28例原发性心肌淀粉样变性患者的临床及影像学特征

Clinical and imaging characteristics of 28 patients with cardiac amyloidosis

目的分析原发性心肌淀粉样变性(cardiac amyloidosis,CA)患者的临床及影像学特征。方法回顾性分析复旦大学附属中山医院心内科2008年至2014年间经病理活检确诊为CA的28例患者临床资料,分析患者的临床表现及心电图(electrocardiogram,ECG)、心脏超声(echocardiology,UCG)及心脏磁共振(cardiac magnetic resonance,CMR)特征。结果患者的临床表现多样,累及多个系统。ECG常见肢体导联低电压及胸前导联R波递增不良;UCG显示室间隔不同程度增厚,可见毛玻璃样改变,呈颗粒样强回声;CMR显示左室(或合并右室)室壁增厚,收缩活动减弱伴弥漫性延迟强化。28例患者住院期间有1例因病情进展迅速死亡,死亡原因为心力衰竭。12例接受化疗,应用马法兰联合泼尼松(MP方案)或联合万珂的化疗方案。其余患者放弃治疗自动出院。结论 CA患者临床症状及体征缺乏特异性,对于原因不明的心功能不全患者,心肌活检及心外组织活检结合临床表现、ECG、UCG及CMR可确诊。

Objective To analyze the clinical and imaging characteristics of patients with cardiac amyloidosis （CA）. Methods Clinical data of 28 patients confirmed by biopsy as CA from Zhongshan Hospital, Fudan Univeusity during 2008 and 2014 were retrospectively analyzed. Clinical manifestations, data of electrocardiogram （ECG）, echocardiography （UCG） and cardiac magnetic resonance （CMR） were collected for the evaluation. Results The CA patients presented a variety of clinical manifestations and could affect multiple systems. Low voltage in limb leads and poor R wave progression in precordial leads were frequently observed on ECG. UCG showed varying degrees of ventricular septum thickening, ground glass changes and hyper-echoic granular. CMR revealed thickening of the left ventricular （or combined with right ventricular） wall. Contractile activity weakened and diffuse delayed enhancement. One of the 28 patients died for heart failure during the hospitalization due to rapid progressed heart failure. Twelve patients were treated with chemotherapy, while the other patients dropped treatment. Conclusions Clinical manifestations of CA patients lack of specificity on clinical manifestation. For unexplained heart dysfunction, CA can be diagnosed by biopsy of exocardial tissue in combination with ECG, UCG and CMR.