Abstract

Introduction: TTR cardiac amyloidosis (ATTR-CM) is a progressive, infiltrative cardiomyopathy that is characterized by the deposition of amyloid fibril extracellularly in the myocardium. Patients with ATTR-CM are known to be at higher risk for arrhythmias and conduction disease, likely due to changes in the cardiac anatomy and disruption of the conduction system by amyloid fibrils. Our study aims to determine whether presence of an arrhythmia or conduction disease is associated with mortality and heart failure hospitalizations in patients with ATTR-CM and V142I mutation. Hypothesis: Patients with arrhythmia or conduction disease will have higher rates of heart failure hospitalizations (HFH) and mortality than those without it. Methods: We conducted a retrospective chart review of patients diagnosed with ATTR-CM with V142I mutations enrolled in Emory University's Cardiac Amyloidosis Clinic between January 2014 and April 2022. Kaplan-Meier (KM) curves stratified by presence of atrial arrhythmia, ventricular arrhythmia, or conduction disease were generated for mortality and first HFH within 2 years of initial diagnosis. Log-rank test was used to compare KM curves. A p-value < 0.05 was considered significant. Results: 111 patients were identified to have ATTR-CM with V142I mutation. 51.4% (57) of the patients had an atrial arrhythmia (fibrillation or flutter), 46.8% (52) had conduction abnormalities (defined as second or third degree heart block). 44.1% (49) had ventricular arrhythmias (including NSVT, sustained VT, and/or VF). Patients who had atrial arrhythmia and conduction disease were more likely to have HFH within 2 years compared to those who did not (p = 0.0011, p = 0.044 respectively). Patients with atrial arrhythmia, ventricular arrhythmia, or conduction disease were not at higher risk of mortality within 2 years (p=0.21, p=0.71, p=0.66 respectively). Conclusions: For patients with ATTR-CM with V142I mutation, atrial arrhythmia and conduction disease were risk factors for HFH within 2 years. Atrial and ventricular arrhythmia and conduction disease were not associated with higher mortality within 2 years.

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