Department of Medicine, Temple University Hospital-Jeanes Campus, Philadelphia, PA, USA.
Department of Medicine, Temple University Hospital-Jeanes Campus, Philadelphia, PA, USA.
J Cardiol. 2023 May;81(5):429-433. doi: 10.1016/j.jjcc.2023.02.009. Epub 2023 Mar 7.
Cardiac amyloidosis is an underdiagnosed disease that is caused by myocardial deposition of misfolded light chain (AL) or transthyretin (ATTR) amyloid fibrils, leading to restrictive cardiomyopathy and eventually death if untreated. Ventricular arrhythmias are common in cardiac amyloidosis, and the prevalence is higher in AL than ATTR. There are multiple suspected pathogenic mechanisms for ventricular arrhythmia including activation of inflammatory cascade from direct amyloid deposition, and electro-mechanical as well as autonomic dysfunction due to systemic amyloid deposition. Cardiac amyloidosis is associated with an increased risk of sudden cardiac death, and the risk is higher in AL than ATTR. Finally, the role of implantable cardioverter-defibrillators in cardiac amyloidosis is controversial, and while successful termination of life-threatening ventricular arrhythmias has been reported in few studies, there has been no evidence of improvement in outcomes when used for primary prevention in patients with cardiac amyloidosis.
心脏淀粉样变性是一种未被充分诊断的疾病，由心肌中错误折叠的轻链 (AL) 或转甲状腺素蛋白 (ATTR) 淀粉样纤维沉积引起，导致限制性心肌病，如果未经治疗，最终会导致死亡。心脏淀粉样变性中常见室性心律失常，AL 型比 ATTR 型更为常见。室性心律失常的发病机制有多种推测，包括直接淀粉样沉积引发炎症级联反应，以及全身淀粉样沉积导致的电机械和自主神经功能障碍。心脏淀粉样变性与心源性猝死风险增加相关，AL 型比 ATTR 型更高。最后，植入式心脏复律除颤器在心脏淀粉样变性中的作用存在争议，虽然少数研究报告了成功终止危及生命的室性心律失常，但在心脏淀粉样变性患者中用于一级预防时，并未有改善结局的证据。
