ARRS 2022 Abstracts

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E1074. I Run in Families: Hereditary Thoracic Aneurysm and Dissection (H-TAAD) - A Pictorial Review
Authors
  1. Anitha Kini; The Ottawa Hospital
  2. Elena Pena; The Ottawa Hospital
  3. Carole Dennie; The Ottawa Hospital
  4. Giselle Revah; The Ottawa Hospital
Background
Heritable thoracic aortic aneurysm and dissection (H-TAAD) is a group of congenital conditions that predispose to diseases of the thoracic aorta.

Educational Goals / Teaching Points
This exhibit aims to discuss clinical and genetic features of H-TAAD; list imaging features of H-TAAD; and summarize importance of genetic testing to guide clinical surveillance and surgical intervention

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
This exhibit includes: bicuspid aortic valve (most common, usually spares aortic root, even in absence of hemodynamically significant valve dysfunction); Marfan syndrome (usually affects aortic root [tulip bulb aortic root]; MVP Loeys-Dietz syndrome (Marfan features but arterial tortuosity and aneurysm rupture at small size); Turner syndrome (bicuspid valve and coarctation); and vascular Ehlers-Danlos (affects medium-size vessels, risk for surgery due to organ fragility).

Conclusion
H-TAAD are abnormalities that lead to aortic wall weakness or abnormal aortic hemodynamic profiles, predisposing patients to aortic dilatation, aneurysm formation, and acute aortic complications.