Novel insights on ventricular arrhythmias in cardiac amyloidosis: an updated narrative review.

Source: PubMed, NCBI / U.S. National Library of Medicine

Cardiac amyloidosis (CA) poses a significant risk for ventricular arrhythmias and sudden cardiac death. Arrhythmic risk differs by subtype: the light-chain variant is often more arrhythmogenic due to direct light-chain toxicity, while the transthyretin form is dominated by conduction system disease. Although advanced imaging, particularly cardiac magnetic resonance, accurately predicts all-cause mortality, it fails to reliably distinguish patients who will have a shockable arrhythmic event from those who will die of progressive heart failure or electromechanical dissociation. This limitation undermines the evidence for prophylactic implantable cardioverter-defibrillator therapy, leaving its role highly controversial. This review critically examines the current evidence on the pathophysiology, risk stratification, and management of ventricular arrhythmias in CA by analyzing the utility and limitations of diagnostic tools and evaluating therapeutic strategies, including the emerging role of catheter ablation. Furthermore, we discuss how effective disease-modifying therapies might redefine the natural history of CA, making the prevention of sudden cardiac death an even more critical challenge.