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Final ID: Poster #: EDU-006

When the Arteries Lose Their Stretch: Imaging of Cardiovascular Manifestations in Williams Syndrome

Purpose or Case Report: Objectives
-Understand the genetic and pathophysiologic basis of the vascular disease in Williams–Beuren syndrome (WBS).
-To show our CT angiography (CTA) protocol tailored for patients with Williams syndrome, optimizing image quality and diagnostic yield while minimizing radiation and contrast exposure.
-To illustrate, through a case-based approach, the spectrum of cardiovascular abnormalities identifiable on CT angiography (CTA), emphasizing the characteristic imaging findings of supravalvar aortic stenosis (SVAS), peripheral pulmonary artery stenosis (PPS), and associated coronary, renal, and systemic arterial stenoses
-Recognize imaging pitfalls and risk features for surgical or anesthetic complication

Teaching points
-CTA provides a single comprehensive evaluation of coronary, pulmonary, systemic, and renal vasculature critical in a disorder where lesions are multifocal.
-SVAS is the dominant lesion; PPS is the most dynamic. CT differentiates between the two and maps to their extent.
-Coronary ostial stenosis is the main cause of sudden cardiac events.
-Renal and systemic arterial lesions explain hypertension and require inclusion of abdominal CTA in baseline studies.
-3D reconstructions are essential for surgical planning and multidisciplinary discussions.
Methods & Materials:
Results:
Conclusions:
Meeting Info:
Session Info:

Posters - Educational

Cardiovascular/Lymphatics

IPR Posters - Educational

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Poster____EDU-006.pdf
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