心壁错构性发育不良与原发性心肌病  

Association between dysplastic development of ventricular wall and primary cardiomyopathy： pathomorphological observations on 92 hearts from transplant recipients

作　　者：宋来凤[1] 王红月[1] 赵红[1] 吕凤英[1] 胡盛寿[2] 黄洁[3] 李莉[1] 刘蕾[1] 王琳琳[1] 

机构地区：[1]中国医学科学院北京协和医学院心血管病研究所阜外心血管病医院病理-生理实验中心病理研究室,100037 [2]中国医学科学院北京协和医学院心血管病研究所阜外心血管病医院心外科,100037 [3]中国医学科学院北京协和医学院心血管病研究所阜外心血管病医院心内科,100037

出　　处：《中华心血管病杂志》2008年第10期897-902,共6页Chinese Journal of Cardiology

摘　　要：目的探讨心壁错构性发育不良的病理形态表现、特点、类型及其与原发性心肌病的关系。方法以2004年6月至2007年12月底阜外心血管病医院进行心脏移植的92例心脏为观察对象，在心脏离体后立即进行了肉眼观察、测量和摄影记录，并进行了全面的病理组织学观察。结果心壁的错构性发育见于多种类型的心脏病，且在原发性心肌病中更为多见，差别只是错构的严重程度和分布范围的不同。心壁内存在轻、中度错构不一定就是疾病，但过多的出现能明显影响心脏的结构和功能，只有某种类型的错构性发育较集中或突出时，病理和临床方面才呈现出某一类型的错构性心肌病。错构的范围和程度与原发性心肌病的心脏扩张表现和心力衰竭出现的早晚有一定的正相关趋势。92例中58例为原发性心肌病，34例为非原发性心肌病，原发性心肌病中有25例为错构性心肌病，占原发性心肌病的43．1％。心壁错构性发育异常的病理形态表现主要有：（1）心壁的过度增殖肥厚；（2）纤维／脂肪组织或脂肪／纤维组织替代；（3）心肌细胞的排列紊乱；（4）冠状动脉结构不良等。错构性心肌病一般是一种错构形式为主的多种错构形式组合，相同临床表现的错构性心肌病，其病理基础不完全相同，纤维-脂肪替代为主的多见于扩张型心肌病和致心律失常性右心室心肌病，心肌排列紊乱多伴随于肥厚型心肌病，冠状动脉结构不良常因心肌缺血而扩张。结论心壁错构性发育不良是心脏结构变异的常见形态表现，只有错构的分布比较密集、弥漫时才表现出某一特定类型的心肌病，错构比较密集、弥漫或伴有严重心肌变性者易表现出心脏扩张和心力衰竭。Objective To investigate the morphological characteristics and types of ventricular wall with dysplastie development and their associations to primary cardiomyopathy. Methods Ninety-two hearts from heart transplant patients were studied soon after explanation from 2004 to 2007. Gross examination/ measurement, histopathology and photography were performed. Results Dysplastie development of ventrieular wall could be evidenced in patients with various heart diseases but more often in patients with primary eardiomyopathy, though the extension and distribution of dysplastie development of ventricular wall varied between patients with or without primary cardiomyopathy. Severe dysplastic development of ventrieular wall is associated with clinical dysplastic eardiomyopathy. The range of extension and degree of dysplasia in the ventrieular wall correlated positively to heart dilation/failure and time point of heart failure development. The incidence of severe ventricular wall dysplasia was 27.17% in all transplanted hearts and was 43.1% （25/58） in hearts diagnosed as primary cardiomyopathy （P 〈 0. 05 ）. The main pathological changes of dysplastic hearts were： （1） extensive proliferative hypertrophy of the heart wall, （2） fibrous/fat or fat/fibrous tissue replacement of normal myocardium, （3） disarrangement of myocardial fibers, （4） dysplastie change in the medium-sized intramural arteries. Dysplastie eardiomyopathy was presented mainly as a combination of several forms of dysplasia. The same clinical manifestations of dysplastie eardiomyopathy patients did not always show the same pathologic changes. Fibrous-fat tissue replacement was commonly found in dilated eardiomyopathy and arrhythmogenic fight ventricular cardiomyopathy. Disarrangement of myocardium was often accompanied by hypertrophic eardiomyopathy. Dysplasia of intramural arteries could result in heart dilatation due to myocardial ischemia. Conclusion Dysplasia of ventrieular wall is a common variation of heart structure. Only severe

关 键 词：心肌疾病 心脏移植 心壁结构不良 

分 类 号：R686[医药卫生—骨科学]