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.