Early onset of cardiomyopathy and primary prevention of sudden death in X-linked Emery–Dreifuss muscular dystrophy

Nigro, Gerardo; Russo, Vincenzo; Ventriglia, Vega Maria; Cioppa, Nadia Della; Palladino, Alberto; Nigro, Vincenzo; Calabrò, Raffaele; Nigro, Giovanni; Politano, Luisa

doi:10.1016/j.nmd.2009.12.004

« Previous Next »Neuromuscular Disorders
Volume 20, Issue 3 , Pages 174-177, March 2010

Early onset of cardiomyopathy and primary prevention of sudden death in X-linked Emery–Dreifuss muscular dystrophy

Gerardo Nigro
- Department of Cardiothoracic and Respiratory Sciences, Arrhythmology Unit, Second University of Naples, Monaldi Hospital, Naples, Italy
Vincenzo Russo
- Department of Cardiothoracic and Respiratory Sciences, Arrhythmology Unit, Second University of Naples, Monaldi Hospital, Naples, Italy
- Corresponding author. Address: Chair of Cardiology – Monaldi Hospital, Via Leonardo Bianchi, 80131 Naples, Italy. Tel.: +39 081 7064149.
Vega Maria Ventriglia
- Department of Experimental Medicine, Cardiomiology and Medical Genetics, Second University of Naples, Naples, Italy
Nadia Della Cioppa
- Department of Anesthesiology, Pediatric Cardiac Surgery, Monaldi Hospital, Naples, Italy
Alberto Palladino
- Department of Experimental Medicine, Cardiomiology and Medical Genetics, Second University of Naples, Naples, Italy
Vincenzo Nigro
- Department of General Pathology, Second University of Naples, Naples, Italy
- Centre of Excellence for Cardiovascular Diseases, Second University of Naples, Naples, Italy
Raffaele Calabrò
- Department of Cardiothoracic and Respiratory Sciences, Arrhythmology Unit, Second University of Naples, Monaldi Hospital, Naples, Italy
Giovanni Nigro
- Department of Experimental Medicine, Cardiomiology and Medical Genetics, Second University of Naples, Naples, Italy
- Centre of Excellence for Cardiovascular Diseases, Second University of Naples, Naples, Italy
Luisa Politano
- Department of Experimental Medicine, Cardiomiology and Medical Genetics, Second University of Naples, Naples, Italy
- Centre of Excellence for Cardiovascular Diseases, Second University of Naples, Naples, Italy

Received 15 December 2008; received in revised form 2 December 2009; accepted 3 December 2009.

Abstract

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.

Keywords: Sudden cardiac death, X-linked Emery–Dreifuss muscular dystrophy, Implantable cardioverter-defibrillator, Novel mutation, Primary prevention, Cardiomyopathy