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Arrhythmias and sudden death
Original research article
Study of indications for cardiac device implantation and utilisation in Fabry cardiomyopathy
  1. Ravi Vijapurapu1,2,3,
  2. Tarekegn Geberhiwot3,4,
  3. Ana Jovanovic5,
  4. Shanat Baig1,2,3,
  5. Sabrina Nordin6,
  6. Rebecca Kozor7,
  7. Francisco Leyva8,
  8. Dipak Kotecha1,2,
  9. Nigel Wheeldon9,
  10. Patrick Deegan10,
  11. Rosemary A Rusk11,
  12. James C Moon6,
  13. Derralynn A Hughes12,
  14. Peter Woolfson13,
  15. Richard P Steeds1,2
  1. 1 Department of Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, UK
  2. 2 Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK
  3. 3 Department of Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham, UK
  4. 4 Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK
  5. 5 Mark Holland Metabolic Unit, Salford Royal Hospitals NHS Trust, Salford, UK
  6. 6 Department of Cardiology, Barts Heart Centre, London, UK
  7. 7 Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia
  8. 8 Aston Medical Research Institute, Aston Medical School, Birmingham, UK
  9. 9 South Yorkshire Cardiothoracic Centre, Northern General Hospital, Sheffield, UK
  10. 10 Department of Medicine, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  11. 11 Department of Cardiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
  12. 12 Lysosomal Storage Disorder Unit, Royal Free London NHS Foundation Trust, London, UK
  13. 13 Department of Cardiology, Salford Royal Hospitals NHS Trust, Salford, UK
  1. Correspondence to Dr Tarekegn Geberhiwot, Endocrinology, Queen Elizabeth Hospital Birmingham, Birmingham B15 2GW, UK; tarekegn.geberhiwot{at}uhb.nhs.uk

Abstract

Background Fabry disease is a treatable X-linked condition leading to progressive cardiomyopathy, arrhythmia and premature death. Atrial and ventricular arrhythmias contribute significantly to adverse prognosis; however, guidance to determine which patients require cardiovascular implantable electronic devices (CIEDs) is sparse. We aimed to evaluate indications for implantation practice in the UK and quantify device utilisation.

Methods In this retrospective study, we included demographic, clinical and imaging data from patients in four of the largest UK Fabry centres. Ninety patients with Fabry disease were identified with CIEDs implanted between June 2001 and February 2018 (FD-CIED group). To investigate differences in clinical and imaging markers between those with and without devices, these patients were compared with 276 patients without a CIED (FD-control).

Results In the FD-CIED group, 92% of patients with permanent pacemakers but only 28% with implantable cardioverter-defibrillators had a class 1 indication for implantation. A further 44% of patients had defibrillators inserted for primary prevention outside of current guidance. The burden of arrhythmia requiring treatment in the FD-CIED group was high (asymptomatic atrial fibrillation:

29%; non-sustained ventricular tachycardia requiring medical therapy alone: 26%; sustained ventricular tachycardia needing anti-tachycardia pacing/defibrillation: 28%). Those with devices were older, had greater LV mass, more scar tissue and larger atrial size.

Conclusions Arrhythmias are common in Fabry patients. Those with cardiac devices had high rates of atrial fibrillation requiring anticoagulation and ventricular arrhythmia needing device treatment. These are as high as those in hypertrophic cardiomyopathy, supporting the need for Fabry-specific indications for device implantation.

  • fabry
  • arrhythmia
  • defibrillator
  • prognosis
  • risk

This is an open access article distributed in accordance with the Creative Commons Attribution 4.0 Unported (CC BY 4.0) license, which permits others to copy, redistribute, remix, transform and build upon this work for any purpose, provided the original work is properly cited, a link to the licence is given, and indication of whether changes were made. See: https://creativecommons.org/licenses/by/4.0/.

https://doi.org/10.1136/heartjnl-2019-315229

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    Footnotes

    • Contributors RPS and TG conceived and designed the study. RV, PW, DH, AJ, RAR, PD, RPS and TG cared for patients, analysed the data and drafted the manuscript. All coauthors contributed to data interpretation and subsequent editing of the manuscript.

    • Funding The study was funded by the Mucopolysaccharidosis UK patient society (MPS UK).

    • Competing interests None declared.

    • Patient consent for publication Not required.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.

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