Current and Emerging Imaging Techniques in Patients with Genetic Aortic Syndromes

TY - JOUR

T1 - Current and Emerging Imaging Techniques in Patients with Genetic Aortic Syndromes

AU - Weinrich, Julius Matthias

AU - Lenz, Alexander

AU - Girdauskas, Evaldas

AU - Adam, Gerhard

AU - von Kodolitsch, Yskert

AU - Bannas, Peter

N1 - © Georg Thieme Verlag KG Stuttgart · New York.

PY - 2020/1

Y1 - 2020/1

N2 - 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.

AB - 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.

KW - Aortic Diseases/diagnostic imaging

KW - Aortic Valve/abnormalities

KW - Bicuspid Aortic Valve Disease

KW - Computed Tomography Angiography/methods

KW - Diagnostic Imaging/methods

KW - Echocardiography/methods

KW - Ehlers-Danlos Syndrome/diagnostic imaging

KW - Four-Dimensional Computed Tomography/methods

KW - Heart Valve Diseases/diagnostic imaging

KW - Humans

KW - Loeys-Dietz Syndrome/diagnostic imaging

KW - Magnetic Resonance Angiography/methods

KW - Marfan Syndrome/diagnostic imaging

KW - Pulse Wave Analysis/methods

KW - Rare Diseases

KW - Turner Syndrome/diagnostic imaging

U2 - 10.1055/a-0914-3321

DO - 10.1055/a-0914-3321

M3 - SCORING: Review article

C2 - 31170731

VL - 192

SP - 50

EP - 58

JO - ROFO-FORTSCHR RONTG

JF - ROFO-FORTSCHR RONTG

SN - 1438-9029

IS - 1

ER -