Teresi Lucio, Trimarchi Giancarlo, Liotta Paolo, Restelli Davide, Licordari Roberto, Carciotto Gabriele, Francesco Costa, Crea Pasquale, Dattilo Giuseppe, Micari Antonio, Emdin Michele, Berruezo Antonio, Di Bella Gianluca
Department of Clinical and Experimental Medicine, University of Messina, 98100 Messina, Italy.
Heart Institute, Teknon Medical Centre, 08022 Barcelona, Spain.
J Clin Med. 2024 Sep 20;13(18):5588. doi: 10.3390/jcm13185588.
Electrophysiological aspects of cardiac amyloidosis (CA) are still poorly explored compared to other aspects of the disease. However, electrocardiogram (ECG) abnormalities play an important role in CA diagnosis and prognosis and the management of arrhythmias is a crucial part of CA treatment. Low voltages and a pseudonecrosis pattern with poor R-wave progression in precordial leads are especially common findings. These are useful for CA diagnosis and risk stratification, especially when combined with clinical or echocardiographic findings. Both ventricular and supraventricular arrhythmias are common in CA, especially in transthyretin amyloidosis (ATTR), and their prevalence is related to disease progression. Sustained and non-sustained ventricular tachycardias' prognostic role is still debated, and, to date, there is a lack of specific indications for implantable cardiac defibrillator (ICD). On the other hand, atrial fibrillation (AF) is the most common supraventricular arrhythmia with a prevalence of up to 88% of ATTR patients. Anticoagulation should be considered irrespective of CHADsVA score. Furthermore, even if AF seems to not be an independent prognostic factor in CA, its treatment for symptom control is still crucial. Finally, conduction disturbances and bradyarrhythmias are also common, requiring pacemaker implantation in up to 40% of patients.
与心脏淀粉样变性(CA)的其他方面相比,其电生理方面的研究仍较少。然而,心电图(ECG)异常在CA的诊断、预后以及心律失常的管理中起着重要作用,心律失常的管理是CA治疗的关键部分。低电压以及胸前导联R波进展不良的假性坏死模式是特别常见的表现。这些对于CA的诊断和风险分层很有用,尤其是与临床或超声心动图结果相结合时。室性和室上性心律失常在CA中都很常见,尤其是在转甲状腺素蛋白淀粉样变性(ATTR)中,其发生率与疾病进展相关。持续性和非持续性室性心动过速的预后作用仍存在争议,并且迄今为止,对于植入式心脏除颤器(ICD)缺乏具体的指征。另一方面,心房颤动(AF)是最常见的室上性心律失常,在ATTR患者中的发生率高达88%。无论CHADsVA评分如何,都应考虑抗凝治疗。此外,即使AF似乎不是CA的独立预后因素,但其症状控制治疗仍然至关重要。最后,传导障碍和缓慢性心律失常也很常见,高达40%的患者需要植入起搏器。