Nguyen Huan T, Nguyen Chuyen T H
Department of Geriatrics and Gerontology, University of Medicine and Pharmacy at Ho Chi Minh City, 217 Hong Bang Street, Ward 11, District 5, Ho Chi Minh City 70000, Vietnam.
Department of Cardiology, Thong Nhat Hospital, 1 Ly Thuong Kiet, Ward 7, Tan Binh District, Ho Chi Minh city 70000, Vietnam.
Eur Heart J Case Rep. 2020 Oct 29;4(6):1-7. doi: 10.1093/ehjcr/ytaa325. eCollection 2020 Dec.
Cardiac amyloidosis, a progressive cardiac disease, results from the accumulation of undegraded proteinaceous substrates in the extracellular matrix of the heart. It may present as acute coronary syndrome (ACS); therefore, a clear distinction remains challenging in clinical practice. We describe a case of cardiac amyloidosis mimicking ACS.
A 72-year-old man experienced chest discomfort for 2 days. He gradually developed dyspnoea during the preceding month. Electrocardiogram (ECG) showed sinus rhythm with right bundle branch block and low voltage. Echocardiography revealed concentric left ventricular thickening, biatrial dilation, and preserved ejection fraction with predominantly left ventricular basal hypokinesis. Serial testing of the cardiac biomarkers showed persistently increased high-sensitive cardiac troponin T levels and normal serum creatine kinase myocardial band levels. He was diagnosed with ACS with haemodynamic stability. However, coronary angiography demonstrated non-obstructive coronary arteries. Furthermore, significant macroglossia and periorbital purpura were noticed. Laboratory investigations revealed elevated serum immunoglobulin free light chain (FLC) kappa and lambda levels with an increased FLC ratio. Histological analysis of the biopsied abdominal skin confirmed amyloidosis.
Cardiac amyloidosis often presents as restrictive cardiomyopathy. The usual symptoms include dyspnoea and peripheral oedema. Chest pain may manifest rarely, leading to misdiagnosis as coronary artery disease. Some findings suggestive of cardiac amyloidosis include clinical signs such as amyloid deposits, dyspnoea, low ECG voltage, and basal-predominant hypokinesis with relative apical sparing in echocardiography. Serum FLC test and abdominal skin biopsy can confirm the diagnosis of amyloidosis when a myocardial biopsy is not feasible.
心脏淀粉样变性是一种进行性心脏疾病,由未降解的蛋白质底物在心脏细胞外基质中积累所致。它可能表现为急性冠状动脉综合征(ACS);因此,在临床实践中进行明确区分仍具有挑战性。我们描述一例酷似ACS的心脏淀粉样变性病例。
一名72岁男性,胸部不适2天。在前一个月中逐渐出现呼吸困难。心电图(ECG)显示窦性心律伴右束支传导阻滞及低电压。超声心动图显示左心室向心性增厚、双房扩大,射血分数保留,主要为左心室基底段运动减弱。心脏生物标志物的系列检测显示高敏心肌肌钙蛋白T水平持续升高,血清肌酸激酶同工酶水平正常。他被诊断为血流动力学稳定的ACS。然而,冠状动脉造影显示冠状动脉无阻塞。此外,还注意到明显的巨舌和眶周紫癜。实验室检查显示血清免疫球蛋白游离轻链(FLC)κ和λ水平升高,FLC比值增加。腹部皮肤活检的组织学分析证实为淀粉样变性。
心脏淀粉样变性常表现为限制性心肌病。常见症状包括呼吸困难和外周水肿。胸痛可能很少出现,导致误诊为冠状动脉疾病。一些提示心脏淀粉样变性的发现包括临床体征,如淀粉样沉积物、呼吸困难、ECG低电压,以及超声心动图显示的以基底段为主的运动减弱伴心尖相对保留。当心肌活检不可行时,血清FLC检测和腹部皮肤活检可确诊淀粉样变性。