Abstract #2530

Altered Aortic Geometry in Pediatric Patients with Marfan Syndrome

Roel LF van der Palen^1,2, Julio Garcia², Alex J Barker², Michael J Rose³, Luciana Young⁴, Arno AW Roest¹, Michael Markl^2,5, Joshua D Robinson^4,6, and Cynthia K Rigsby³

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, ⁴Division of Pediatric Cardiology, Ann & Robert Lurie Children’s Hospital of Chicago, Chicago, IL, United States, ⁵Department of Biomedical Engineering, McCormick School of Engineering, Northwestern University, Chicago, IL, United States, ⁶Department 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 originate in the ascending aorta (AAo), one-third in the descending aorta (DAo). Diameter plays an important role in risk stratification. However, aortic dimension alone may not capture the complex changes in aortic geometry that are often encountered in MFS patients, e.g. elongation and changes in overall shape of the aorta. Aortic 3D geometry was systematically investigated in a cohort of children and adolescents with MFS and was compared to an age appropriate control cohort: altered aortic geometry in pediatric MFS patients was detected.

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