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Prevalence and predictors of bradyarrhythmias requiring Permanent Pacing in patients with Anderson-Fabry disease.
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  • luigi tassetti,
  • Carlo Fumagalli,
  • Alessia Argirò,
  • Mattia Zampieri,
  • Martina Gori,
  • Federica Verrillo,
  • Chiara Zocchi,
  • Francesco Cappelli,
  • Iacopo Olivotto
luigi tassetti
University Hospital Careggi

Corresponding Author:luigi.tassetti.1990@gmail.com

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Carlo Fumagalli
University Hospital Careggi
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Alessia Argirò
University Hospital Careggi
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Mattia Zampieri
University Hospital Careggi
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Martina Gori
University Hospital Careggi
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Federica Verrillo
University Hospital Careggi
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Chiara Zocchi
University Hospital Careggi
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Francesco Cappelli
University Hospital Careggi
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Iacopo Olivotto
University Hospital Careggi
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Abstract

Introduction: Bradyarrhythmias are an established red flag for storage cardiac conditions including Anderson-Fabry disease (AFD). The prevalence of bradyarrhythmias requiring a pacemaker (PM) and their timing in AFD is unresolved. We evaluated prevalence and predictors of PM requirement in a large AFD cohort, investigating the occurrence of bradyarrhythmias as initial versus late manifestation. Methods: we retrospectively evaluated 82 consecutive AFD patients referred to our multidisciplinary referral centre from 1994 to 2020 with a median follow up of 6.9 years, identifying those requiring pacing. Univariable analysis was performed to identify cardiac features associated with PM implantantion. Results: Five of 82 (6%) AFD patients required PM implantation (5/39, i.e. 13% of those with cardiac involvement), always in the context of advanced cardiomyopathy. In none, bradyarrhythmias were the presenting feature. Indications included sick sinus syndrome in 3 patients, advanced atrio-ventricular block in 2 patients. QRS prolongation during follow up strongly correlated with the onset of bradyarrhythmias. Conclusions: Severe bradyarrhythmias are relatively frequent in patients with AFD cardiomyopathy, but do not represent a mode of presentation, occurring late in the disease course and always in the context of advanced cardiac involvement. Monitoring QRS variations over time may help to identify patients requiring pacing.
14 Nov 2021Submitted to Journal of Cardiovascular Electrophysiology
15 Nov 2021Submission Checks Completed
15 Nov 2021Assigned to Editor
16 Nov 2021Reviewer(s) Assigned
29 Dec 2021Review(s) Completed, Editorial Evaluation Pending
04 Jan 2022Editorial Decision: Revise Minor
17 Jan 20221st Revision Received
17 Jan 2022Submission Checks Completed
17 Jan 2022Assigned to Editor
17 Jan 2022Reviewer(s) Assigned
02 Feb 2022Review(s) Completed, Editorial Evaluation Pending
03 Feb 2022Editorial Decision: Accept
May 2022Published in Journal of Cardiovascular Electrophysiology volume 33 issue 5 on pages 1072-1078. 10.1111/jce.15409