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Acute Aortic Disease.. - Index of

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Figure 1.C Note from schematic depictions that typical dissection (1) <strong>of</strong>ten impairs<br />

branch vessel flow, but this never happens with IMH and PAU (2). Abbreviations: IMH,<br />

intramural hematoma; PAU, penetrating aortic ulcer.<br />

Figure 6.2 Variable clinical presentation <strong>of</strong> individuals with transforming growth factor<br />

β receptor (TGFBR1 or TGFBR2) mutations. Individuals may present with hypertelorism<br />

and malar flattening (A); bifid uvula (B); marked hypertelorism with exotropia (C);<br />

premature fusion <strong>of</strong> the coronal suture <strong>of</strong> the skull (arrow, D); marked tortuosity <strong>of</strong> the<br />

aorta (arrow head ), aortic root and subclavian artery aneurysms (arrows) or tortuous<br />

abdominal aorta (arrow) (E,F,H); micrognathia, retrognathia, downslanting palpebral<br />

fissures, and skeletal features including pectus excavatum, scoliosis, and pes planus (G,I,J).<br />

Source: From Refs. 21, 51.

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