Left ventricular noncompaction cardiomyopathy in Duchenne muscular dystrophy carriers

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.>.