Cardiomyopathy in becker muscular dystrophy: Overview

doi:10.4330/wjc.v8.i6.356

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World Journal of Cardiology

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World J Cardiol. Jun 26, 2016; 8(6): 356-361
Published online Jun 26, 2016. doi: 10.4330/wjc.v8.i6.356

Cardiomyopathy in becker muscular dystrophy: Overview

Rady Ho, My-Le Nguyen, Paul Mather

Rady Ho, My-Le Nguyen, Paul Mather, Department of Internal Medicine, Thomas Jefferson University Hospital, Philadelphia, PA 19107, United States

Author contributions: Ho R and Nguyen ML conducted literatures search and wrote the entire manuscript; Mather P reviewed and edited the manuscript.

Conflict-of-interest statement: The authors have no conflict of interest to report.

Correspondence to: Rady Ho, MD, Department of Internal Medicine, Thomas Jefferson University Hospital, 111 S. 11^th St, Philadelphia, PA 19107, United States. rady.ho@jefferson.edu

Telephone: +1-407-4040231 Fax: +1-215-5030052

Received: December 27, 2015
Peer-review started: December 28, 2015
First decision: February 2, 2016
Revised: April 11, 2016
Accepted: April 21, 2016
Article in press: April 22, 2016
Published online: June 26, 2016
Processing time: 179 Days and 7.4 Hours

Abstract

Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. Cardiac involvement in BMD has been described and cardiomyopathy represents the number one cause of death in these patients. In this paper, the pathophysiology, clinical evaluations and management of cardiomyopathy in patients with BMD will be discussed.

Keywords: Becker muscular dystrophy; Cardiomyopathy; X-linked recessive disorder; Dystrophin

Core tip: Becker muscular dystrophy (BMD) is an X-linked recessive disorder involving mutations of the dystrophin gene. This condition is rare but not uncommon. However, there are limited articles on this topic. Patients with BMD can present with mental retardation and diffuse muscular dystrophy. Cardiomyopathy is the number one cause of death in BMD. This paper aims to provide a comprehensive overview of BMD pathophysiology and management. The paper will discuss both the established treatments as well as exciting new research on gene therapy.