Electrophysiologic and cardiovascular manifestations of Duchenne and Becker muscular dystrophies.

ABSTRACT

There have been significant advances in the diagnosis and management of the hereditary muscular disorders Duchenne and Becker muscular dystrophy (DMD and BMD). Cardiac electrophysiologic and cardiovascular involvement has long been important in the surveillance, care, and prognosis of patients with both BMD and DMD and is the leading cause of mortality in patients with DMD. With improved long-term prognosis, rhythm disorders and progressive cardiomyopathy with resultant heart failure are increasingly common. This review aimed to provide an overview to electrophysiologists and cardiologists of the cardiac electrophysiologic phenotypes and genetics of BMD and DMD and to highlight the recent discoveries that have advanced clinical course and management. A systematic review was performed of the diagnosis and management of DMD and BMD. The Cochrane Library, PubMed, MEDLINE, Europe PubMed Central, AMED, and Embase databases were accessed for available evidence. The research reported in this paper adhered to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Evidence from randomized controlled trials and studies cited in expert consensus and practice guidelines are examined. Advanced imaging techniques and a spectrum of rhythm disorders associated with the progressive cardiomyopathy are presented. Early initiation of heart failure therapies, the role of cardiac implantable devices, and novel gene therapies approved for use with the potential to alter the disease course are discussed. When profound cardiac and cardiac electrophysiologic involvement is diagnosed and treated earlier, outcomes for DMD and BMD patients may be improved.