CAJ | 학술논문

目的对心脏移植受体心脏进行组织学及超微结构观察,探讨原发性限制型心肌病的组织学和亚细胞学特点及其与临床的关系.方法 9例原发性限制型心肌病的受体心脏,离体后立即进行肉眼检查、测量和摄像记录,进行光镜及透射电镜观察;对病变的意义进行临床病理分析;用Masson染色标记心肌间质的纤维化病变,评估心肌纤维化范围和程度.结果原发性限制型心肌病组织形态学表现为:心肌间质大量纤维组织呈网格状包绕心肌细胞增生,心肌细胞变性及极向紊乱;纤维化病变以左心室为重,其次为室间隔;透射电镜显示心肌间质大量纤维增生,心肌细胞肌节结构异常,肌原纤维稀少、缺失、排列紊乱,闰盘数量、分布及形态异常.2例左心室射血分数(LVEF)降低者的心肌细胞变性及极向紊乱的程度重于LVEF值正常组.结论原发性限制型心肌病的组织学表现以心肌间质纤维组织包绕心肌细胞为特征,全心受累,以左心室为重;肌节发育异常为其超微结构特点.组织学及超微结构检查有助于本病的病因学诊断,亦为深入进行基因遗传学研究提供形态学依据.
목적 대심장이식수체심장진행조직학급초미결구관찰,탐토원발성한제형심기병적조직학화아세포학특점급기여림상적관계.방법 9례원발성한제형심기병적수체심장,리체후립즉진행육안검사、측량화섭상기록,진행광경급투사전경관찰;대병변적의의진행림상병리분석;용Masson염색표기심기간질적섬유화병변,평고심기섬유화범위화정도.결과 원발성한제형심기병조직형태학표현위:심기간질대량섬유조직정망격상포요심기세포증생,심기세포변성급겁향문란;섬유화병변이좌심실위중,기차위실간격;투사전경현시심기간질대량섬유증생,심기세포기절결구이상,기원섬유희소、결실、배렬문란,윤반수량、분포급형태이상.2례좌심실사혈분수(LVEF)강저자적심기세포변성급겁향문란적정도중우LVEF치정상조.결론 원발성한제형심기병적조직학표현이심기간질섬유조직포요심기세포위특정,전심수루,이좌심실위중;기절발육이상위기초미결구특점.조직학급초미결구검사유조우본병적병인학진단,역위심입진행기인유전학연구제공형태학의거.
Objective To investigate the histopathological features of primary restrictive cardiomyopathy (PRCM).Methods Nine extransplanted hearts from heart transplantation recipients were examined.Gross and histopathological findings were observed,photographed and final pathological diagnosis was compared to clinical diagnosis.The myocardial ultrastructure changes were determined using transmission electron microscopy.Results The hallmark pathologic feature of PRCM was distinguished by myocardial cell degeneration and hyperplastic collagen fibrils around the myocardial cells.Fibrosis was severer in left ventricle free wall than in ventricular septum and right ventricle.The degree of myocardial cell degeneration and poloidal disorder were severer in patients with reduced ejection fraction (EF) than in patients with preserved EF.Transmission electron microscope evidenced severe interstitial fibrosis,myofibrillar changes of sarcomere structure,abnormalities both on intercalated disc number and distribution.Conclusions PRCM is characterized by hyperplastic collagen fibrils around the cardiomyocytes.Fibrosis is severer in left ventricle than in right ventricle.Sarcomere dysplasia is the main cause of PRCM,and ultrastructural examination is helpful for PRCM diagnosis.