Duchenne muscular dystrophy (DMD) is the most common neuromuscular disease in boys. It is an X-linked recessive disease caused by dystrophin deficiency. With improved medico-surgical management, patients experience increased longevity and, as a result, a higher incidence of cardiac complications.
In an article published in 2018, French myologists were interested in the predictive value of an electrocardiographic biomarker observed with a high frequency in DMD, in this case the presence of a left bundle branch block (LBBB).
The medical files of 121 adults with DMD (18 to 41 years of age) were retrospectively reviewed during their multidisciplinary assessment in Garches or the Institute of Myology in Paris. Thirteen per cent had this abnormality, the presence of which was correlated with the subsequent occurrence of cardiac complications and therefore, a higher mortality. Residual traces of dystrophin were detected in some patients by immunocytochemistry and Western blots, but without being clearly correlated with the severity of cardiac abnormalities. Although a cardiac resynchronization therapy (CRT) device was offered to 2 patients, the authors emphasize the interest of these new implantable devices and the need to conduct pilot studies in this area.
