Prophylactic implantation of cardioverter-defibrillator in patients with severe cardiac amyloidosis and high risk for sudden cardiac death

Background - Cardiac light-chain amyloidosis carries a high risk for death predominantly from progressive cardiomyopathy or sudden death (SCD). Independent risk factors for SCD are syncope and complex nonsustained ventricular arrhythmias. - Objective - The purpose of this study was to test whether p...

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Main Authors: Kristen, Arnt (Author) , Dengler, Thomas (Author) , Hegenbart, Ute (Author) , Schönland, Stefan (Author) , Goldschmidt, Hartmut (Author) , Sack, Falk-Udo (Author) , Voss, Frederik (Author) , Becker, Rüdiger (Author) , Katus, Hugo (Author) , Bauer, Alexander (Author)
Format: Article (Journal)
Language:English
Published: [February 2008]
In: Heart rhythm
Year: 2008, Volume: 5, Issue: 2, Pages: 235-240
ISSN:1556-3871
DOI:10.1016/j.hrthm.2007.10.016
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.hrthm.2007.10.016
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S1547527107010260
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Author Notes:Arnt V. Kristen, Thomas J. Dengler, Ute Hegenbart, Stefan O. Schonland, Hartmut Goldschmidt, Falk-Udo Sack, Frederik Voss, Rüdiger Becker, Hugo A. Katus, Alexander Bauer

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520 |a Background - Cardiac light-chain amyloidosis carries a high risk for death predominantly from progressive cardiomyopathy or sudden death (SCD). Independent risk factors for SCD are syncope and complex nonsustained ventricular arrhythmias. - Objective - The purpose of this study was to test whether prophylactic placement of an implantable cardioverter-defibrillator (ICD) reduces SCD in patients with cardiac amyloidosis. - Methods - Nineteen patients with histologically proven cardiac amyloidosis and a history of syncope and/or ventricular extra beats (Lown grade IVa or higher) received an ICD. - Results - During a mean follow-up of 811 ± 151 days, two patients with sustained ventricular tachyarrhythmias were successfully treated by the ICD. Two patients underwent heart transplantation, and seven patients died due to electromechanical dissociation (n = 6) or glioblastoma (n = 1). Nonsurvivors more often showed progression of left ventricular wall thickness, low-voltage pattern, ventricular arrhythmias (Lown grade IVa or higher), and higher N-terminal pro-brain natriuretic peptide levels than did survivors. Bradycardias requiring ventricular pacing (VVI 40/min <1%, DDD 60/min 6% ± 1%) occurred only rarely. - Conclusion - Patients with cardiac amyloidosis predominantly die as a result of electromechanical dissociation and other diagnoses not amenable to ICD therapy. Selected patients with cardiac amyloidosis may benefit from ICD placement. Better predictors of arrhythmia-associated SCD and randomized trials are required to elucidate the impact of ICD placement in high-risk patients with cardiac amyloidosis. 
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