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Fig. 2A 15-month-old girl with hypertelorism, bifid uvula,
arachnodactyly, patent ductus arteriosus, and progressive severe aortic root
dilatation. Genetic testing confirmed Loeys-Dietz syndrome. Sagittal oblique
(A) and coronal (B) volume rendering from IV contrast-enhanced
MDCT image show severe enlargement of aortic root (short arrows,
B) and coil that was previously placed in patient's patent ductus
arteriosus (arrowheads). Patient underwent valve-sparing aortic root
replacement and ligation of patent ductus arteriosus. Note tortuosity of right
vertebral artery (long arrows, B).