Current and Emerging Imaging Techniques in Patients with Genetic Aortic Syndromes

Julius Matthias Weinrich; Alexander Lenz; Evaldas Girdauskas; Gerhard Adam; Yskert von Kodolitsch; Peter Bannas

doi:10.1055/a-0914-3321

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren, Inhaltsverzeichnis

Rofo 2020; 192(01): 50-58
DOI: 10.1055/a-0914-3321

Review

Aktuelle Bildgebungs-Strategien bei genetisch bedingten Erkrankungen der Aorta

Artikel in mehreren Sprachen: English | deutsch

Julius Matthias Weinrich

¹Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

,

Alexander Lenz

¹Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

,

Evaldas Girdauskas

²Department of Cardiovascular Surgery, University Heart Center Hamburg, Hamburg, Germany

,

Gerhard Adam

¹Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

,

Yskert von Kodolitsch

³Department of Cardiology, University Heart Center Hamburg, Hamburg, Germany

,

Peter Bannas

¹Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

› Institutsangaben

Abstract

Zusammenfassung

Hintergrund Genetische Aortenerkrankungen wie das Marfan- oder Loeys-Dietz-Syndrom umfassen ein relativ kleines, jedoch wichtiges kardiovaskuläres Patientenkollektiv. Die betroffenen Patienten unterscheiden sich durch diverse Phänotypen, haben jedoch alle bereits in jungem Alter ein sehr hohes Risiko für ein akutes Aortensyndrom. Sowohl in der Akutsituation als auch im Rahmen der Vorsorge ist die Schnittbildgebung unverzichtbar. In diesem Übersichtsartikel stellen wir die häufigsten genetischen Aortenerkrankungen und die für sie empfohlene Bildgebung vor. Darüber hinaus geben wir einen Ausblick auf moderne Methoden zur Erfassung der Hämodynamik bei Aortenerkrankungen.

Methode Diese Übersichtsarbeit basiert auf der Kombination publizierter Expertenmeinungen sowie den klinischen Standards unseres auf Aortenerkrankungen spezialisierten Zentrums.

Ergebnisse und Schlussfolgerungen Die radiologische Bildgebung ist zentraler Bestandteil der initialen Diagnosestellung und Verlaufskontrolle genetischer Aortenerkrankungen und unterscheidet sich in Abhängigkeit der Grunderkrankung. Durch Kenntnis der Besonderheiten genetischer Aortenerkrankungen und der empfohlenen Bildgebungstechniken trägt der Radiologe wesentlich zur optimalen Patientenbetreuung bei. Die genaue Vermessung der Aortendiameter beeinflusst maßgeblich den interdisziplinären Beschluss eines prophylaktischen chirurgischen Aortenersatzes. Moderne Bildgebungstechniken wie die 4D-Fluss-MRT und die Pulswellengeschwindigkeit haben das Potenzial, die individualisierte Risikostratifizierung bei Patienten mit genetischen Aortenerkrankungen zu verbessern.

Kernaussagen:

Patienten mit genetischen Aortenerkrankungen haben bereits in jungem Alter ein erhöhtes Risiko für vaskuläre Komplikationen wie das akute Aortensyndrom.
Die Schnittbildgebung ist sowohl in der Akutsituation als auch im Rahmen der Vorsorge unverzichtbar.
Die empfohlenen Zeitabstände und die Zielorgane für bildgebende Verlaufsuntersuchungen unterscheiden sich in Abhängigkeit von der Aggressivität der genetischen Aortenerkrankung.
Die CT-Angiografie sollte nur in der Akutsituation oder in der präoperativen bzw. präinterventionellen Therapieplanung eingesetzt werden.
Im Rahmen der Vorsorge empfiehlt sich die native MR-Angiografie der Aorta.

Zitierweise

Weinrich JM, Lenz A, Girdauskas E et al. Current and Emerging Imaging Techniques in Patients with Genetic Aortic Syndromes. Fortschr Röntgenstr 2020; 192: 50 – 58

Key words

genetic aortic syndrome - Loeys-Dietz-syndrome - Ehlers-Danlos-syndrome - Marfan-syndrome

Volltext

Referenzen

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