Electrical storm in a patient with Duchenne muscular dystrophy cardiomyopathy triggered by abrupt ß-blocker interruption.

We present a case of a young man with Duchenne muscular dystrophy cardiomyopathy (DMDC) having an implantable cardioverter defibrillator for secondary prevention, who presented with electrical storm shortly after ß-blocker interruption. The patient was stabilized and remained free of ventricular arrhythmias soon after reinitiating b-adrenoreceptor antagonists. The present case highlights the importance of sympathetic blockage in patients with DMDC due to existing pathophysiology of excess diastolic Ca2+ leak from sarcoplasmic reticulum as a result of ryanodine receptor dysfunction.