Cardiomyopathy is a frequent finding in patients with muscular dystrophy. To determine whether there was evidence of heart disease, we studied the mouse model dy/dy, which is a naturally occurring mutant model for human congenital muscular dystrophy (CMD). The molecular defect has characterized as a deficiency in the expression of laminin-α2, a component of the extracellular matrix. The phenotype associated with the homozygous dy/dy mouse includes a severely progressive myopathy that leaves the animals with pronounced weakness (predominantly in the hind legs), severe kyphosis, runting, and premature death at six months or less. Nine dy/dy and five control C57BL/6J male mice were studied at 14 and 18 weeks of age. Echocardiograms were performed on anesthetized animals using an ATL™ HDI 3000 cardiac ultrasound system with a 10 MHZ curvilinear array transducer. Measurements were made using 2D echo guided M-mode traces during systole and diastole for left ventricular inner diameter, left ventricular posterior wall thickness, and intraventricular septal thickness. As a group, the dy/dy mice had mild, but progressive hypertrophic cardiomyopathy that was evident by 18 weeks. A sub-group (4 animals) had severe hypertrophic cardiomyopathy that was evident grossly by echocardiography. Laminin interfaces between the extracellular matrix and the dystrophin-associated glycoprotein complex, which in turn binds to dystrophin and the cytoskeletal structural protein, actin. Our findings implicate this group of proteins as having an important role in cardiac disease, and validates the applicability of echocardiography in the mouse model.