Abstract
IntroductionAscending aorta aneurysm and dissection are the most common cardiovascular complications affecting Marfan syndrome patients (MFS). Recent large increase in life expectancy of MFS driven the growing prevalence of descending aorta (DAo) dilation and dissection. Despite local abnormal vortices in the proximal Dao were related to local dilation, their origins have never been explored. We investigated the link between aortic geometrical characteristics and abnormal flow pattern in the thoracic aorta of MFS.MethodsFifty-tree MFS without significant aortic valve disease and forty age-matched healthy volunteers (HV) were prospectively included in 4D flow-MRI study, obtaining flow field and angiography. Spatial distribution of flow (in-plane rotational flow (IRF) and systolic flow reversal ratio (SFRR)) and geometric (diameter, ellipticity and curvature) parameters were investigated.ResultsCompared to HV, MFS presented larger aortic diameters only in the proximal AAo (p < 0.001) and DAo (p = 0.028). Increased ellipticity and a more distal location for the peak of aortic curvature were evident, even in the absence of dilation. Through most of the thoracic aorta, IRF was substantially lower in MFS, while SFRR was larger. Interestingly, non-dilated MFS had decreased IRF in the thoracic aorta compared to HV, although SFRR was not increased. Statistically-significant bivariate relations were found between arch IRF and arch ellipticity (R = −0.34) and proximal DAo peak curvature (R = −0.35). Local diameter was negatively correlated with local IRF (R = −0.3) and positively correlated to local SFRR (R = 0.605).ConclusionsMFS presented altered ellipticity and curvature distribution, which are related to abnormal flow patterns even in the absence of dilation.
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