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Left ventricular noncompaction cardiomyopathy in Duchenne muscular dystrophy carriers.


ABSTRACT: Duchenne and Becker muscular dystrophies are X-linked hereditary myopathies secondary to a dystrophinopathy resulting in progressive cardiomyopathy and heart failure. The most commonly associated cardiac involvements in these patients are dilated cardiomyopathy and conduction abnormalities; however, recent studies have shown a high prevalence of left ventricular noncompaction cardiomyopathy in patients with Duchenne muscular dystrophy. Furthermore, there is increasing awareness of cardiomyopathy in female heterozygous dystrophinopathy carriers. We report a case series of two dystrophinopathy carriers with the dilated form of left ventricular noncompaction cardiomyopathy, a newly identified association. .

SUBMITTER: Parent JJ 

PROVIDER: S-EPMC6279986 | biostudies-literature | 2015 Jan

REPOSITORIES: biostudies-literature

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Left ventricular noncompaction cardiomyopathy in Duchenne muscular dystrophy carriers.

Parent John J JJ   Moore Ryan A RA   Taylor Michael D MD   Towbin Jeffrey A JA   Jefferies John L JL  

Journal of cardiology cases 20141003 1


Duchenne and Becker muscular dystrophies are X-linked hereditary myopathies secondary to a dystrophinopathy resulting in progressive cardiomyopathy and heart failure. The most commonly associated cardiac involvements in these patients are dilated cardiomyopathy and conduction abnormalities; however, recent studies have shown a high prevalence of left ventricular noncompaction cardiomyopathy in patients with Duchenne muscular dystrophy. Furthermore, there is increasing awareness of cardiomyopathy  ...[more]

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