心肌淀粉样变性患者的临床特征研究

Analysis of clinical characteristics of patients with myocardial amyloidosis

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)、99mTc-焦磷酸(99mTc-PYP)心肌显像等特点。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。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、99mTc-PYP心肌显像及心外组织活检可明确诊断。

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),99mTc-PYP myocardial imaging were collected.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 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.Conclusions The clinical manifestations of CA lack specificity.The combined finding of UCG and ECG were early diagnostic clues.Combination CMR,99mTc-PYP myocardial imaging and extracardiac tissue biopsy can prove the diagnosis of CA.