二叶式主动脉瓣疾病——发病机理和治疗的新感悟（英文）.pptVIP

* * * * * * * * * * * * In BAV patients, deficient fbn-1 microfibrils detach SMCs in the aortic media from adjacent matrix The SMCs are stimulated by detachment to undergo an altered phenotype and increase constitutive expression and release of MMP-2 Activated MMP-2 degrades adjacent fbn-1 stimulating further SMC detachment and MMP-2 activation Accumulating MMP-2 degrades the elastin and collagen meshwork disrupting the structural support of the elastic lamallae After medial matrix disruption, hemodynamic stress in the aortic root exceeds the capacity of the weakened aortic root resulting in dilatation results FBN-1 Gene Mutation (?) Decreased FBN-1 Production FBN-1 Deficiency SMC Detachment from Elastin Laminae MMP-2 Release Matrix Degradation Apoptosis Medial Degeneration Aortic Dilatation A Novel Mechanism for Aortic Dilatation Secondary Events Anatomic Patterns of Aortic Dilation Custom Tailoring Required Patterns of Aortic Dilation in Bicuspid Aortic Valves A: aortoventricular jxn B: Sinuses of Valsalva C: Sinotubular jxn D: Tubular ascending aorta E: Proximal innominate F: Distal innominate G: Proximal left subclavian H: Distal left subclavian I: Proximal descending J: Descending at diaphragm Patterns of Aortic Dilation in Bicuspid Aortic Valves Root (Sinuses) Dilation (Valved Conduit or Repair) Ascending Aorta (Supracoronary Graft) Ascending+Transverse Arch (RAA and Hemiarch) Aortopathy in Bicuspid Valve When Should the Ascending Aorta Be Replaced in Patients with Bicuspid Aortic Valve Disease? Borger, Fedak, Verma et al. JTCVS 2005 Current recommendations for replacement of the ascending aorta (RAA): 5.5 cm in diameter 5.0 cm in Marfan’s patients Kouchoukos NT, Dougenis D. N Engl J Med 1997;336:1876-88 Optimal diameter for RAA in pts undergoing aortic valve surgery is unknown Hinge Point (6cm) may be different in BAV 30 times higher risk of post-operative dissection post AVR if AA 5.0cm Lower threshold for RAA in BAV patients (because of congeni