Xu Zhanwen, Li Yaqin
Department of Cardiology, Affiliated Hospital of Hebei University, Baoding, China.
Cardiol Young. 2022 Dec;32(12):2029-2031. doi: 10.1017/S1047951122000993. Epub 2022 Apr 25.
Cardiac amyloidosis presented with normal interventricular septum is an extremely rare entity, and diagnosis may be difficult. This report discusses a 44-year-old female who presented with worsening dyspnoea on exertion, orthopnoea, and lower-extremity oedema. Electrocardiogram depicted low voltage in limb leads and a pseudoinfarct pattern. Echocardiogram revealed biatrial dilatation without changes of ventricular chambers and restrictive filling physiology. A diagnosis of cardiac amyloidosis was considered. Cardiac MRI was pursued, showing delayed gadolinium enhancement, and this ultimately led to the myocardial biopsy confirming the diagnosis of cardiac amyloidosis. The case suggests that patients who present with heart failure of uncertain aetiology, amyloidosis could be a cause of cardiomyopathy despite the absence of "classical" echocardiographic features of amyloid deposition such as an increased interventricular septum thickness or"brilliant sparkled"appearance of the myocardium.
室间隔正常的心脏淀粉样变性是一种极其罕见的疾病，诊断可能具有挑战性。本报告讨论了一名44岁女性，她出现劳力性呼吸困难加重、端坐呼吸和下肢水肿。心电图显示肢体导联低电压和假性梗死图形。超声心动图显示双房扩大，心室腔无变化及限制性充盈生理改变。考虑诊断为心脏淀粉样变性。进行了心脏磁共振成像，显示钆延迟强化，最终心肌活检确诊为心脏淀粉样变性。该病例表明，对于病因不明的心力衰竭患者，尽管缺乏淀粉样蛋白沉积的“经典”超声心动图特征，如室间隔厚度增加或心肌“明亮闪烁”外观，但淀粉样变性仍可能是心肌病的病因。
