ABC Heart Fail Cardiomyop 2021; 1(2): 103-111
Ventricular Arrythmias in Cardiac Amyloidosis: It is Possible to Prevent Sudden Death?
Carlos A. Dumont
, María Inés Sosa Liprandi
Abstract
Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy that occurs secondary to deposition of misfolded protein in the myocardium. The two most common subtypes are amyloid light chain amyloidosis and amyloid transthyretin amyloidosis. CA frequently results in congestive cardiac failure and arrhythmias, due to a disruption in cardiac substrate with subsequent electromechanical remodeling. Disease progression is usually demonstrated by development of progressive pump failure, which may be seen with a high arrhythmic burden, usually portending a poor prognosis. Arrhythmias are common, and many commonly used pharmacological therapies may be poorly tolerated and lead to clinical decompensation in this population, adding complexity to the co-management of these conditions. Studies are required to assess the risks and benefits of catheter ablation for ventricular tachycardia, with no current data showing a mortality benefit. The role of implantable cardioverter-defibrillator therapy is controversial, with benefits seen predominantly in early phases of the disease process. High-quality evidence and guideline recommendations are limited with regard to the management of arrhythmias. Providers are often left to clinical experience and expert consensus to aid in decision-making. This review summarizes both historical and contemporary data and describes evidence-based strategies for managing ventricular arrhythmias and their sequelae in patients with CA.
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