International Journal of Cardiovascular ResearchISSN: 2324-8602

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Case Report, Int J Cardiovasc Res Vol: 2 Issue: 4

Role of an Implantable Cardioverter Defibrillator in Emery Dreifuss Muscular Dystrophy: A Case Report and a Review of the Literature

Priyank Shah1*, Nishant Gupta1, Sharad Bajaj1, Mahesh Bikkina1, Fayez Shamoon2 and Raja’a Kaddaha1
1St. Joseph’s Regional Medical Center, Department of Cardiology, 703 Main Street, Paterson, New Jersey-07503, USA
2St. Michael’s Medical Center, Department of Cardiology, 111 Central Ave, Newark, New Jersey-07102, USA
Corresponding author : Priyank P Shah
249 Belleville Avenue, Apt 21-B, Bloomfield, NJ, 07003
Tel: 862-591-4954; Fax: 973-754-3376
E-mail: [email protected]
Received: June 13, 2013 Accepted: July 16, 2013 Published: July 24, 2013
Citation: Shah P, Gupta N, Bajaj S, Bikkina M, Shamoon F, et al. (2013) Role of an Implantable Cardioverter Defibrillator in Emery Dreifuss Muscular Dystrophy: A Case Report and a Review of the Literature. Int J Cardiovasc Res 2:4. doi:10.4172/2324-8602.1000133

Abstract

Role of an Implantable Cardioverter Defibrillator in Emery Dreifuss Muscular Dystrophy: A Case Report and a Review of the Literature

Emery dreifuss muscular dystrophy (EDMD) is one of the three most common inherited muscular dystrophies characterized by a triad of contractures, muscle weakness, and cardiac involvement (conduction abnormalities and/or cardiomyopathy). It can be inherited as X-linked recessive, autosomal dominant or autosomal recessive pattern. Cardiac involvement is evident in virtually all cases by the end of 4th decade. There is no relationship between the severity of cardiac and skeletal muscle involvement, irrespective of the mode of inheritance and type of mutations.

Keywords: Emery dreifuss muscular dystrophy; Sudden cardiac death; Defibrillator

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