An international consortium of researchers and clinicians investigated disorders of cardiac function and/or cardiac rhythm during the course of Emery-Dreifuss muscular dystrophy linked to the gene encoding emerin (EMD1) of X-linked recessive transmission :
- longitudinal clinical and electrophysiological data from 38 men with EMD1 and 21 symptomatic female EMD1 transmitters were analysed in the study,
- the incidence of severe conduction disorders and significant deterioration in left ventricular function appears to be very high in this population,
- these complications are comparable to those observed in patients with the autosomal dominant form of Emery-Dreifuss muscular dystrophy (EMD2).
Consequently, the authors recommend a more pro-active prophylactic approach for EMD1.
