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目的:探讨临床早期识别与诊断心肌淀粉样变性的方法与依据。方法:回顾性分析23例心肌淀粉样变性患者临床表现、心电图及心脏超声、冠状动脉造影等特点,分析研究与病理诊断的一致性。结果:23例(100%)患者均有心功能不全表现,17例(73.9%)表现为限制性充盈障碍(E/A>1.5和DT<140 ms);蛋白尿15例(65.2%);超声心动图检查心肌回声增强、呈颗粒闪光点回声(“闪耀症”)22例(95.7%);心脏M超检查左心室增厚(“质”),23例(100%)均有不同程度室间隔增厚(>12 mm),20例(86.9%)左心室后壁增厚(>12 mm),而心电图肢体导联呈低电压(“电”)21例(91.3%)(“质/电”矛盾现象);住院期间死亡13例(56.5%),随访死亡6例(26.1%),从诊断至死亡时间1～19个月。结论:心力衰竭+心室壁“质/电”矛盾现象+心肌间“闪耀症”诊断心肌淀粉样变性敏感性高,可作为临床早期识别与诊断的方法和依据。
Objective: To explore the early clinical identification and diagnosis of myocardial amyloidosis methods and basis. Methods: The clinical manifestations, electrocardiogram, echocardiography and coronary angiography were retrospectively analyzed in 23 patients with myocardial amyloidosis. The consistency between the study and the pathological diagnosis was analyzed. Results: Twenty-three (100%) patients had cardiac dysfunction, 17 (73.9%) had restrictive filling disorders (E / A> 1.5 and DT <140 ms), 15 (65.2%) had proteinuria, Echocardiography showed enhanced echocardiography in 22 patients (95.7%) with granular flash echoes (“blaze”), 23 patients (100%) with left ventricular hypertrophy There were varying degrees of interventricular septum thickening (> 12 mm), 20 (86.9%) left ventricular posterior wall thickening (> 12 mm), and 21 cases of electrocardiographic limb leads with low voltage (91.3 13 cases (56.5%) died during hospitalization, followed up 6% (26.1%) of deaths, from diagnosis to death time of 1 to 19 months. Conclusion: The diagnosis of myocardial amyloidosis in heart failure + ventricular wall “substance / electricity ” paradox inter-myosin “shine syndrome ” is sensitive and can be used as the method and basis for early clinical diagnosis and identification.