二叶式主动脉瓣疾病——发病机理和治疗的新感悟（英文）PPT.ppt

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 congenital aortopathy)? When Should RAA be Done in BAV Patients? To determine incidence of long-term ascending aorta complications (aneurysm, dissection or sudden death) and survival in BAV pts with mild aortic dilatation (40 – 50 mm) versus no dilatation To determine the threshold diameter for RAA in BAV patients undergoing aortic valve surgery Purpose of Study Diameter of ascending aorta determined by direct measurement, TEE, or intaoperative description: “normal” → less than 4.0 cm “mildly dilated” → 4.0 – 4.4 cm “moderately dilated” → 4.5 – 4.9 cm Patients with aortic diameters 5.0 cm underwent RAA and were e