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1 1 . 1 .. Marfan syndrome MFS .. 1958 1 . 1 .. 1 : .. 1 2 3 . cystic medial necrosis CMN cyst . 4 . 2 . a . CMN CMN. 8 19 3 5 . MFS CMN 82 5 . CMN MFS . CMN . CMN 6 . b .. 5,7 . 8 . 498 03912 1 1.. 1 .. 2 .. 1 . c .. 7,9 . 9 . shear stress . entry 7 . 2 . 2 . a .. 1 Marfan . FBN1 . A . 2 1 498 03912. B .. 2 Loeys Dietz Loeys Dietz syndrome: LDS . TGFBR1 TGFBR2 LDS MFS . 10 MFS .. 11 MFS A .. 3 Ehlers Danlos vascular type Ehelers Danlos syndrome: vEDS . COL3A1 vEDS EDS 1 . MFS .. 12 MFS LDS .. 4 ACTA2 . ACTA2 13 . ACTA2 . 5 bicuapid aortic valve: BAV.

2 BAV 14 40 9 2 . BAV 15 2 1 . BAV . 16 1 . BAV 17 NOTCH1 . 18 . 6 . 19 . MYH11 SMAD3 . 20 Turner 45XO 21 . 7 . MFS 5 22 . MFS 60 LDS 6 ACTA2 5 . 23 MFS MFS . MFS 10 . b . 1 . 1 .. 1 3 vaso vasorum VV . 498 03912 1 3. 1 3 24 1 .. 25 .. 2 . 1 26,27 Yanagi . 13 27 .. 3 . 24 9 . 28 3 3 .. 4 Be het .. 29 .. 5 .. 30 MFS . 31 .. 32 . 6 . penetrating atherosclerotic ulcer . 33 .. 28 . 3 : . 1 shear stress . 3 . 2 3 1 3 . vasa vasorum VV VV . 4 1 498 03912. 1 3 . 2 3 . shear stress .. 24 . 2 .. 25 . 3 . 3 .. 2 cm . computer simulation 34.

3 4 . intramural hematoma . Hirst autopsy 4 . 1 A . CT 52 . 78 26 CT . 35 CT 22 .. 2011 36 IMH . IMH with ULP . ULP IMH . Hirst 1 .. 1 .. VV .. 5 .. 1 3 . 498 03912 1 5. 37 1 3 . VV . VV .. MFS.. shear stress .. computer simulation .. 1 Hirst AE Jr, et al. Dissecting aneurysm of the aorta a review of 505 cases. Medicine Baltimore . 1958 37 217 79. 2 Schlatmann TJ, et al. Histologic changes in the normal aging aorta implications for dissecting aortic aneurysm. Am J Cardiol. 1977 39 13 20. 3 Wilson SK, et al. Aortic dissecting aneurysms causative factors in 204 subjects.

4 Arch Pathol Lab Med. 1982 106 175 80. 4 Larson EW, et al. Risk factors for aortic dissection a necropsy study of 161 cases. Am J Cardiol. 1984 53 849 55. 5 Nakashima Y, et al. Dissecting aneurysm a clinicopathologic and histopathologic study of 111 autop- sied cases. Hum Pathol. 1990 21 291 6. 6 Carlson RG, et al. Cystic medial necrosis of the ascending aorta in relation to age and hypertension. Am J Cardiol. 1970 25 411 5. 7 . J Jpn Coll Angiol 2005 45 1003 9 . 8 Roberts WC, et al. Aortic medial elastic fiber loss in acute ascending aortic dissection.

5 Am J Cardiol. 2011 108 1639 44. 9 Nakashima Y, et al. Alterations of elastic architecture in human aortic dissecting aneurysm. Lab Invest. 1990 62 751 60. 10 Loeys BL, et al. Aneurysm syndromes caused by mutations in the TGF beta receptor. N Engl J Med. 2006 355 788 98. 11 Kono AK, et al. High prevalence of vertebral artery tortuosity of Loeys Dietz syndrome in comparison with Marfan syndrome. Jpn J Radiol. 2010 28 273 7. 12 Oderich GS, et al. The spectrum, management and clinical outcome of Ehlers Danlos syndrome type . a 30 year experience.

6 J Vasc Surg. 2005 42 98 106. 13 Morisaki H, et al. Mutation of ACTA2 gene as an important cause of familial and non familial non . syndromatic thoracic aortic aneurysm and or dissection. Hum Mutat. 2009 30 1406 11. 14 Cripe L, et al. Bicuspid aortic valve is heritable. J Am Coll Cardiol. 2004 44 138 43. 15 Januzzi JL, et al. Characterizing the young patient with aortic dissection results from the Interna- tional Registry of Aortic Dissection IRAD .. J Am Coll Cardiol. 2004 43 665 9. 6 1 498 03912. 16 Hahn RT, et al. Association of aortic dilation with regurgitant, stenotic and functionally normal bicus- pid aortic valves.

7 J Am Coll Cardiol. 1992 19 283 8. 17 Bonderman D, et al. Mechanisms underlying aortic dilatation in congenital aortic valve malformation. Circulation. 1999 99 2138 43. 18 Garg V, et al. Mutations in NOTCH1 cause aortic valve disease. Nature. 2005 437 270 4. 19 Zhu L, et al. Mutations in myosin heavy chain 11 cause a syndrome associating thoracic aortic aneu- rysm aortic dissection and patent ductus arteriosus. Nat Genet. 2006 38 343 9. 20 van de Laar IM, et al. Phenotypic spectrum of the SMAD3 related aneurysms osteoarthritis syndrome.

8 J Med Genet. 2012 49 47 57. 21 Lin AE, et al. Aortic dilation, dissection, and rupture in patients with Turner syndrome. J Pediatr. 1986 109 820 6. 22 Nienaber CA, et al. International Registry of Acute Aortic Dissection. Gender related differences in acute aortic dissection. Circulation. 2004 109 3014 21. 23 Akutsu K, et al. Genetic analysis of young adult patients with aortic disease not fulfilling the diagnos- tic criteria for Marfan syndrome. Circ J. 2010 74 990 7. 24 Angouras D, et al. Effect of impaired vasa vasorum flow on the structure and mechanics of the thoracic aorta implications for the pathogenesis of aortic dissection.

9 Eur J Cardiothorac Surg. 2000 17 . 468 73. 25 Gao F, et al. Fluid structure interaction within a layered aortic arch model. J Biol Phys. 2006 32 . 435 54. 26 Sampol G, et al. Obstructive sleep apnea and thoracic aorta dissection. Am J Respir Crit Care Med. 2003 168 1528 31. 27 Yanagi H, et al. Acute aortic dissection associated with sleep apnea syndrome. Ann Thorac Cardiovasc Surg. 2013 19 456 60. 28 Tamori Y, et al. Coexistent true aortic aneurysm as a cause of acute aortic dissection. Circ J. 2009 . 73 822 5. 29 Miller DV, et al.

10 Surgical pathology of noninfectious ascending aortitis a study of 45 cases with emphasis on an isolated variant. Am J Surg Pathol. 2006 30 1150 8. 30 Nasiell J, et al. Aortic dissection in pregnancy the incidence of a life threatening disease. Eur J Obstet Gynecol Reprod Biol. 2010 149 120 1. 31 Oskoui R, et al. Aortic dissection in women 40 years of age and the unimportance of pregnancy. Am J Cardiol. 1994 73 821 3. 32 Reilly JM, et al. Hydrocortisone rapidly induces aortic rupture in a genetically susceptible mouse. Arch Surg.