschätzung der pulmonalen Hämodynamik ermöglicht. Aktuell können MS-CT und MRT als komplementäre Untersuchungsverfahren angesehen werden, die umfassende Informationen bei Patienten mit vermuteter CTEPH ermöglichen.

¹ dedicated to Prof. Hans Schild on the occasion of his 60^th anniversary and one of my most important teachers of radiology. K-FK

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