THE CARDIOMYOPATHY OF MUSCULAR DYSTROPHY: REPORT OF TWO CASES WITH A REVIEW OF THE LITERATURE.
About 50% of patients with progressive muscular dystrophy have a cardiomyopathy, manifested commonly by tachycardia, but also by arrythmias, refractory congestive heart failure and sudden death. Studies from the literature report manifold but nonspecific electrocardiographic changes in 41% to 85% of patients with progressive muscular dystrophy. The principal lesion is a diffuse myocardial fibrosis with minor degenerative changes in myocardial fibres unaccompanied by significant inflammation. The heart is enlarged and has a prominent deposit of epicardial fat. The myocardium is pale, coarse, flabby and friable, often showing gross evidence of scarring. The dilated chambers often contain mural thrombus.
D001145:Arrhythmias, Cardiac; D006332:Cardiomegaly; D009202:Cardiomyopathies; D004562:Electrocardiography; D004699:Endocardium; D006321:Heart; D006331:Heart Diseases; D006333:Heart Failure; D006343:Heart Septal Defects; D006801:Humans; D009136:Muscular Dystrophies; D009206:Myocardium; D010336:Pathology; D013610:Tachycardia
ARRHYTHMIA; ELECTROCARDIOGRAPHY; ENDOCARDIUM; HEART DISEASES; HEART ENLARGEMENT; HEART FAILURE, CONGESTIVE; HEART SEPTAL DEFECTS; MUSCULAR DYSTROPHY; MYOCARDIUM; PATHOLOGY; TACHYCARDIA