摘要
目的探讨限制型心肌病患者心肌淀粉样变(CA)改变的特征及其对临床诊断及鉴别诊断的意义,同时对心力衰竭治疗的影响。方法选择2016年1月至2018年6月住院治疗的限制型心肌病患者67例进行回顾性研究,分别对患者的心电图特征改变、超声心动图、心脏磁共振、心肌病理特点进行分析,及其与心力衰竭的关系。结果限制型心肌病患者的心电图特征为心肌肥厚但肢体导联低电压。超声心动图提示心肌内可见散在分布的闪烁颗粒样回声,左室整体收缩功能减低,室壁运动弥漫性减弱,EF值在40%~49%之间是其诊断的重要依据。心脏磁共振提示弥漫心肌运动异常与延迟强化是诊断的关键依据;病理结果提示偏光下见苹果绿遮光物,考虑淀粉样变。刚果红色染色局部阳性是诊断、鉴别诊断的金标准。结论心肌淀粉样变性与限制型心肌病密切相关,是其重要的病理基础表现,为该病的诊断、鉴别诊断及治疗提供理论依据,此类患者多数为收缩功能轻度减低的心力衰竭,这有利于指导临床医师的治疗以及进一步拓展研究。
Objective To explore the characteristics of cardiac amyloidosis(CA)changes in patients with restrictive cardiomyopathy and its significance for clinical diagnosis and differential diagnosis,as well as its influence on the treatment of heart failure.Methods A retrospective study was conducted on 67 hospitalized patients with restrictive cardiomyopathy from January 2016 to June 2018.ECG changes,echocardiogram,cardiac magnetic resonance imaging and myocardial pathological characteristics were analyzed,and their relationship with heart failure was investigated.Results The electrocardiogram of restrictive cardiomyopathy was characterized by myocardial hypertrophy but limb lead low voltage.Echocardiographic features suggest scattered scintillation particle-like echoes can be seen in myocardium,the overall systolic function of left ventricle is reduced,the motion of ventricular wall is diffuse weakened,and EF value is between 40%and 49%,which is an important basis for its diagnosis.Cardiac magnetic resonance imaging suggests that diffuse myocardial motion abnormalities and delayed enhancement were the key basis for diagnosis.Pathological results showed that apple green shade was seen under polarized light.Local positiveness of Congo red staining was the golden standard for diagnosis and differential diagnosis.Conclusion Amyloidosis of myocardium is closely related to restrictive cardiomyopathy and is an important pathological basic manifestation.It provides theoretical basis for the diagnosis,differential diagnosis and treatment of this disease.Most of these patients are heart failure with slightly reduced systolic function.It is helpful to guide clinicians’treatment and further expand research.
作者
翟莉
吕俊刚
李明
张琳
王娜
王松涛
ZHAI Li;LV Jun-gang;LI Ming;ZHANG Lin;WANG Na;WANG Song-tao(The Third Department of Internal Medicine,Beijing Armed Police Corps Hospital of the Chinese People's Armed Police Force,Beijing 100027,China;President Ojfice,Beijing Armed Police Corps Hospital of the Chinese People's Armed Police Force,Beijing 100027,China)
出处
《中国分子心脏病学杂志》
CAS
2020年第5期3517-3521,共5页
Molecular Cardiology of China
基金
中国医学科学院医学与健康科技创新工程计划协同创新团队项目(2016-I2M-3-006)。
