Recurrent ventricular tachycardia in a patient with A19D mutation-associated hereditary transthyretin amyloidosis: a case report.

Eur Heart J Case Rep

Department of Cardiology, Centre Hospitalier Universitaire Rennes, Pontchaillou Hospital, 2 rue Henri le Guillloux, Rennes 35000, France.

Published: June 2024

Background: Previous literature suggests that patients with transthyretin amyloidosis (ATTR) experience a high burden of ventricular arrhythmias. Despite this evidence, optimal strategies for arrhythmia prevention and treatment remain subject to debate.

Case Summary: We report the case of a patient with hereditary ATTR cardiomyopathy who developed recurrent ventricular tachycardia prior to a decline in his left ventricular ejection fraction (LVEF). Although he ultimately received an intracardiac device (ICD) for secondary prevention of ventricular tachycardia, his clinical course begets the question of whether more aggressive arrhythmia prevention upfront could have prevented his global functional decline.

Discussion: Given the advent of new disease-modifying therapies for ATTR, it is imperative to reconsider antiarrhythmic strategies in these patients. New decision tools are needed to decide what additional parameters (beyond LVEF ≤ 35%) may warrant ICD placement for primary prevention of ventricular arrhythmias in these patients.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11192166PMC
http://dx.doi.org/10.1093/ehjcr/ytae273DOI Listing

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