Electrical storm in a patient with Duchenne muscular dystrophy cardiomyopathy triggered by abrupt β‐blocker interruption. Issue 6 (15th June 2017)

• Record Type: Journal Article
• Authors: Fragakis, Nikolaos; Sotiriadou, Melani; Krexi, Lydia; Vassilikos, Vassilios
• Is Part Of: Annals of noninvasive electrocardiology. Volume 22:Issue 6(2017:Nov.)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2017-06-15
• Subjects: β‐blocker -- cardiomyopathy -- duchenne muscular dystrophy -- electrical storm -- implantable cardioverter defibrillator; Electrocardiography -- Periodicals; Arrhythmia -- Periodicals; 616.1207547
• DOI: 10.1111/anec.12477
• Languages: English
• ISSNs: 1082-720X

Abstract

Abstract : 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 Ca 2+ leak from sarcoplasmic reticulum as a result of ryanodine receptor dysfunction.