Cheng Zhong-wei, Tian Zhuang, Kang Lin, Chen Tai-bo, Fang Li-gang, Cheng Kang-an, Zeng Yong, Fang Quan
Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China.
Zhonghua Xin Xue Guan Bing Za Zhi. 2010 Jul;38(7):606-9.
To summarize the electrocardiography and echocardiography features of patients with cardiac amyloidosis (CA) diagnosed by endo-myocardial biopsy (EMB).
A total of 20 consecutive patients [7 men, mean age (50 ± 12) years] referred for EMB because of clinical suspicion of CA from September 2006 to October 2009 were included in the study. Primary CA was diagnosed in 11 out of 20 patients (55%) by EMB and biomarkers examination. The electrocardiography and echocardiography features were analyzed.
The voltage of all the limb leads were low in the 11 CA patients [mean values of (0.33 - 0.51) mV], the incidence of low voltage and pseudo-infarction patterns were 45% and 45%, respectively. Concentric hypertrophy and normal left ventricular diameters were evidenced in all CA patients on echocardiography, left atrial enlargement (n = 10, 91%), granular/sparking appearance of the myocardium (n = 9, 82%) and moderate to large pericardial effusion (n = 7, 64%) as well as left ventricular systolic dysfunction (n = 8, 73%) were often presented in CA patients.
The diagnosis of primary CA should be considered in patients with unknown origin of heart failure, concentric hypertrophy and normal left ventricular diameters with granular/sparking appearance of the myocardium or pericardial effusion presented on echocardiography and low voltage of limb leads or pseudo-infarction pattern presented on electrocardiography. EMB and serum (urine) biomarkers examinations should be then performed to confirm or exclude the diagnosis of CA.
总结经心内膜心肌活检（EMB）确诊的心脏淀粉样变性（CA）患者的心电图和超声心动图特征。
纳入2006年9月至2009年10月因临床怀疑CA而接受EMB检查的20例连续患者[7例男性，平均年龄（50±12）岁]。20例患者中有11例（55%）通过EMB和生物标志物检查确诊为原发性CA。分析其心电图和超声心动图特征。
11例CA患者所有肢体导联电压均低[平均值为（0.33 - 0.51）mV]，低电压和假性梗死图形的发生率分别为45%和45%。超声心动图显示所有CA患者均有向心性肥厚且左心室直径正常，常出现左心房扩大（n = 10，91%）、心肌颗粒样/闪烁样外观（n = 9，82%）、中至大量心包积液（n = 7，64%）以及左心室收缩功能障碍（n = 8，73%）。
对于不明原因心力衰竭、超声心动图显示向心性肥厚且左心室直径正常伴有心肌颗粒样/闪烁样外观或心包积液、心电图显示肢体导联低电压或假性梗死图形的患者，应考虑原发性CA的诊断。然后应进行EMB和血清（尿液）生物标志物检查以确诊或排除CA诊断。
