心肌淀粉样变性临床特点及影像学特征  

Clinical characteristics and cardiac imaging features of patients with cardiac amyloidosis

作　　者：赵蕾[1] 田庄[1] 方全[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院心内科,100730

出　　处：《中华心血管病杂志》2015年第11期960-964,共5页Chinese Journal of Cardiology

摘　　要：目的总结心肌淀粉样变的临床特点和影像学特别是磁共振成像（MRI）的表现特征。 方法回顾分析自2013年1月至2014年12月北京协和医院住院期间经组织病理活检证实为心肌淀粉样变的31例患者的临床表现、心电图、超声心动图和心脏MRI的特点。 结果心肌淀粉样变患者发病年龄较晚，平均（54±11）岁，且多见于男性（20例，64.5%），血压基本正常。患者入院症状各异，以下肢水肿（13例，41.9%）、胸闷憋气（12例，38.7%）、腹痛腹泻（9例，29.0%）等表现多见。患者心电图以肢体导联低电压（20例，64.5%）、胸导R波递增不良（20例，64.5%）、假性病理性Q波（17例，54.8%）、ST–T改变（27例，87.1%）为主要表现。超声心动图结果示31例患者左心房增大25例（80.6%），室间隔增厚22例（71%），心内膜下出现毛玻璃样改变12例（38.7%），心室限制性舒张功能减低24例（77.4%），左心室收缩功能减低14例（45.2%），左心室射血分数〈50% 10例（32.3%）。31例患者中7例进行了MRI心脏扫描和延迟增强扫描，其中6例有不同程度的左心室室壁和（或）室间隔增厚，3例出现房间隔增厚，4例伴有左心房增大，3例伴有右心房增大，MRI延迟强化示3例患者均出现心内膜弥漫性延迟强化和室间隔肥厚并延迟强化，其中1例伴房间隔延迟强化，2例出现心内膜下延迟强化，2例表现为室间隔和游离壁强化。结论临床上心肌淀粉样变患者主要以胸闷气促、下肢水肿等为临床表现，心电图示肢体导联低电压、胸导R波递增不良和假性病理性Q波，超声心动图示心室壁增厚伴毛玻璃改变，心脏MRI提示延迟强化。在未进行病理活检的情况下如果患者具有上述特征可高度怀疑心肌淀粉样变。ObjectiveTo summarize the clinical characteristics and cardiac imaging features by focusing the diagnostic value of MRI in patients with cardiac amyloidosis （CA） . MethodsA total of 31 cases with pathologically proven CA from January 2013 to December 2014 were included in this retrospective study. ResultsPatients expressed typical disease manifestations at a late age （54±11） years. Majority patients were male （20 cases, 64.5%） in this cohort. Blood pressure was normal, 13 cases （42.9%） presented with edema in lower limbs, 12 cases （38.7%） with chest distress and dyspnea and 9 cases （29.0%） with abdominal pain and diarrhea. Electrocardiogram （ECG） features were as follows： 20 cases （64.5%） with low voltage in limb leads, 20 cases （64.5%） with poor R-wave progression in precordial leads, 17 cases （54.8%） with pseudo-necrotic Q wave and 27 cases （87.1%） with ST-T changes. Echocardiography examination showed that 25 （80.6%） of 31 cases were with left atrial enlargement, 22 cases （71%） with increased ventricular septal thickness and 12 cases （38.7%） with myocardial ground-glass opacity, 24 cases （77.4%） presented restrictive left ventricular filling pattern, 14 cases （45.2%） showed impaired left ventricular systolic function and 10 cases （32.3%） expressed abnormal left ventricular eject function （〈50%）. Cardiac MRI features were as follows： among 31 patients, 7 patients underwent cardiac MRI. Left ventricular and interventricular septum hypertrophy were vsulized in 6 cases, increased thickness of interatrial spetum in 3 cases, left atrial enlargement in 4 cases and right atrial enlargement in 3 cases. MRI also revealed a distinct diffuse delayed gadolinium enhancement of subendocardial and interventricular septum in 3 cases, 1 of which was with delayed enhancement of interatrial spetum. ConclusionsClinically, CA diagnosis should be considered for patients with manifestations of chest distress and edema in lower limbs, ECG features

关 键 词：心肌疾病 淀粉样变性 磁共振成像 活组织检查 

分 类 号：R542.2[医药卫生—心血管疾病]