Bonvicini Eleonora, Preda Alberto, Tognola Chiara, Falco Raffaele, Gidiucci Roberto, Leo Giulio, Vargiu Sara, Varrenti Marisa, Gigli Lorenzo, Baroni Matteo, Carbonaro Marco, Colombo Giulia, Maloberti Alessandro, Giannattasio Cristina, Mazzone Patrizio, Guarracini Fabrizio
Department of Cardiology, S. Chiara Hospital, 38122 Trento, Italy.
Electrophysiology Unit, De Gasperis Cardio Center, Niguarda Hospital, 20162 Milan, Italy.
J Cardiovasc Dev Dis. 2024 Jul 14;11(7):222. doi: 10.3390/jcdd11070222.
Cardiac amyloidosis is the most frequent infiltrative disease caused by the deposition of misfolded proteins in the cardiac tissue, leading to heart failure, brady- and tachyarrhythmia and death. Conduction disorders, atrial fibrillation (AF) and ventricular arrhythmia (VA) significantly impact patient outcomes and demand recognition. However, several issues remain unresolved regarding early diagnosis and optimal management. Extreme bradycardia is the most common cause of arrhythmic death, while fast and sustained VAs can be found even in the early phases of the disease. Risk stratification and the prevention of sudden cardiac death are therefore to be considered in these patients, although the time for defibrillator implantation is still a subject of debate. Moreover, atrial impairment due to amyloid fibrils is associated with an increased risk of AF resistant to antiarrhythmic therapy, as well as recurrent thromboembolic events despite adequate anticoagulation. In the last few years, the aging of the population and progressive improvements in imaging methods have led to increases in the diagnosis of cardiac amyloidosis. Novel therapies have been developed to improve patients' functional status, quality of life and mortality, without data regarding their effect on arrhythmia prevention. In this review, we consider the latest evidence regarding the arrhythmic risk stratification of cardiac amyloidosis, as well as the available therapeutic strategies.
心脏淀粉样变性是最常见的浸润性疾病，由错误折叠的蛋白质沉积于心脏组织所致，可导致心力衰竭、缓慢性和快速性心律失常以及死亡。传导障碍、心房颤动（AF）和室性心律失常（VA）对患者预后有显著影响，需要引起重视。然而，在早期诊断和最佳治疗方面仍有几个问题尚未解决。极度心动过缓是心律失常性死亡的最常见原因，而即使在疾病早期也可发现快速且持续的室性心律失常。因此，尽管植入除颤器的时机仍存在争议，但对于这些患者仍应考虑进行危险分层和预防心源性猝死。此外，淀粉样纤维导致的心房损害与抗心律失常治疗耐药的房颤风险增加以及尽管充分抗凝仍发生复发性血栓栓塞事件有关。在过去几年中，人口老龄化和成像方法的不断进步导致心脏淀粉样变性的诊断率有所上升。已开发出新型疗法来改善患者的功能状态、生活质量和死亡率，但尚无关于其预防心律失常效果的数据。在本综述中，我们考虑了有关心脏淀粉样变性心律失常危险分层的最新证据以及可用的治疗策略。
