Registry Evaluation Provides Insights into Rhythmic Disturbances of Patients with Cardiac Amyloidosis and Implantation of Cardiac Electronic Devices

During a poster session at the 2020 American Heart Association virtual meeting, Jae Hyung Cho, MD, PhD, and colleagues from the Cedars-Sinai Smidt Heart Institute, Los Angeles, CA, discussed the results of a study that evaluated the frequency of cardiac arrhythmias in patients with cardiac amyloidosis and the burden they place on patients, specifically in the form of implantable cardiac electronic devices. They noted that the full extent of the impact of cardiac arrhythmias has not been evaluated in a large patient population and remains to be elucidated.

To evaluate the scope of, and investigate the prevalence and nature of, cardiac arrhythmias in patients with cardiac amyloidosis, researchers examined the Cedars-Sinai amyloidosis registry. They reviewed implantable cardioverter-defibrillator or pacemaker implantation, ambulatory ECG monitoring data, and interrogation data to determine the severity of cardiac arrhythmia burden in patients with cardiac amyloidosis.

Of 156 patients evaluated in the registry, approximately one-third (32.7%) of patients (N = 51) presented with light chain amyloidosis, 101 (64.7%) patients presented with transthyretin amyloidosis, and 4 patients presented with AA amyloidosis (2.6%). Of the 37 (23.7%) patients who received an implantable cardioverter-defibrillator, 23 (14.7%) patients received it for primary prevention, 11 (7.1%) patients received it for secondary prevention of ventricular tachycardia, and 3 (1.9%) patients received it for secondary prevention of ventricular fibrillation. Pacemaker implantation was necessary in 22 (12.1%) patients; 13 (8.3%) patients received it due to sick sinus syndrome and 9 (5.8%) patients required it for high-grade or complete heart block.

The most common form of arrhythmia observed in this study, atrial fibrillation, occurred in more than half (51.3%) of patients (N = 80). The next most common arrhythmia was first-degree atrioventricular block, which was experienced by 31 (19.9%) patients. Fourteen (8.9%) patients experienced sustained ventricular tachycardia during hospitalization, ambulatory monitoring, or device interrogation.

Cardiac amyloidosis frequently is associated with atrial and ventricular arrhythmias, which often necessitates cardiac electronic devices implantation.


Cho JH, Cuk N, Leong D, et al. Rhythmic disturbances in patients with cardiac amyloidosis: analysis from Cedars-Sinai amyloidosis registry. Presented at: American Heart Association Scientific Sessions 2020; November 13-20, 2020. Abstract P1602-2020.

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