Early onset of cardiomyopathy and primary prevention of sudden death in X-linked Emery-Dreifuss muscular dystrophy.
We report the case of 14-year-old boy with X-linked Emery-Dreifuss muscular dystrophy who developed sick sinus syndrome and required placement of an implantable intracardiac cardioverter-defibrillator (ICD) to prevent sudden death. He demonstrated no significant risk factors for sudden death such as depressed left ventricular ejection fraction, or spontaneous or inducible ventricular tachycardia. One month after implantation, the patient experienced one appropriate ICD discharge.
Nigro
Gerardo
G
Department of Cardiothoracic and Respiratory Sciences, Second University of Naples, Monaldi Hospital, Naples, Italy.
Russo
Vincenzo
V
Ventriglia
Vega Maria
VM
Della Cioppa
Nadia
N
Palladino
Alberto
A
Nigro
Vincenzo
V
Calabrò
Raffaele
R
Nigro
Giovanni
G
Politano
Luisa
L
D000293:Adolescent; D009202:Cardiomyopathies / Q000209:etiology* / Q000473:pathology; D016757:Death, Sudden, Cardiac / Q000517:prevention & control*; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D006801:Humans; D008297:Male; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000150:complications*; D012804:Sick Sinus Syndrome / Q000209:etiology; D017180:Tachycardia, Ventricular / Q000209:etiology / Q000517:prevention & control