先天性心脏病Ebstein畸形的组织学及免疫组织化学83例病理分析

Histological and immunohistochemical analysis of 83 cases of congenital heart disease with Ebstein malformation

目的:观察先天性心脏病Ebstein畸形外科修补术后,送检标本的组织病理学及免疫组织化学染色中心肌蛋白表达的变化,以加深对Ebstein畸形的组织病理学认识。方法:收集先天性心脏病Ebstein畸形矫正手术切除送检的右心房、房化心室、三尖瓣及腱索、乳头肌的活检组织块,对各部位标本行石蜡制片,常规HE染色及免疫组织化学染色,显微镜观察心肌组织学改变及心肌蛋白表达情况,进行组间对比分析。结果:83例先天性心脏病Ebstein畸形矫正手术活检标本,显微镜组织学观察及免疫组织化学形态半定量分析发现,在心肌壁厚度变薄、纤维化、炎症反应方面比较,房化心室壁病理改变均较右心房壁明显加重,同时房化心室壁心肌蛋白损失较右心房壁亦明显加重。结论:先天性心脏病Ebstein畸形造成右心房、右心室的心肌壁变薄,心肌灶性纤维化、心内膜弹力纤维、胶原纤维增生增厚,慢性炎症反应等病变比较,以房化心室壁病变更重;免疫组织化学染色对比观察(横纹肌肌动蛋白、肌红蛋白、结蛋白等抗体反应)提示,由于先天性心脏病Ebstein畸形造成了心肌舒缩蛋白的损害,亦以房化心室壁更重。

Objective：To observe the changes of histopathological and immunohistochemical staining in the surgical myocardial tissue specimens of Ebstein anomaly.Methods：The paraffin-embedded tissue sections of right atrium,atrialized ventricle,and three tricuspid chordae,biopsy tissue of papillary muscle,were performed routine hematoxylin and eosin （H＆amp;E） staining.The myocardial contractile and diastolic proteins,such as,striated muscle actin,myoglobin and desmin were tested by immunochemistry,and assessed by semiquantitative analysis.Results：In 83 cases of surgical myocardial tissue specimens of Ebstein anomaly,the pathological changes of myocardial wall thinning,fibrosis and inflammation were obersved in the ventricular wall and atrialized ventricular wall.The abnormal changes were significantly increased in ventricular wall,while the myocardial proteins loss in the atrialized ventricular wall was also significantly more than in the right atrial wall.Conclusion：Myocardial wall thinning,fibrosis and inflammation are the main pathological changes in Ebstein anomaly myocardial tissue.The histological damage in the atrialized ventricular wall is more serious than in the right atrial wall.