Electrophysiological Manifestations of Cardiac Amyloidosis: JACC: CardioOncology State-of-the-Art Review

Jack Hartnett, Wael Jaber, Matthew Maurer, Brett Sperry, Mazen Hanna, Patrick Collier, Divyang R Patel, Oussama M Wazni, Eoin Donnellan, Jack Hartnett, Wael Jaber, Matthew Maurer, Brett Sperry, Mazen Hanna, Patrick Collier, Divyang R Patel, Oussama M Wazni, Eoin Donnellan

Abstract

Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy caused by the extracellular deposition of amyloid fibrils in the myocardium. Although cardiac amyloidosis patients primarily present with heart failure symptoms, arrhythmias and conduction system disease are frequently encountered. Atrial fibrillation (AF) is observed in up to 70% of patients at the time of diagnosis, and patients typically have controlled ventricular rates caused by concomitant conduction system disease. Thromboembolic risk is particularly high in patients with CA and AF, and left atrial thrombi have been observed even in the absence of clinically diagnosed AF. Atrioventricular nodal and infra-Hisian disease are common, and permanent pacemakers are frequently required. The use of implantable cardioverter-defibrillators in this population is controversial. This review summarizes the published data and therapeutic strategies surrounding arrhythmias and conduction system disease with the goal of aiding clinicians managing the clinical complexities of CA.

Keywords: AF, atrial fibrillation; AL, light chain; ATTR, transthyretin; AV, atrioventricular; CA, cardiac amyloidosis; DCCV, direct current cardioversion; ICD, implantable cardioverter-defibrillator; atrial fibrillation; cardiac amyloidosis; cardiac implantable electronic devices; conduction abnormalities; congestive heart failure.

Conflict of interest statement

Dr Maurer has received grant support from National Institutes of Health R01HL139671, R21AG058348, and K24AG036778; has received consulting income from Pfizer, Eidos, Prothena, Akcea, GlaxoSmithKline, Intellia, Regeneron, and Alnylam; and his institution has received clinical trial funding from Pfizer, Prothena, Eidos, and Alnylam. Dr Hanna has served on advisory boards for Pfizer, Alnylam, Eidos, and Akcea. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

© 2021 The Authors.

Figures

Graphical abstract
Graphical abstract
Central Illustration
Central Illustration
Electrophysiologic Manifestations of Cardiac Amyloidosis Electrophysiological manifestations (dark blue box) and management considerations (light blue box) of cardiac amyloidosis include atrial fibrillation, LAA thrombus, and atrioventricular nodal disease. There is also a risk of left and right bundle branch block in both light chain and transthyretin cardiac amyloidosis. ICD = implantable cardioverter-defibrillator; LAA = left atrial appendage.
Figure 1
Figure 1
Echocardiographic Features of Atrial Myopathy in Cardiac Amyloidosis Transesophageal echocardiogram in a patient with transthyretin cardiac amyloidosis demonstrating the following: (A) an enlarged left atrium; (B) left atrial appendage thrombus with overlying sludge; and (C) low left atrial appendage emptying velocity (16.5 cm/s).
Figure 2
Figure 2
Electrocardiographic Features of Cardiac Amyloidosis Electrocardiograms demonstrating (A) atrial fibrillation, (B) right ventricular apical pacing, and (C) biventricular pacing in patients with transthyretin cardiac amyloidosis. (D) An electroanatomic map in the posteroanterior projection showing extensive atrial scarring (red) in an enlarged left atrium. The areas in purple denote healthy left atrial myocardium.

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