课件：心肌淀粉样变性.pptVIP

Prognosis 预后差，无论何种治疗方法，出现心脏受累后预后更差，发生充血性心力衰竭后中位存活期为6个月。 肌钙蛋白、NT-proBNP是心脏淀粉样变性患者预后判断的敏感标志物。 THANK YOU SUCCESS * * 可编辑 Two-dimensional (2D) echocardiographic image (parasternal long-axis view) from a patient with AL cardiac amyloidosis showing normal biventricular dimensions,granular “sparkling” ventricular wall appearance, concentric left ventricular wall thickening, and thickened mitral valve leaflets suggesting infiltration. Ao ：aorta; IVS interventricular septum; LA left atrium; LV left ventricle;RV right ventricle. * Horizontal long-axis (A) and short-axis (B) at the mid-ventricular level, showing pleural (*) and pericardial effusions (**) and asymmetrical hypertrophy (maximal end-diastolic wall thickness: septum 22 mm, lateral wall 9 mm, wall thickness ratio of 2.6). Corresponding images using the delayed-enhancement magnetic resonance imaging technique: C and D, respectively. The horizontal long-axis image demonstrates global subendocardial hyperenhancement of both ventricles, both atria and the interatrial septum. The short-axis image displays a ring of subendocardial hyperenhancement (arrows). * LOGO 心肌淀粉样变性 （Cardiac Amyloidosis） References J.B. Selvanayagam et al, Evaluation and management of the cardiac amyloidosis, J Am Coll Cardiol 50 (2007), 2101–2110 Rodney H. Falk, MD, Diagnosis and Management of the Cardiac Amyloidoses, Circulation. 2005 ;112:2047-2060 Definition Amyloidosis is a clinical disorder caused by extracellular deposition of insoluble abnormal ?brils, derived from aggregation of misfolded normally soluble protein. ＞1/100,000 person-year Classification AA型系统性淀粉样变性 ---继发于慢性炎症，累及肾为主 AL型系统性淀粉样变 ---原发疾病，最常见，单克隆免疫球蛋白轻链沉积 遗传性系统性淀粉样变 ---转甲状腺素蛋白、载脂蛋白、纤维蛋白原基因突变 老年性系统性淀粉样变 ---60岁以下少见，男性多见，累及心脏为主 透析相关性淀粉样变 ---β2微球蛋白潴留、被修饰，好发于骨关节部位