临床与病理杂志2017，Vol.37Issue(8)：1587-1592,6.DOI:10.3978/j.issn.2095-6959.2017.08.007
致心律失常性心肌病受体心脏病理形态学及超微结构
Morphological and ultrastructural study in heart transplantation recipients with arrhythmogenic cardiomyopathy
摘要
Abstract
Objective: To investigate the histopathological and ultrastructural features of arrhythmogenic cardiomyopathy (ACM). Methods: The clinical and pathological data of 38 ACM patients with heart transplantation were retrospectively studied, the clinical characters, gross observation and histopathological findings were analysed.The correlation between the histological type, myocardial ultrastructure changes and clinical electrocardiogram performance in seven cases were identified using transmission electron microscopy. Results: Classical right-dominant subtype (57.9％) was the most common, followed by biventricular forms (31.6％), then left-dominant subtypes with predominant LV involvement (10.5％). The histological changes of classical right-dominant subtypes were mainly fatty replacement, and that of the left dominant subtypes were mainly fibroadipose replacement (P<0.05). Transmission electron microscope found that sarcomere supercontraction, overstretch or myofibril fracture were ubiquity, and abnormality intercalated discs were observed in 85.7％ (6/7), immature myocardial cell or organelles could were observed in 71.4％. Patients of classical right-dominant subtypes were younger than that of the other two groups (P=0.02). Arrhythmia types were associated with anatomical classification (P=0.01). The incidence of ventricular arrhythmias: biventricular (88.9％) > RV-dominant (42.3％) > RV-dominant (0％). Histologic changes of left ventricular associated with heart weight increased (P<0.01). The left ventrical ejection fraction (LVEF) did not show a relationship with anatomical type or hishistopathological type(P>0.05). Conclusion: Histological and ultrastructural examination on ACS could help the etiological diagnosis, and also provide the morphological basis for further genetic studies.关键词
致心律失常性心肌病/心脏移植/心肌病/临床病理Key words
arrhythmogenic cardiomyopathy/heart transplantation/cardiomyopathy/clinical pathology引用本文复制引用
孙洋,赵红..致心律失常性心肌病受体心脏病理形态学及超微结构[J].临床与病理杂志,2017,37(8):1587-1592,6.基金项目
院所特色项目基金 (2012-FWTS-03),院所青年基金 (2015-F05).This work was supported by Chinese Academy of Medical Sciences characteristics Project Foundation (2012-FWTS-03) and Youth Science Fund (2015-F05), China. （2012-FWTS-03）
