Abstract #2714

Aortic hemodynamics in pediatric Marfan patients compared to healthy pediatric subjects: heterogeneity in the Marfan population

Roel LF van der Palen^1,2, Alex J Barker², Emilie Bollache², Michael J Rose³, Pim van Ooij⁴, Julio Garcia², Luciana Young⁵, Arno AW Roest¹, Michael Markl^2,6, Cynthia K Rigsby³, and Joshua D Robinson^5,7

¹Department of Pediatric Cardiology, Willem-Alexander Children and Youth Center, Leiden University Medical Center, Leiden, Netherlands, ²Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, IL, United States, ³Department of Medical Imaging, Ann & Robert Lurie Children’s Hospital of Chicago, Chicago, IL, United States, ⁴Department of Radiology, Academic Medical Center, Amsterdam, Netherlands, ⁵Division of Pediatric Cardiology, Ann & Robert Lurie Children’s Hospital of Chicago, Chicago, IL, United States, ⁶Department of Biomedical Engineering, McCormick School of Engineering, Northwestern University, Chicago, IL, United States, ⁷Department of Pediatrics, Ann & Robert Lurie Children’s Hospital of Chicago, Chicago, IL, United States

Marfan syndrome (MFS) is a connective tissue disease with high risk of aortic dissection/rupture. Two-thirds of dissections occur in the ascending aorta, one-third in the descending aorta. Diameter plays an important role in risk stratification. However, recent literature has shown diameter only accounts for 50% of the dissections in the descending aortic region. It is not well known how aortic hemodynamics interact with the altered vascular structure of these aortas and how it may impact dilatation. A cohort of MFS children and an age appropriate control group were evaluated with 4D flow MRI: already distinct abnormalities are present in childhood.

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