Vijapurapu, Ravi, Bradlow, William, Leyva, Francisco, Moon, James C., Zegard, Abbasin, Lewis, Nigel, Kotecha, D., Jovanovic, Ana, Hughes, Derralynn A., Woolfson, Peter, Steeds, Richard P. and Geberhiwot, Tarekegn (2022). Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy. Orphanet Journal of Rare Diseases, 17 (1),
Abstract
Background: Fabry disease (FD) is a treatable X-linked condition leading to progressive cardiac disease, arrhythmia and premature death. We aimed to increase awareness of the arrhythmogenicity of Fabry cardiomyopathy, by comparing device usage in patients with Fabry cardiomyopathy and sarcomeric HCM. All Fabry patients with an implantable cardioverter defibrillator (ICD) implanted in the UK over a 17 year period were included. A comparator group of HCM patients, with primary prevention ICD implantation, were captured from a regional registry database. Results: Indications for ICD in FD varied with 72% implanted for primary prevention based on multiple potential risk factors. In FD and HCM primary prevention devices, arrhythmia occurred more frequently in FD over shorter follow-up (HR 4.2, p < 0.001). VT requiring therapy was more common in FD (HR 4.5, p = 0.002). Immediate shock therapy for sustained VT was also more common (HR 2.5, p < 0.001). There was a greater burden of AF needing anticoagulation and NSVT in FD (AF: HR 6.2, p = 0.004, NSVT: HR 3.1, p < 0.001). Conclusion: This study demonstrates arrhythmia burden and ICD usage in FD is high, suggesting that Fabry cardiomyopathy may be more ‘arrhythmogenic’ than previously thought. Existing risk models cannot be mutually applicable and further research is needed to provide clarity in managing Fabry patients with cardiac involvement.
Publication DOI: | https://doi.org/10.1186/s13023-021-02133-4 |
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Divisions: | College of Health & Life Sciences > Aston Medical School > Translational Medicine Research Group (TMRG) College of Health & Life Sciences > Aston Medical School |
Additional Information: | Copyright © The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made |
Uncontrolled Keywords: | Risk,Prognosis,Hypertrophic cardiomyopathy,Arrhythmia,Fabry,Defibrillator |
Publication ISSN: | 1750-1172 |
Last Modified: | 09 Dec 2024 09:02 |
Date Deposited: | 27 Jun 2023 08:58 |
Full Text Link: | |
Related URLs: |
https://ojrd.bi ... 023-021-02133-4
(Publisher URL) |
PURE Output Type: | Article |
Published Date: | 2022-01-06 |
Accepted Date: | 2021-11-27 |
Submitted Date: | 2021-09-30 |
Authors: |
Vijapurapu, Ravi
Bradlow, William Leyva, Francisco Moon, James C. Zegard, Abbasin Lewis, Nigel Kotecha, D. Jovanovic, Ana Hughes, Derralynn A. Woolfson, Peter Steeds, Richard P. Geberhiwot, Tarekegn |