孤立性心肌致密化不全ppt课件.ppt

孤立性心肌致密化不全;概述;概述;心肌发生学;心肌发生学;←心室小梁形成模式图。椭球体受到应力而屈曲发生在其最大曲率的区域（a），这导致应力分布的不一致，这“拉”出向心性的脊（b），最终形成小梁（c） ;心肌发生学;;Eduardo Zambrano, et al. 2002;病理;大体标本; 一名21岁男性患者的心脏断面，显示了心室内小梁及小梁间隐窝，左心室纤维弹力增生。（ Oechslin EN, et al. 2000 ）;组织学;;流行病学;遗传背景;;Fukiko Ichida, et al. 2001 ;临床表现;心功能障碍;心律失常;心室血栓形成和体循环栓塞;此外，Chin TK等人还首先报道了患者具有的典型面容：前额宽大、耳际低、颧弓高 然而对几组成年人的研究则很少发现此典型面容。也许说明这种典型面容在儿童患者中较为多见;;;;特殊检查;超声心动;;R Jenni, et al. 2001;R Jenni, et al. 2001;超声心动;超声心动;;CT增强;↑T1加权冠状断面（A）和矢状断面（B），内层（小箭头）示致密不全心肌，外层（大箭头）示正常心肌。 →T2加权（C）显示心尖区高信号，是由纤维增生导致的微循环改变、血栓形成及该区域活动减弱等造成。;PET;治疗;预后;Grant RT: Development of the cardiac coronary vessels in the rabbit. Heart 1926;13:261-271 Grant RT. An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart 1926;13:841-2 Davignon AL, DuSane JW, Kincaid OW, et al. Pulmonary atersia with intact ventricular septum: report of two cases studied by selective angiography and right heart catheterization. Am Heart J 1961;62:690-7 Vernall DG. The human embryonic heart in the seventh week. Am J Anat 1962;111:17-24 Lauer RM, Fink HP, Petry EL, Dunn MI, Diehl AM. Angiographic demonstration of intramyocardial sinusoids in pulmonary-valve atresia with intact ventricular septum and hypoplastic right ventricle. N Engl J Med 1964;271:68-72 Chenard J, Samson M, Beaulieu M: Embryonal sinusoids in the myocardium: Report of a case successfully treated surgically. Can Med Assoc J 1965;92:1356-1357 Dusek J, Bohuslac O, Duskova M: Postnatal persistence of spongy myocardium with embryonic blood supply. Arch Pathol 1975;99:312-317 Chin TK, Perloff JK, Williams RG, Jue K, Mohrmann R. Isolated noncompaction of the ventricular myocardium: A study of eight cases. Circulation 1990;82:507-513 Dewey J. Conces, Thomas Ryan, Robert D. Tarver. Noncompaction of ventricular myocardium: CT appearance. AJR April 1991;156:717-718;Richardson P, McKenna W, Bristow M, et al. Report of the 1995? World Health Organization/International Society and Federation of Cardiology task force