目的:研究心肌淀粉样变性（CA）患者的临床特点，寻找早期诊断线索，优化诊断流程。方法:回顾2016年1月至2019年12月我院经病理确诊的CA患者14例，分析其临床表现、实验室检查、心电图（ECG）、心脏超声（UCG）、心脏核磁共振（CMR）、n 99mTc-焦磷酸（n 99mTc-PYP）心肌显像等特点。n 结果:临床表现为充血性心力衰竭11例，ECG呈肢体导联低电压（或）左胸导联r波递增不良和（或）病理性Q波12例，所有患者UCG均显示不同程度左室壁增厚，8例患者接受CMR检查，7例表现为钆增强扫描延迟现象。行n 99mTc-PYP核素心肌静息显像的8例患者中，3例心肌摄取2~3级，1例2级，均为转甲状腺素蛋白心肌淀粉样变（ATTR）。随访时间1~3.5年，3例死亡，2例自动出院，9例好转。n 结论:CA临床表现缺乏特异性，UCG联合ECG可作为早期筛查手段，结合CMR、n 99mTc-PYP心肌显像及心外组织活检可明确诊断。n “,”Objective:Objectives To study the clinical characteristics of patients with cardiac amyloidosis (CA) , find clues for early diagnosis, and improve the diagnosis process.Methods:Fourteen patients with CA confirmed by pathology were reviewed in our hospital from January 2016 to December 2019. Their clinical manifestations, laboratory examinations, electrocardiogram (ECG) , echocardiogram (UCG) , cardiac magnetic resonance imaging (CMR) , n 99mTc-PYP myocardial imaging were collected.n Results:Eleven cases presented congestive heart failure. Main features of ECG were low voltage in limb leads, poor R wave increments in left chest lead and pathological Q wave, which occurred in 12 cases. UCG showed different degree of left ventricular wall thickening. Eight patients underwent CMR, 7 of them showed delayed gadolinium enhanced scan. Among the 8 patients who underwent n 99mTc PYP radionuclide myocardial rest imaging, 3 cases had 2-3 grades myocardial uptake, and 1 had grade 2 myocardial uptake, all of them were ATTR. UCG showed different degrees of left ventricular wall thickening. During the follow-up period of 1 to 3.5 years, 3 patients died, 2 patients were discharged automatically, and 9 patients improved.n Conclusions:The clinical manifestations of CA lack specificity. The combined finding of UCG and ECG were early diagnostic clues. Combination CMR, n 99mTc-PYP myocardial imaging and extracardiac tissue biopsy can prove the diagnosis of CA.n