International guidelines for heart rhythm disorders in NMD

International specialists in neuromuscular pathology associated with cardiologists have established practical guidelines for rhythm disorders encountered in diseases such as Duchenne and Becker muscular dystrophies, limb-girdle muscular dystrophies (LGMD), nucleopathies (Emery-Dreifuss type), myotonic dystrophies (DM1 or DM2), facioscapulohumeral myopathy and two mitochondrial diseases:

  • the conclusions of the document are the result of an analysis of the literature on the subject, but also of exchanges on the practices and expertise of specialists in the field;
  • they were then endorsed by the respective learned societies;
  • the authors emphasise the need for a shared decision between the referring clinician, the patient and his or her family, especially when invasive therapeutic interventions are involved,
  • and the frequent dissociation between motor and cardiac symptoms, hence the need for systematic rhythmic monitoring at regular intervals.


2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders. Groh WJ, Bhakta D, Tomaselli GF et al. Heart Rhythm. 2022 Apr 26:S1547-5271(22)01946-4.