Résumé :
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Cardiac involvement is frequent in DM1 and represents the primary cause of death in 20 to 29% of patients. However, few data concerning cardiac complications in young DM1 patients are available. We report a series of 27 DM1 patients aged 10 to 20 year old (16.3+/-2.6 years; male=20) with cardiac complications requiring a specific pharmacological treatment or the implantation of a device (pacemaker or cardioverter defibrillator). DM1 clinical phenotype was congenital (n=3), infantile (n=10), mild (n=12) or exclusively cardiac (n=2). Symptoms were present in 20 patients (palpitations=14, syncope or near syncope=7, sudden death=2) and occurred during exercise in 11 patients. Cardiac complications were supraventricular arrhythmias (n=9), atrio-ventricular block (n=3), ventricular arrhythmias (n=2), left ventricular dysfunction (n=1). Combined complications, i.e. arrhythmias and/or conductive disease and/or ventricular dysfunction were present in 10 patients. This study confirms the existence of severe cardiac involvement in young DM1 patients. Supraventricular and ventricular arrhythmias induced by exercise are the most frequent complications. Therefore, cardiac investigations are necessary in patients > 10 year old. An exercise testing should be performed, principally in patients who practice sport. As cardiac complications are frequently combined, cardiac follow up should be particularly close in patients who have even only mild abnormalities.
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