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Rofo 2020; 192(01): 50-58
DOI: 10.1055/a-0914-3321
Review
© Georg Thieme Verlag KG Stuttgart · New York

Current and Emerging Imaging Techniques in Patients with Genetic Aortic Syndromes

Artikel in mehreren Sprachen: English | deutsch
Julius Matthias Weinrich
1   Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Alexander Lenz
1   Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Evaldas Girdauskas
2   Department of Cardiovascular Surgery, University Heart Center Hamburg, Hamburg, Germany
,
Gerhard Adam
1   Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Yskert von Kodolitsch
3   Department of Cardiology, University Heart Center Hamburg, Hamburg, Germany
,
Peter Bannas
1   Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
› Institutsangaben
Weitere Informationen

Publikationsverlauf

24. Dezember 2018

24. April 2019

Publikationsdatum:
06. Juni 2019 (online)

Auch verfügbar auf
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Abstract

Background Patients with genetic aortic syndromes such as Marfan or Loeys-Dietz syndrome have a decreased life expectancy due to the risk of aortic dissection and rupture. Imaging plays an important role in the acute setting but also in the initial diagnosis and image-based monitoring. In this article, we provide an overview of the most common genetic aortic syndromes and recommended imaging strategies. Furthermore, we highlight modern imaging methods allowing for the quantification of hemodynamic changes in aortic disease.

Method This is a narrative review article on genetic aortic syndromes and recommended imaging strategies, where we take into account expert opinions and standard-of-care practices from our own center.

Results and Conclusion Radiological imaging plays a key role in the initial diagnosis and surveillance of patients with genetic aortic syndromes. Radiologists contribute significantly to the multi-disciplinary setting of genetic aortic syndromes with knowledge of special features and recommended imaging methods. Accurate measurement of the aorta is crucial, particularly in terms of diameter-based surgical treatment algorithms. Modern imaging methods like 4D-flow MRI and pulse wave velocity have a potential to further improve individualized risk stratification in patients with genetic aortic syndromes.

Key points: 

  • The risk for cardiovascular complications such as acute aortic syndrome is increased in patients with genetic aortic syndromes.

  • Recommended time intervals between image-based monitoring depend on the underlying aortic disease.

  • CT-angiography should be used only in the acute setting.

  • Non-contrast MR-angiography is adequate for screening and image-based monitoring of patients with genetic aortic syndromes.

Citation Format

  • 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
 

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