马凡氏综合征的诊断和治疗医学培训课件.ppt

Figure 5a.??Aortic arch graft Sundaram B et al. Radiographics 2007;27:1583-1594 ?2007 by Radiological Society of North America 手术治疗 Figure 5b.??Aortic arch graft Sundaram B et al. Radiographics 2007;27:1583-1594 ?2007 by Radiological Society of North America 手术治疗 Figure 5d.??Aortic arch graft Sundaram B et al. Radiographics 2007;27:1583-1594 ?2007 by Radiological Society of North America 手术治疗 Figure 6a.??Elephant trunk technique Sundaram B et al. Radiographics 2007;27:1583-1594 ?2007 by Radiological Society of North America 手术治疗 Lead productive and satisfying lives ! The best hope for living into one’s 70’s or even 80’s lies ! 感谢您我一起走过激情与梦想； 感谢您我一起面对现实与理想； 感谢您我一起品尝胜利与艰辛； 感谢您我一起憧憬未来与成功； 感谢您我一起欢笑、一起成长！ The incidence of the disorder is approximately 1 in 5000, sporadic mutations making up a quarter of all cases. Noparticular racial or geographical factors are recognised features. 海曼——对于论坛里60、70年代出生的战友应该是很熟悉的名字，当时我们的传媒非常地不发达，但是那时美国女排是我们的死对手，海曼是主攻手，所以当时国内媒体多有报道。她是美国一代女排名将，主攻手，身高1.95m，1986.1.24在日本参加职业联赛的一场比赛常规替换下场休息时，刚刚在场边坐下就静静倒下死亡，医生初步诊断为心脏病发作，开始家属无法接受，仅仅32岁，平常非常健康的主攻手就这样离去，尸体解剖显示夹层动脉瘤破裂，马方综合症的并发症。当时，海曼的死震惊世界！一个顶尖运动员经过无数体检居然没能诊断出这种致命的疾病。当时的医疗水平决定这种当时疾病主要通过家族或遗传史来确诊。患者由于长骨发育过长而具有修长的身材，因此从事体育运动有一定的优势！海曼猝死的原因在于胸主动脉夹层动脉瘤破裂——这是Marfan综合征的严重并发症。 林肯于1 8 6 5年4月1 5日遇刺身亡 The upper body to lower body segment ratio is reduced (＜0.85), this may be exaggerated by spinal deformity 有气胸,也有肺大疱。肺大疱破裂，所以右上是局限性气胸，继续向下肺大疱被纤维组织封闭，顾继续向下可以看到很多肺大疱。 MVP is age-dependent and more common in women with Marfan syndrome. The incidence reaches 60 to 80 percent, and the valve leaflets generally have an elongated and redundant appearance. Progression of severity, as judged by the appearance or worsening of mitral regurgitation by clinical and echocardiographic criteria, occurs in at least 25 percent of patients, a much higher rate than in MVP found in the general population. The mitral annulus dilates and contributes to the regurgitation, as do stretching and occasional rupture of chordae.