Left ventricular noncompaction cardiomyopathy in Duchenne muscular dystrophy carriers

Authors

• John J. Parent, Cincinnati Children's Hospital Medical Center
• Ryan A. Moore, Cincinnati Children's Hospital Medical Center
• Michael D. Taylor, Cincinnati Children's Hospital Medical Center
• Jeffrey A. Towbin, Cincinnati Children's Hospital Medical Center
• John L. Jefferies, Cincinnati Children's Hospital Medical Center

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.<. Learning objective: Dystrophinopathy carriers can manifest cardiac disease in the form of cardiomyopathy. We present a novel finding of carriers who manifest their cardiomyopathy in the form of left ventricular noncompaction, dilated phenotype. This has been described previously in patients with Duchenne muscular dystrophy.>.

Publication Title

Journal of Cardiology Cases

Recommended Citation

Parent, J., Moore, R., Taylor, M., Towbin, J., & Jefferies, J. (2015). Left ventricular noncompaction cardiomyopathy in Duchenne muscular dystrophy carriers. Journal of Cardiology Cases, 11 (1), 7-9. https://doi.org/10.1016/j.jccase.2014.08.010