A European study clarifies the cardiac phenotype of children with muscular laminopathy

European neuropaediatricians and cardiologists have compiled the clinical and paraclinical data, in particular cardiac data, of a large cohort of 28 children with various phenotypes of laminopathy:

  • 13 presented with an Emery-Dreifuss type phenotype, 11 with LMNA-related congenital muscular dystrophy (L-CMD), two with limb-girdle muscular dystrophy and two with moderate muscular deficiency,
  • During follow-up, six patients developed dilated cardiomyopathy and five malignant cardiac arrhythmias, with these two complications occurring concomitantly in four patients,
  • Severe arrhythmias appear to be a feature of early-onset L-CMD.

These data will be used to draw up practical recommendations for monitoring these patients, who remain at high risk of cardiac complications.


Characterization of cardiac involvement in children with LMNA-related muscular dystrophy. Cesar S, Campuzano O, Cruzalegui J et al. Front Cell Dev Biol. 2023 Mars.