儿童同种异体原位心脏移植受者12例心脏临床病理学观察  

Pediatric cardiac allograft transplantation: a clinicopathological study of twelve recipient hearts

作　　者：李红霞[1] 安然 李静[1] 谢晓丽 金华 李倩茹 周更须 刘爱军[1] Li Hongxia;An Ran;Li Jing;Xie Xiaoli;Jin Hua;Li Qianru;Zhou Gengxu;Liu Aijun(Department of Pathology,the Seventh Medical Center of People′s Liberation Army of China General Hospital,Beijing100700,China;Department of Pediatric Cardiac Surgery,the Seventh Medical Center of People′s Liberation Army of China General Hospital,Beijing100700,China)

机构地区：[1]解放军总医院第七医学中心病理科,北京100700 [2]解放军总医院第七医学中心儿科医学部儿童心脏外科,北京100700

出　　处：《中华病理学杂志》2025年第1期46-51,共6页Chinese Journal of Pathology

摘　　要：目的深入认识终末期心脏组织病理学形态改变及病变程度, 并探讨其临床病理联系。方法收集解放军总医院第七医学中心2022年5月至2023年11月接受儿童同种异体原位心脏移植患者的受者心脏12例, 对受者心脏进行大体检查、常规HE染色、网织纤维和Masson三色等特殊染色后光镜观察, 并对形态变化进行半定量分析。同时, 二代测序DMD基因1例、致病性线粒体DNA变异1例, 检测痰宏基因1例, 并结合临床资料及相关文献进行综合分析。结果儿童心脏移植受者心脏12例, 包括11例扩张型心肌病, 1例暴发性心肌炎。扩张型心肌病患儿年龄3~15岁(中位年龄12岁), 组织病理学形态以心肌细胞肥大、核多形性以及心肌间质的纤维瘢痕形成为主要表现, 心肌排列紊乱, 心肌间可见脂肪组织浸润。1例扩张型心肌病基因检测证实为贝氏肌营养不良, 1例线粒体病线粒体DNA变异检测未发现致病性突变。暴发性心肌炎患儿5岁, 痰宏基因检测结果检出流感嗜血杆菌, 镜下表现为心肌全层弥漫性淋巴细胞及浆细胞浸润, 散在嗜酸性粒细胞及少量中性粒细胞。结论心脏移植是治疗终末期心脏病的有效方法。扩张型心肌病以心肌细胞肥大和核形态改变为主, 同时伴有间质的纤维化和脂肪浸润, 病变程度受多种因素影响。暴发性心肌炎以弥漫性淋巴细胞和浆细胞浸润为主。Objective To analyze the morphologic changes and the extent of severity in end-stage heart disease;and to explore the correlation with their clinical features.Methods Twelve cases of recipients who underwent pediatric cardiac allograft transplantation were collected from May 2022 to November 2023 at the Seventh Medical Center of People′s Liberation Army of China General Hospital.Gross pathologic examinations were performed and morphological changes were observed under a light microscope after HE,Masson′s trichrome,and reticulin staining.Semi-quantitative analysis of morphologic changes was performed.One case received DMD genetic testing,one received mtDNA variation testing for mitochondriopathy,and 1 received metagenomics next-generation sequencing.Clinical data and related literature were reviewed for comprehensive analysis.Results There were 12 recipient hearts including 11 dilated cardiomyopathy(DCM)and 1 fulminant myocarditis(FM).The median age of DCM was 12 years(range,3 to 15 years).DCM showed cardiomyocyte hypertrophy,cardiomyocyte disarray,nuclear morphological changes,interstitial fibrosis and fatty infiltration.One DCM was confirmed as Becker muscular dystrophy by DMD genetic testing.No pathogenic mutations were found in 1 patient that received mtDNA variation testing.H.influenzae was detected in the case of FM.FM showed diffuse and full-thickness inflammatory cell infiltration by large numbers of lymphocytes and plasma cells,scattered eosinophils,and few neutrophils.Conclusions Cardiac transplantation is an excellent treatment for end-stage heart disease.The morphological features of DCM include cardiomyocyte hypertrophy,nuclear morphological changes,interstitial fibrosis and fatty infiltration.The severity of the lesion is influenced by multiple factors.FM predominantly presents diffuse infiltration of lymphocytes and plasma cells.

关 键 词：心脏移植 心肌病 扩张型 心肌炎 

分 类 号：R726.5[医药卫生—儿科]