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Gefäßmedizin Scan - Zeitschrift für Angiologie, Gefäßchirurgie, diagnostische und interventionelle Radiologie 2019; 06(03): 227-239
DOI: 10.1055/a-0986-9611
CME-Fortbildung
© Georg Thieme Verlag KG Stuttgart · New York

Aktuelle Bildgebungsstrategien bei genetisch bedingten Erkrankungen der Aorta

Julius Matthias Weinrich
,
Alexander Lenz
,
Evaldas Girdauskas
,
Gerhard Adam
,
Yskert von Kodolitsch
,
Peter Bannas
Subject Editor: Wissenschaftlich verantwortlich gemäß Zertifizierungsbestimmungen für diesen Beitrag ist Dr. Julius Matthias Weinrich, Hamburg.
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Publication History

Publication Date:
26 September 2019 (online)

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Genetische Aortenerkrankungen sind vielfältig und haben aufgrund hoher Morbiditäts- und Mortalitätsraten einen besonderen Stellenwert. Die radiologische Bildgebung ist essenziell in der Diagnosestellung, Verlaufskontrolle und Indikationsstellung zum prophylaktischen Aortenwurzelersatz. Im folgenden Artikel werden die häufigsten genetischen Aortenerkrankungen und die empfohlene Bildgebung mit Ausblick auf moderne Techniken vorgestellt.
Kernaussagen

  • Genetische Aortenerkrankungen wie das Marfan- oder das 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 vaskuläre Komplikationen wie ein akutes Aortensyndrom.

  • Die radiologische Bildgebung ist zentraler Bestandteil der initialen Diagnosestellung und Verlaufskontrolle genetischer Aortenerkrankungen und unterscheidet sich in Abhängigkeit von der Grunderkrankung. Durch Kenntnis der Besonderheiten genetischer Aortenerkrankungen und der empfohlenen Bildgebungstechniken trägt der Radiologe wesentlich zur optimalen Patientenbetreuung bei.

  • Sowohl in der Akutsituation als auch im Rahmen der Vorsorge ist die Schnittbildgebung unverzichtbar.

  • Die genaue Vermessung der Aortendiameter beeinflusst maßgeblich den interdisziplinären Beschluss eines prophylaktischen chirurgischen Aortenersatzes.

  • Die CTA sollte nur in der Akutsituation oder in der präoperativen bzw. präinterventionellen Therapieplanung eingesetzt werden.

  • Im Rahmen der Vorsorge empfiehlt sich die native MRA der Aorta.

  • Die empfohlenen Zeitabstände und die Zielorgane für bildgebende Verlaufsuntersuchungen unterscheiden sich in Abhängigkeit von der Aggressivität der genetischen Aortenerkrankung.

  • Moderne Bildgebungstechniken wie die 4-D-Fluss-MRT und die Bestimmung der Pulswellengeschwindigkeit haben das Potenzial, die individualisierte Risikostratifizierung bei Patienten mit genetischen Aortenerkrankungen zu verbessern.

Schlüsselwörter

Genetisch bedingtes Aortensyndrom - Loeys-Dietz-Syndrom - Ehlers-Danlos-Syndrom - Marfan-Syndrom - bildgebende Diagnostik
 

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