摘要目的 探讨心肌致密化不全的临床病理特征.方法 收集5例心肌致密化不全患者的临床症状、体征、心电图及超声心动图情况,测量心脏的大体情况,包括心脏的重量、纵长、横宽及各瓣膜环的长度、心室流人道和流出道的长度、心室壁的厚度.常规制片,磷钨酸苏木精染色、弹力纤维染色,光镜观察心肌情况及冠状动脉的改变.结果 5例患者均为男性,29-57岁,临床均有胸闷症状,心电图显示心律失常,超声心动图检查3例诊断为心肌致密化不全,病理分析可见受累心腔内显示有异常粗大的肌小梁和交错深陷的隐窝,从心底到心尖致密心肌逐渐变薄;心内膜为增厚的纤维组织,内层非致密心肌肌束粗大紊乱、细胞核畸形,外层致密心肌肌束走行及形态基本正常,细胞核大小均匀.结论 心肌致密化不全具有特有的病理组织学变化,临床无特异性表现,超声心动图是临床诊断心肌致密化不全的主要辅助手段之一.

abstractsObjective To investigate the clinicopathologic manifestations of non-compaction of ventricular myocardium(NVM). Methods Clinical data, electrocardiograms, echocardiography images and pathologic changes were studied in five cases of non-compaction of ventricular myocardium. Results The patient's ages ranged from 29 to 57 years old, all were males. Abnormal electrocardiograms were obtained in all of the 5 cases. Among them, 3 were diagnosed using echocardiography. Histopathologie examination showed that there were abnormally coarse muscle trabeculation and deep recesses, interlacing in arrangement, over the inner wall of the heart chambers. The compacted myocardium became thinning down gradually from the base to the apex of the heart. The non-compacted myocardium bundles locating close to the endocardium were coarse and orderless in arrangement, nuclei were irregular and abnormal,nevertheless, the arrangement and appearance of the muscle bundles near by the pericardium part were essentially normal and the cell nuclei were evenly distributed. Conclusion There are no specific clinical manifestations obtained in patients with non-compaction of ventricular myceardium, however, the pathologic changes are characteristic and a clinical diagnosis can be made by using echocardiography.