Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy

Orphanet Journal of Rare Diseases

James C. Moon¹  William Bradlow²  Abbasin Zegard²  Francisco Leyva³  D. Kotecha⁴  Richard P. Steeds⁴  Ravi Vijapurapu⁵  Peter Woolfson⁶  Tarekegn Geberhiwot⁷  Derralynn A. Hughes⁸  Ana Jovanovic⁹  Nigel Lewis^1,10 

[1] Department of Cardiology, Barts Heart Centre, London, UK;Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK;Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK;Aston Medical Research Institute, Aston Medical School, Birmingham, UK;Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK;Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK;Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK;Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK;Department of Endocrinology, Department of Inherited Metabolic Disorders, Queen Elizabeth Hospital, B15 2TH, Edgbaston, Birmingham, UK;Department of Cardiology, Salford Royal Hospital, Salford, UK;Department of Endocrinology, Department of Inherited Metabolic Disorders, Queen Elizabeth Hospital, B15 2TH, Edgbaston, Birmingham, UK;Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK;Lysosomal Storage Disorder Unit, Royal Free Hospital, London, UK;Mark Holland Metabolic Unit, Salford Royal Hospital, Salford, UK;South Yorkshire Cardiothoracic Centre, Northern General Hospital, Sheffield, UK;

关键词: Fabry;    Hypertrophic cardiomyopathy;    Arrhythmia;    Defibrillator;    Prognosis;    Risk;   

DOI  :  10.1186/s13023-021-02133-4

来源: Springer

【 摘 要 】

BackgroundFabry 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.ResultsIndications 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).ConclusionThis 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.

【 授权许可】

CC BY