摘要
目的 观察左心室致密化不全受体心脏超微结构和组织学特点,并探讨二者及临床表现间的关系.方法 2004年6月至2014年6月阜外心血管病医院进行心脏移植的左心室致密化不全患者11例,记录患者的临床资料,心脏移植术后对受体心脏行肉眼检查、测量和拍照记录,光镜及透射电镜观察,对病变的意义进行临床病理分析.结果 11例患者受体心脏大体表现以左心室壁异常粗大的肌小梁和深陷的肌隐窝为特征,小梁层与致密层比值≥2.0;病变以左心室心尖部为重,其次为左心室游离壁.组织学见心内膜中较多胚胎早期的黏液基质样物,以及发育不成熟的心肌细胞.透射电镜显示心肌细胞内线粒体的形态、数目及分布异常;心肌细胞有肌丝、肌节、闰盘结构等发育异常;心肌细胞内基质样物质及核周糖原增加.各组织学类型中均可见心肌内基质增多及闰盘异常;心脏质量≥350 g的受体心脏可出现线粒体增多;LVEF≥30％的患者受体心脏多表现为心肌内基质增多,LVEF＜ 30％者多表现为闰盘异常.结论 左心室致密化不全超微结构特征为肌丝、肌节、线粒体及闰盘结构异常等心肌发育障碍,大体及组织学表现呈小梁层与致密层比值≥2.0的典型特点,且二者与临床心功能障碍有关.
Objective To investigate the association between clinical and histopathological features in patients with left ventricular non-compaction cardiomyopathy （LVNC）.Methods Histopathological examinations were made on 11 LVNC recipient hearts from June 2004 to June 2014 in Fuwai Hospital,myocardial ultrastructure changes were detected using transmission electron microscopy.Association between clinical and pathological features were analyzed.Results Patients were （24 ± 11） years old.There were 6 patients with mucus matrix LVNC,3 patients with fibrous fatty infiltration,and 2 patients with cardiomyocytes proliferation.The gross morphological changes of LVNC hearts were characterized by numerous and prominent trabeculations with deep intratrabecular recesses in left ventricular myocardium.Ratios of the thicker noncompacted endocardial layer （N） and thin epicardial compacted layer （C） （N/C ratio） were ≥2.0,and the most serious lesions were located in the left ventricular apex,and followed by the left ventricular free wall.Histological microscopic examinations evidenced numerous matrix-like material and immature cardiomyocytes on endocardial tissue.Transmission electron microscopy revealed mitochondrial abnormalities on morphology,number,and distribution,underdeveloped cardiomyocytes and anomalies of intercalated disc structure,increased deposition of extracellular matrix-like substance and perinuclear glycogen.Pathological changes on cytoplasmic matrix and intercalated disc were present in all three tissue types of LVNC in this cohort and mitochondria hyperplasia was detected in patients with fibrous fatty infiltration.Heart weight ≥ 350 g is often associated with increased number of mitochondria.Increased cytoplasmic matrix was often detected in patients with LVEF ≥ 30％ while intercalated disc anomalies were often detected in patients with LVEF ＜ 30％.Conclusion Histological changes were closely related clinical features in patients with LVNC.
出处
《中华心血管病杂志》
CAS
CSCD
北大核心
2015年第5期418-422,共5页
Chinese Journal of Cardiology
基金
院所特色项目基金（2012-FWTS-03）
关键词
心肌疾病
组织学
超微结构
Cardiomyopathies
Histology
Microscope,electron,transmission
