Management of Arrythmias in Cardiac Amyloidosis

Cardiac amyloidosis presents a diverse spectrum of electrical disturbances that significantly impact patient outcomes and management. This chapter comprehensively reviews the three main categories of electrical manifestations in cardiac amyloidosis. The prevalence and characteristics of these disturbances vary notably between amyloid subtypes, with AL cardiac amyloidosis showing higher rates of arrhythmias (up to 60%) compared to ATTR cardiac amyloidosis (20–40%). Atrial fibrillation, the most common arrhythmia, presents unique challenges in management, including high thrombotic risk regardless of conventional risk scores and limited tolerance to standard rate-control medications. Ventricular arrhythmias, more prevalent in AL amyloidosis, pose significant mortality risk with one-year mortality rates of 50% in affected patients, though the role of ICD therapy remains controversial due to high rates of electromechanical dissociation. Conduction system disorders, particularly affecting the His-Purkinje system, are highly prevalent and often necessitate pacing, with evidence suggesting potential adverse effects from high right ventricular pacing burden and possible benefits from biventricular pacing. The chapter explores the pathophysiology, clinical implications, and management strategies for each type of electrical disturbance, incorporating emerging evidence and evolving treatment approaches. The complex interplay between the restrictive physiology of cardiac amyloidosis and its electrical manifestations necessitates careful individualization of management strategies, as conventional anti-arrhythmic approaches often prove poorly tolerated. Understanding these electrical manifestations and their management challenges is crucial for optimizing outcomes in this unique patient population.