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Unmasking Early Wild-Type Transthyretin Amyloidosis Cardiomyopathy in a Patient With Refractory Atrial Fibrillation and Unremarkable Cardiac Imaging
Circulation: Heart Failure ( IF 9.7 ) Pub Date : 2018-07-01 , DOI: 10.1161/circheartfailure.117.004812
Danny Varedi 1 , Tibor Kovacsovics 2 , Erinn Downs Kelly 3 , Jo Abraham 1 , Jared Cowley 2 , Kelsey Barrell 1 , Monica P. Revelo 1 , Josef Stehlik 1 , Stavros Drakos 1 , Nassir Marrouche 1 , Brent Wilson 1 , Eric A. Swanson 1 , James Fang 1 , Jose Nativi-Nicolau 1
Affiliation  

A 78-year-old man with atrial flutter/fibrillation unresponsive to 3 cardioversions, 1 flutter, and 2 left atrial ablations, myocardial infarction in 2000 treated with a stent to the left anterior descending coronary, hypertension, bilateral carpal tunnel surgery (15 years before), and lumbar spinal stenosis presented with anemia in March 2015. He also complained of dysphagia and was referred for an esophagogastroduodenoscopy. Esophageal biopsies revealed esophagitis. The submucosal vessels in both the duodenal and gastric biopsies showed the presence of a slight thickening by acellular eosinophilic material that raised the possibility of amyloid on routine hematoxylin and eosin–stained slides (Figure [A] and [B]). This finding prompted investigation with Congo red stain, which demonstrated gastric and duodenal amyloid deposition within the submucosal blood vessels. Figure. Patient with wild-type cardiac amyloidosis. A , Duodenal submucosa with abnormal vessels that appear thickened by waxy, eosinophilic amorphous material suggestive of amyloid. B , Gastric biopsy with abnormal vessels within the muscularis mucosae wherein the vessels appear thickened by waxy, eosinophilic amorphous material suggestive of amyloid. C , Transthoracic echocardiogram in the parasternal view demonstrates septal thickness of 1.1 cm and posterior wall thickness of 0.9 cm. D , Cardiac magnetic resonance with septal thickness of 1.4 cm. E , 12-lead ECG shows atrial fibrillation, mild criteria for left ventricular hypertrophy, and nonspecific …

中文翻译:

揭露难治性心房颤动和心脏成像不明显的早期野生型运甲状腺素蛋白淀粉样变性心肌病。

一名78岁的男性,患有房扑/纤颤,对3次心脏复律,1扑和2例左房消融无反应,2000年用左前降支支架治疗心肌梗塞,高血压,双侧腕管手术(15年)之前),并在2015年3月出现腰椎管狭窄症并伴有贫血。他还抱怨吞咽困难,并接受了食管胃十二指肠镜检查。食管活检发现食管炎。十二指肠和胃活检组织中的粘膜下血管均显示有脱细胞嗜酸性物质轻微增厚,这增加了常规苏木精和曙红染色玻片上淀粉样蛋白的可能性(图[A]和[B])。这一发现促使人们对刚果红染色进行了调查,这表明胃和十二指肠淀粉样蛋白在粘膜下血管内沉积。数字。野生型心脏淀粉样变性病患者。A,十二指肠粘膜下层具有异常的血管,似乎由蜡质,嗜酸性的无定形物质暗示淀粉样蛋白而增厚。B,在胃粘膜内具有异常血管的胃活检,其中所述血管看起来被蜡状,嗜酸性的无定形物质增厚,提示淀粉样蛋白。C,胸骨旁视图的经胸超声心动图显示中隔厚度为1.1厘米,后壁厚度为0.9厘米。D,间隔厚度为1.4 cm的心脏磁共振。E,12导联心电图显示房颤,左心室肥大的轻度标准和非特异性… 十二指肠粘膜下层血管异常,由蜡质,嗜酸性无定形物质暗示淀粉样蛋白而增厚。B,在胃粘膜内具有异常血管的胃活检,其中所述血管看起来被蜡状,嗜酸性的无定形物质增厚,提示淀粉样蛋白。C,胸骨旁视图的经胸超声心动图显示中隔厚度为1.1厘米,后壁厚度为0.9厘米。D,间隔厚度为1.4 cm的心脏磁共振。E,12导联心电图显示房颤,左心室肥大的轻度标准和非特异性… 十二指肠粘膜下层血管异常,由蜡质,嗜酸性无定形物质暗示淀粉样蛋白而增厚。B,在胃粘膜内具有异常血管的胃活检,其中所述血管看起来被蜡状,嗜酸性的无定形物质增厚,提示淀粉样蛋白。C,胸骨旁视图的经胸超声心动图显示中隔厚度为1.1厘米,后壁厚度为0.9厘米。D,间隔厚度为1.4 cm的心脏磁共振。E,12导联心电图显示房颤,左心室肥大的轻度标准和非特异性… 嗜酸性无定形物质,提示淀粉样蛋白。C,胸骨旁视图的经胸超声心动图显示中隔厚度为1.1厘米,后壁厚度为0.9厘米。D,间隔厚度为1.4 cm的心脏磁共振。E,12导联心电图显示房颤,左心室肥大的轻度标准和非特异性… 嗜酸性无定形物质,提示淀粉样蛋白。C,胸骨旁视图的经胸超声心动图显示中隔厚度为1.1厘米,后壁厚度为0.9厘米。D,间隔厚度为1.4 cm的心脏磁共振。E,12导联心电图显示房颤,左心室肥大的轻度标准和非特异性…
更新日期:2018-07-18
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