Avula Sindhu Reddy, Handa Rishin, Balakrishnan Bathmapriya, Girard Steven
Department of Internal Medicine, Saint Joseph Mercy Ann Arbor Hospital, Ypsilanti, Michigan, USA.
Department of Cardiovascular Medicine, Saint Joseph Mercy Ann Arbor Hospital, Ypsilanti, Michigan, USA.
BMJ Case Rep. 2017 Aug 7;2017:bcr-2017-220538. doi: 10.1136/bcr-2017-220538.
A previously healthy 65-year-old woman presented with progressive symptoms of heart failure. Low-voltage ECG and findings on echocardiography were concerning for infiltrative cardiomyopathy. Cardiac MRI showed biventricular late gadolinium enhancement, and endomyocardial biopsy confirmed monoclonal immunoglobulin light-chain (AL) amyloidosis. Bortezomib-based chemotherapy was initiated, but the patient continued to clinically deteriorate. She required hospital readmission after resuscitated out-of-hospital cardiac arrest attributed to progressive conduction disease, and a permanent pacemaker was implanted. Chest CT angiography showed a small subsegmental pulmonary embolism (PE), but anticoagulation was withheld as her lower extremity Doppler was negative. One month later, another pulseless electrical arrest occurred, due to massive PE. Thereafter, she had refractory class IV congestive heart failure with severe right ventricular dysfunction, and was deemed unsuitable for stem-cell or heart transplantation. This case highlights the predilection for thromboembolism in AL cardiac amyloidosis.
一名65岁既往健康的女性出现进行性心力衰竭症状。心电图低电压及超声心动图检查结果提示浸润性心肌病。心脏磁共振成像显示双心室晚期钆增强，心内膜活检确诊为单克隆免疫球蛋白轻链（AL）淀粉样变性。开始基于硼替佐米的化疗，但患者临床症状持续恶化。因进行性传导疾病导致院外心脏骤停复苏后，她需要再次入院，并植入了永久性起搏器。胸部CT血管造影显示有一个小的亚段肺栓塞（PE），但由于下肢多普勒检查阴性，未进行抗凝治疗。1个月后，因大面积肺栓塞发生了另一次无脉电活动骤停。此后，她出现难治性IV级充血性心力衰竭伴严重右心室功能障碍，被认为不适合进行干细胞或心脏移植。该病例突出了AL型心脏淀粉样变性中血栓栓塞的倾向。
