Open AccessElectrical storm in a patient with Duchenne muscular dystrophy cardiomyopathy triggered by abrupt β‐blocker interruption
Author(s) -
Fragakis Nikolaos,
Sotiriadou Melani,
Krexi Lydia,
Vassilikos Vassilios
Publication year - 2017
Publication title -
annals of noninvasive electrocardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.494
H-Index - 48
eISSN - 1542-474X
pISSN - 1082-720X
DOI - 10.1111/anec.12477
Subject(s) - medicine , duchenne muscular dystrophy , cardiomyopathy , cardiology , ryanodine receptor , muscular dystrophy , implantable cardioverter defibrillator , heart failure , receptor
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.