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Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy.
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.
Afiliación
  • Vijapurapu R; Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK.
  • Bradlow W; Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK.
  • Leyva F; Department of Endocrinology, Department of Inherited Metabolic Disorders, Queen Elizabeth Hospital, Edgbaston, Birmingham, B15 2TH, UK.
  • Moon JC; Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK.
  • Zegard A; Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK.
  • Lewis N; Aston Medical Research Institute, Aston Medical School, Birmingham, UK.
  • Kotecha D; Department of Cardiology, Barts Heart Centre, London, UK.
  • Jovanovic A; Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK.
  • Hughes DA; South Yorkshire Cardiothoracic Centre, Northern General Hospital, Sheffield, UK.
  • Woolfson P; Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK.
  • Steeds RP; Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK.
  • Geberhiwot T; Mark Holland Metabolic Unit, Salford Royal Hospital, Salford, UK.
Orphanet J Rare Dis ; 17(1): 6, 2022 01 06.
Article en En | MEDLINE | ID: mdl-34991670
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.
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Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Cardiomiopatía Hipertrófica / Enfermedad de Fabry / Taquicardia Ventricular / Desfibriladores Implantables Tipo de estudio: Etiology_studies / Prognostic_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2022 Tipo del documento: Article

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Cardiomiopatía Hipertrófica / Enfermedad de Fabry / Taquicardia Ventricular / Desfibriladores Implantables Tipo de estudio: Etiology_studies / Prognostic_studies / Risk_factors_studies Límite: Humans Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2022 Tipo del documento: Article