Management of Arrhythmias in Cardiac Amyloidosis

JACC Clin Electrophysiol. 2020 Apr;6(4):351-361. doi: 10.1016/j.jacep.2020.01.004.


Cardiac amyloidosis is characterized by extracellular protein fibril deposition in the myocardium leading to restrictive heart failure. Both atrial and ventricular arrhythmias, along with conduction disease, are common in cardiac amyloidosis, and are often highly symptomatic and poorly tolerated. Many commonly used therapeutics such as beta-blockers, calcium-channel blockers, and digoxin may be poorly tolerated and lead to clinical decompensation in this population, adding complexity to the co-management of these conditions. In addition, studies have shown that atrial fibrillation with cardiac amyloidosis carries a high risk of stroke and systemic embolism, making anticoagulation indicated in all patients regardless of CHA2DS2-VASc score. Ventricular arrhythmias are common, whereas an implantable cardioverter-defibrillator has not been shown to improve survival. Conduction disease is also common and permanent pacemaker placement is often needed. High-quality evidence and guideline recommendations are limited with regard to the management of arrhythmias in cardiac amyloidosis. Providers are often left to clinical experience and expert consensus to aid in decision-making. In this focused review, we outline current guideline recommendations, summarize both historical and contemporary data, and describe evidence-based strategies for managing arrhythmias and their sequelae in patients with cardiac amyloidosis.

Keywords: atrial fibrillation; cardiac amyloidosis; heart failure; stroke; ventricular arrhythmias.

Publication types

  • Review

MeSH terms

  • Amyloidosis* / complications
  • Amyloidosis* / therapy
  • Atrial Fibrillation* / complications
  • Atrial Fibrillation* / therapy
  • Defibrillators, Implantable*
  • Heart
  • Humans
  • Stroke*