二叶式主动脉瓣疾病——发病机理和治疗的新感悟（英文）文件材料.ppt

Bicuspid Aortic Valve DiseaseNew Insights in Pathogenesis Treatment;Overview ;Etiology and Mechanisms;Coronary Anomalies in BAV;Presentation of BAV Disease: Correlation With Patient Age;Anomalies Associated With BAV Disease;BAV and Aortic Dissection;Molecular Mechanisms of Aortic Dilatation in Bicuspid Aortic Valve Disease: Role of Matrix Remodeling ; - 50% of young asymptomatic patients - Independent of valve function - Associated degeneration of aortic media - Etiology and mechanisms unknown ;The Role of Fibrillin-1 ;PNAS 1999; 96: 3891 ;Verma S, Circ Res 2001; 88(1): 37;Fibrillin-1 Deficient Mouse: Fibrillin-1 Deficient Tissues Release MMPs ;Matrix Disruption in BAV Patients;Objective ;Methods ;;Increased MMP-2 Activity;Increased MMP-2 Activity;Novel Mechanism of Aortic Dilation;FBN-1 Gene Mutation (?);Anatomic Patterns of Aortic Dilation Custom Tailoring Required;Patterns of Aortic Dilation in Bicuspid Aortic Valves;Patterns of Aortic Dilation in Bicuspid Aortic Valves;;;When Should the Ascending Aorta Be Replaced in Patients with Bicuspid Aortic Valve Disease?;Current recommendations for replacement of the ascending aorta (RAA): 5.5 cm in diameter 5.0 cm in Marfan’s 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 ;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 ;BAV Dysfunction;Valve Prosthesis;Causes of Reoperation;;; BAV disease is associated with a moderate risk of ascending aortic complications after AVR ; ;;;;;;;; ;Targeting MMP in Aneurysm Remodeling and Rupture;Ongoing Areas of Interest