Arrhythmogenesis in Fabry Disease

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• Orsborne C, Bradley J, Bonnett LJ, Pleva LA, Naish JH, Clark DG, et al. Validated model for prediction of adverse cardiac outcome in patients with Fabry disease. J Am Coll Cardiol. 2022;80(10):982–94. This important studyhighlights how risk-prediction for arrhythmia can be accurately modelled in Fabry disease using advance cardiac magnetic resonance techniques but requires further external validation.

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•• Vijapurapu R, Roy A, Demetriades P, Warfield A, Hughes DA, Moon J, et al. Systematic review of the incidence and clinical risk predictors of atrial fibrillation and permanent pacemaker implantation for bradycardia in Fabry disease. Open Heart. 2023;10(2). This review assessing atrial fibrillation and bradycardia requiring device implantation concludes that whilst reporting of both are limited to single-centre studies and have variable reporting dependent on diagnostic modality, the burden of brady-arrhythmia and atrial fibrillation requiring therapy are likely to be much higher than reported. Factors such as left ventricular hypertrophy, age and atrial volume dilatation may contribute to the burden of arrhythmia.

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