CAJ | 학술논문

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.
목적 관찰좌심실치밀화불전수체심장초미결구화조직학특점,병탐토이자급림상표현간적관계.방법 2004년6월지2014년6월부외심혈관병의원진행심장이식적좌심실치밀화불전환자11례,기록환자적림상자료,심장이식술후대수체심장행육안검사、측량화박조기록,광경급투사전경관찰,대병변적의의진행림상병리분석.결과 11례환자수체심장대체표현이좌심실벽이상조대적기소량화심함적기은와위특정,소량층여치밀층비치≥2.0;병변이좌심실심첨부위중,기차위좌심실유리벽.조직학견심내막중교다배태조기적점액기질양물,이급발육불성숙적심기세포.투사전경현시심기세포내선립체적형태、수목급분포이상;심기세포유기사、기절、윤반결구등발육이상;심기세포내기질양물질급핵주당원증가.각조직학류형중균가견심기내기질증다급윤반이상;심장질량≥350 g적수체심장가출현선립체증다;LVEF≥30％적환자수체심장다표현위심기내기질증다,LVEF＜ 30％자다표현위윤반이상.결론 좌심실치밀화불전초미결구특정위기사、기절、선립체급윤반결구이상등심기발육장애,대체급조직학표현정소량층여치밀층비치≥2.0적전형특점,차이자여림상심공능장애유관.
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.