Imaging-Based 4D Aortic Pressure Mapping in Marfan Syndrome Patients: A Matched Case-Control Study

Leidenberger, T; Gordon, Yaron; Farag, Mina; Delles, Michael; Sanches, Augusto F.; Fink, Mary Alexander; Kallenbach, Klaus; Kauczor, Hans‐Ulrich; Rengier, Fabian

doi:10.1016/j.athoracsur.2019.08.048

Cited by 9 publications

(7 citation statements)

References 22 publications

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“…Surgical intervention is recommended when aortic diameter is greater than 5.5 cm (4.5-5 cm in case of MFS, BAV or other genetical disorders) or if the patient is symptomatic or if the growth rate is greater than 0.5 cm/y (59,73,77,80). But aneurysm rupture or dissection can also occur when aortic diameter is within the normal range with an incidence of 5-10% (81,82). As a matter of fact, the risk of rupture is connected to elastin degradation, increase in glycosaminoglycans (GAGs) and in wall permeability.…”

Section: Aortic Aneurysmmentioning

confidence: 99%

4D flow imaging of the thoracic aorta: is there an added clinical value?

Catapano¹,

Pambianchi²,

Cundari³

et al. 2020

Cardiovasc Diagn Ther

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flow MRI has emerged as a powerful non-invasive technique in cardiovascular imaging, enabling to analyse in vivo complex flow dynamics models by quantifying flow parameters and derived features. Deep knowledge of aortic flow dynamics is fundamental to better understand how abnormal flow patterns may promote or worsen vascular diseases. In the perspective of an increasingly personalized and preventive medicine, growing interest is focused on identifying those quantitative functional features which are early predictive markers of pathological evolution. The thoracic aorta and its spectrum of diseases, as the first area of application and development of 4D flow MRI and supported by an extensive experimental validation, represents the ideal model to introduce this technique into daily clinical practice. The purpose of this review is to describe the impact of 4D flow MRI in the assessment of the thoracic aorta and its most common affecting diseases, providing an overview of the actual clinical applications and describing the potential role of derived advanced hemodynamic measures in tailoring follow-up and treatment.

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Section: Aortic Aneurysmmentioning

confidence: 99%

4D flow imaging of the thoracic aorta: is there an added clinical value?

Catapano¹,

Pambianchi²,

Cundari³

et al. 2020

Cardiovasc Diagn Ther

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“…Over the past decade, several studies using 4D flow MRI have demonstrated the usefulness of this technique for the assessment of abnormal 3D flow patterns and wall shear stress (WSS) in MFS patients [5] , [6] , [7] . Multiple studies have shown altered WSS at predilection sites for aortic dissection in these patients [5] , [6] , [8] , [9] , [10] , [11] , [12] , [13] , [14] . However, it remains a challenge to interpret these findings and to assign elevated or deviant directed WSS to be a cause (by affecting endothelial function) or just a result of aortic degeneration and aneurysm formation in MFS.…”

Section: Introductionmentioning

confidence: 91%

Abnormal aortic hemodynamics are associated with risk factors for aortic complications in patients with marfan syndrome

Andĕl

Ooij

Waard

et al. 2022

IJC Heart & Vasculature

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“…These alterations became more pronounced during the follow-up, but no significant changes were observed in other aortic segments, only in the proximal descending aorta [ 92 ]. Further studies have also been carried out to identify hemodynamic and biomechanical patterns with 4D flow CMR in MFS patients with the aim of detecting clinically relevant predictors for aortic manifestations [ 93 , 94 ].…”

Section: Other Potential Biomarkersmentioning

confidence: 99%

Potential predictors of severe cardiovascular involvement in Marfan syndrome: the emphasized role of genotype–phenotype correlations in improving risk stratification—a literature review

Stengl

Ágg

Pólos

et al. 2021

Orphanet J Rare Dis

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Background Marfan syndrome (MFS) is a genetically determined systemic connective tissue disorder, caused by a mutation in the FBN1 gene. In MFS mainly the cardiovascular, musculoskeletal and ocular systems are affected. The most dangerous manifestation of MFS is aortic dissection, which needs to be prevented by a prophylactic aortic root replacement. Main body The indication criteria for the prophylactic procedure is currently based on aortic diameter, however aortic dissections below the threshold defined in the guidelines have been reported, highlighting the need for a more accurate risk stratification system to predict the occurrence of aortic complications. The aim of this review is to present the current knowledge on the possible predictors of severe cardiovascular manifestations in MFS patients, demonstrating the wide range of molecular and radiological differences between people with MFS and healthy individuals, and more importantly between MFS patients with and without advanced aortic manifestations. These differences originating from the underlying common molecular pathological processes can be assessed by laboratory (e.g. genetic testing) and imaging techniques to serve as biomarkers of severe aortic involvement. In this review we paid special attention to the rapidly expanding field of genotype–phenotype correlations for aortic features as by collecting and presenting the ever growing number of correlations, future perspectives for risk stratification can be outlined. Conclusions Data on promising biomarkers of severe aortic complications of MFS have been accumulating steadily. However, more unifying studies are required to further evaluate the applicability of the discussed predictors with the aim of improving the risk stratification and therefore the life expectancy and quality of life of MFS patients.

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Imaging-Based 4D Aortic Pressure Mapping in Marfan Syndrome Patients: A Matched Case-Control Study

Cited by 9 publications

References 22 publications

4D flow imaging of the thoracic aorta: is there an added clinical value?

4D flow imaging of the thoracic aorta: is there an added clinical value?

Abnormal aortic hemodynamics are associated with risk factors for aortic complications in patients with marfan syndrome

Potential predictors of severe cardiovascular involvement in Marfan syndrome: the emphasized role of genotype–phenotype correlations in improving risk stratification—a literature review

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