心室肌致密化不全1例尸检及临床病理特点

Ventricular noncompaction:a clinicopathological study of an autopsy case

目的探讨心室肌致密化不全(ventricular noncompaction,VNC)的临床病理特点以及鉴别诊断。方法采用HE及免疫组化技术对1例心室肌致密化不全的尸检材料进行观察,并结合文献复习。结果左、右心室肌增厚、疏松,心内膜完整、光滑,向心室肌内凹陷,形成小裂隙,未见其他心脏畸形。镜下见心室肌近心内膜处多量不规则扩张的腔隙,内衬扁平上皮,上皮细胞下为薄层疏松纤维组织,与正常心内膜结构相似。累及左、右心室,以左室为更明显。结论VNC是一种少见但很重要的疾病,有特殊的病理学改变,诊断可以依靠超声心动图;病变常有遗传学的背景,早期准确诊断对治疗及家族筛查均十分重要。

Purpose To study the elinieopathologieal features of ventricular noneompaction （VNC）. Method An autopsy material of VNC was analyzed with HE stain and immunohistochemical technique, and the literatures were reviewed. Results Both left and right ventricular myocardiums were thicken with prominent meshwork of trabeeula and deep recesses. Microscopic finding showed the deep recesses were lined by endothelium with subendothelial loose fiber bundles, which were similar to the rest of the ventrieular endoeardium. Conclusions VNC is reportedly rare and associated with high morbidity and mortality ,which has distinct morphologic features. Echocardiography is the major way to make the diagnosis of VNC. A different genetic background has been described. The early diagnosis of VNC would be benefit not only for treatment but for the screen.