Nicolò Martini , Giulio Sinigiani , Laura De Michieli , Roberta Mussinelli , Martina Perazzolo Marra , Sabino Iliceto , Alessandro Zorzi , Stefano Perlini , Domenico Corrado , Alberto Cipriani
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引用次数: 0
摘要
心脏淀粉样变性(CA)是一种浸润性心肌病,由细胞外淀粉样纤维沉积引起,淀粉样纤维主要来源于野生型或遗传变异型的转甲状腺素或免疫球蛋白轻链的错误折叠。其特征是左心室质量增加和舒张功能障碍,可导致射血分数保留的心力衰竭和/或传导障碍。诊断的依据是淀粉样蛋白沉积的侵入性病理学证明,或使用先进心血管成像技术的非侵入性标准。然而,12 导联心电图(ECG)在评估 CA 患者时仍然至关重要,因为他们可能表现出与左心室肥厚不一致的 QRS 低电压等特殊特征,也可能表现出假性心肌梗死模式、窦房结功能障碍、房室传导阻滞、室上性早搏和室性早搏,这些都支持心肌疾病的存在。为了提高诊断率和改善患者的预后,我们需要对 CA 的这些常见心电图特征有更深刻的认识。在本综述中,我们讨论了目前心电图在 CA 诊断和管理中的作用,重点是最常见的心电图异常和心律紊乱。
Electrocardiographic features and rhythm disorders in cardiac amyloidosis
Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy caused by extracellular deposition of amyloid fibrils, mainly derived from transthyretin, either wild-type or hereditary variants, or immunoglobulin light chains misfolding. It is characterized by an increased left ventricular (LV) mass and diastolic dysfunction, which can lead to heart failure with preserved ejection fraction and/or conduction disturbances. The diagnosis is based on invasive pathology demonstration of amyloid deposits, or non-invasive criteria using advanced cardiovascular imaging techniques. Nevertheless, 12-lead electrocardiogram (ECG) remains of crucial importance in the assessment of patients with CA, since they can manifest peculiar features such as low QRS voltages, in discordance with the LV hypertrophy, but also pseudo-infarction patterns, sinus node dysfunction, atrioventricular blocks, premature supraventricular and ventricular beats, which support the presence of a myocardial disease. Great awareness of these common ECG characteristics of CA is needed to increase diagnostic performance and improve patient's outcome. In the present review, we discuss the current role of the ECG in the diagnosis and management of CA, focusing on the most common ECG abnormalities and rhythm disorders.
期刊介绍:
Trends in Cardiovascular Medicine delivers comprehensive, state-of-the-art reviews of scientific advancements in cardiovascular medicine, penned and scrutinized by internationally renowned experts. The articles provide authoritative insights into various topics, encompassing basic mechanisms, diagnosis, treatment, and prognosis of heart and blood vessel disorders, catering to clinicians and basic scientists alike. The journal covers a wide spectrum of cardiology, offering profound insights into aspects ranging from arrhythmias to vasculopathies.