The Beat Episode 20: ATTR Cardiac Amyloidosis: Bridging Heart Failure and Rhythm Management (On-Demand)-The Beat Episode 20

Video Transcription

Video Summary

This Heart Rhythm Society webinar, sponsored by BridgeBio, focused on transthyretin (ATTR) cardiac amyloidosis (CA), spanning early recognition, diagnosis, treatment, and arrhythmia management. Dr. Kevin Alexander opened describing ATTR amyloidosis as a prevalent, underdiagnosed cause of heart failure, especially common in older adults and Black individuals due to the V122I gene variant. Early extracardiac signs like bilateral carpal tunnel syndrome can precede cardiac manifestations. Diagnosis relies on multimodal imaging including echocardiography, cardiac MRI, and nuclear scintigraphy combined with ruling out monoclonal gammopathy. Dr. Mazen Hanna reviewed disease-modifying therapies—namely tetramer stabilizers (tafamidis, acoramidis) and gene silencers (vutrisiran)—which have demonstrated mortality and morbidity benefits, marking a new era beyond palliative care.<br /><br />Dr. Rael Jaber addressed arrhythmia challenges, emphasizing the high prevalence of atrial fibrillation (AF) in ATTR-CA, poor correlation of standard stroke risk scores (CHA2DS2-VASc) with thrombus risk, and the essential role of transesophageal echocardiography before cardioversion. Early AF ablation may improve rhythm control and survival in early-stage disease but shows limited success later. Dr. Roy John discussed ventricular arrhythmias, highlighting their unusual mechanisms in amyloidosis—often conduction system disease-related bundle branch reentrant VT—and the limited mortality benefit of primary prevention ICDs. Lastly, Dr. Konstantinos Siontis detailed procedural realities of catheter ablation in amyloidosis patients, noting challenging atrial substrate with high complication rates but potential benefits from newer pulsed-field ablation technology.<br /><br />Panel discussion underscored the need for heightened awareness and earlier diagnosis by electrophysiologists, incorporation of arrhythmia management into holistic care, and the urgent requirement for prospective studies in the new treatment era. While therapies have improved survival, optimal arrhythmia strategies remain evolving.

Keywords

Heart Rhythm Society webinar

transthyretin cardiac amyloidosis

ATTR-CA diagnosis

early recognition of ATTR

tetramer stabilizers

gene silencers

atrial fibrillation in amyloidosis

arrhythmia management

catheter ablation challenges

prospective studies in ATTR treatment