Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders Guidelines Pocket Guide - Guideline Central

Evaluation and Management of Arrhythmic Risk in Neuromuscular Disorders

Heart Rhythm SocietyPublished: May 8, 2023

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  • Top 10 Take-Home Messages 
  • Duchenne, Becker, and Recessive Limb-girdle Muscular Dystrophies 
  • Myotonic Dystrophy Types 1 and 2 
  • Emery-Dreifuss and Limb-girdle Type 1B Muscular Dystrophy 
  • Facioscapulohumeral Muscular Dystrophy 
  • Mitochondrial Myopathies Including Friedreich Ataxia 
  • Shared Decision-making and End-of-life Care
  • Tables 
    • Genetics, cardiovascular complications, and NMDs
    • Recessive forms (type 2) of LGMDs associated with CM, listed according to the subtype nomenclature used in this guide
    • Clinical scenarios for the management of arrhythmias in DMD, BMD, and recessive LGMD2
    • Clinical scenarios for the management of arrhythmias in DM1
    • Clinical scenarios for the management of arrhythmias in EDMD and LGMD1B
    • Clinical scenarios for management of patients with FA 
    • NMDs, use of pacemakers and ICDs, shared decision-making principles, and end-of-life care decisions 
    • Clinical scenarios for end-of-life management in atients with NMD
  • Figures 
    • Rhythm management and CIED in patients with DMD, BMD, or recessive LGMD2 and left ventricular dysfunction
    • Rhythm management and CIED implantation in patients with DM1 or DM2 with normal ventricular function and with focus on the risk stratification for the prevention of sudden death
    • Rhythm management and CIED implantation in patients with EDMD or LGMD1B
    • Rhythm management and CIED implantation in patients with mitochondrial myopathies including FA


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