Author + information
- Received October 18, 2019
- Revision received December 28, 2019
- Accepted January 9, 2020
- Published online April 20, 2020.
- Shaun Giancaterino, MD,
- Marcus A. Urey, MD,
- Douglas Darden, MD and
- Jonathan C. Hsu, MD, MAS∗ ()
- Cardiac Electrophysiology Section, Division of Cardiology, Department of Medicine, University of California, San Diego, La Jolla, California
- ↵∗Address for correspondence:
Dr. Jonathan C. Hsu, Cardiac Electrophysiology Section, Division of Cardiology, Department of Medicine, University of California-San Diego, 9452 Medical Center Drive, 3rd Floor, Room 3E-417, La Jolla, California 92037.
• Atrial and ventricular arrhythmias and conduction disease are common in cardiac amyloidosis.
• Atrial fibrillation may be difficult to rate control and carries a high risk of stroke.
• Implantable cardioverter-defibrillator placement for prevention of sudden cardiac death is controversial.
• New therapies are likely to change prognosis and therefore further studies are needed.
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.
Dr. Hsu has received honoraria from Medtronic, Abbott, Boston Scientific, Biotronik, Janssen, Bristol-Myers Squibb, Altathera Pharmaceuticals, and Biosense-Webster; has received research grants from Biosense-Webster and Biotronik; and has equity interest in Acutus Medical and Vektor Medical. Dr. Urey was a member of the Speakers’ Bureau for Akcea, Alynylam, and Pfizer; and has received honoraria from Akcea Therapeutics, Alnylam Pharmaceuticals, and Pfizer. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Clinical Electrophysiology author instructions page.
- Received October 18, 2019.
- Revision received December 28, 2019.
- Accepted January 9, 2020.
- 2020 American College of Cardiology Foundation
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