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Vijapurapu R, Bradlow W, Leyva F, et al. Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy. Orphanet J Rare Dis. 2022;17(1):6doi: 10.1186/s13023-021-02133-4.
Vijapurapu, R., Bradlow, W., Leyva, F., Moon, J. C., Zegard, A., Lewis, N., Kotecha, D., Jovanovic, A., Hughes, D. A., Woolfson, P., Steeds, R. P., & Geberhiwot, T. (2022). Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy. Orphanet journal of rare diseases, 17(1), 6. https://doi.org/10.1186/s13023-021-02133-4
Vijapurapu, Ravi, et al. "Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy." Orphanet journal of rare diseases vol. 17,1 (2022): 6. doi: https://doi.org/10.1186/s13023-021-02133-4
Vijapurapu R, Bradlow W, Leyva F, Moon JC, Zegard A, Lewis N, Kotecha D, Jovanovic A, Hughes DA, Woolfson P, Steeds RP, Geberhiwot T. Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy. Orphanet J Rare Dis. 2022 Jan 06;17(1):6. doi: 10.1186/s13023-021-02133-4. PMID: 34991670; PMCID: PMC8734227.
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Orphanet J Rare Dis. 2022 Jan 06;17(1):6. doi: 10.1186/s13023-021-02133-4.

Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy.

Orphanet journal of rare diseases

Ravi Vijapurapu, William Bradlow, Francisco Leyva, James C Moon, Abbasin Zegard, Nigel Lewis, D Kotecha, Ana Jovanovic, Derralynn A Hughes, Peter Woolfson, Richard P Steeds, Tarekegn Geberhiwot

Affiliations

  1. Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK.
  2. Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK.
  3. Department of Endocrinology, Department of Inherited Metabolic Disorders, Queen Elizabeth Hospital, Edgbaston, Birmingham, B15 2TH, UK.
  4. Aston Medical Research Institute, Aston Medical School, Birmingham, UK.
  5. Department of Cardiology, Barts Heart Centre, London, UK.
  6. South Yorkshire Cardiothoracic Centre, Northern General Hospital, Sheffield, UK.
  7. Mark Holland Metabolic Unit, Salford Royal Hospital, Salford, UK.
  8. Lysosomal Storage Disorder Unit, Royal Free Hospital, London, UK.
  9. Department of Cardiology, Salford Royal Hospital, Salford, UK.
  10. Department of Endocrinology, Department of Inherited Metabolic Disorders, Queen Elizabeth Hospital, Edgbaston, Birmingham, B15 2TH, UK. [email protected].
  11. Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK. [email protected].

PMID: 34991670 PMCID: PMC8734227 DOI: 10.1186/s13023-021-02133-4

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.

© 2022. The Author(s).

Keywords: Arrhythmia; Defibrillator; Fabry; Hypertrophic cardiomyopathy; Prognosis; Risk

References

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