Popeye’s sign and transthyretin amyloidosis
Popeye’s sign and transthyretin amyloidosis
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大力水手征和运甲状腺素蛋白淀粉样变性
DOI：
10.1093/eurheartj/ehac330
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发表时间：
2022
影响因子：
39.3
通讯作者：
Fukumoto Yoshihiro
中科院分区：
文献类型：
--
作者：
Tahara Nobuhiro;Honda Akihiro;Ueda Mitsuharu;Fukumoto Yoshihiro
一位76岁男性，因后纵韧带骨化而行颈椎开门椎板成形术，在本院就诊时出现劳力性呼吸困难的症状。12导联心电图显示I度房室传导阻滞(PQ间期262ms)，电轴左偏，完全性右束支传导阻滞(A组)。经胸超声心动图显示左心室肥厚，最大室壁厚度为17 mm，左心室射血分数为61%(面板B1)。应用斑点追踪的应变超声心动图显示，保留心尖后，整体纵向应变显著减小(图B2)。体格检查发现右眼结膜下出血，上肢多处紫癜性病变，屈臂时左上臂有一个大的隆起，即所谓的大力水手征，表明二头肌腱断裂的标志(见补充材料，视频S1；小组C；箭头)。血清和尿液免疫电泳法和游离轻链测定法均未检测到单抗成分。99mTc标记的骨显像显示心肌摄取3级(Panel D)。最终，心内膜心肌活检和转甲状腺素基因的基因测序分析证实患者为野生型转甲状腺素淀粉样变性(图E)。大力水手征并不常见，但对诊断转甲状腺素淀粉样变性很关键。
A 76-year-old man with a history of cervical open-door laminoplasty for ossification of the posterior longitudinal ligament presented to our hospital with a symptom of exertional dyspnoea. A 12-lead electrocardiogram revealed I-degree atrioventricular block (PQ interval 262 ms), left axis deviation, and complete right bundle branch block (Panel A). A transthoracic echocardiogram indicated left ventricular hypertrophy with a maximum wall thickness of 17 mm and a left ventricular ejection fraction of 61%(Panel B1). Strain echocardiogram using speckled tracking showed marked diminution of global longitudinal strain with apical sparing (Panel B2). Physical examination found subconjunctival hemorrhage in right eye, several purpuric lesions in upper extremities, and a large bulge on his left upper arm when he flexed his arm, so-called Popeye’s sign, indicating a hallmark of biceps tendon rupture (see Supplementary material online, Video S1; Panel C; arrow). A monoclonal component was not detected by serum and urine immune-electrophoresis and free light chain assays. 99mTc-labelled bone scintigraphy showed grade 3 myocardial uptake (Panel D). Eventually, endomyocardial biopsy and genetic sequencing analysis of the transthyretin gene confirmed the patient as a wild-type transthyretin amyloidosis (Panel E). Popeye’s sign is uncommon, but pivotal to diagnose transthyretin amyloidosis.