Electrical storm in a patient with Duchenne muscular dystrophy cardiomyopathy triggered by abrupt β‐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 β‐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.
Source: Annals of Noninvasive Electrocardiology - Category: Cardiology Authors: Tags: CASE REPORT Source Type: research