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Sudden Death of a Carrier of X-Linked Emery-Dreifuss Muscular Dystrophy Ann Int Med 119:900-905, Fishbein,M.C.,et al, 1993 See this aricle in Pubmed Article Abstract All three hearts showed a unique pattern of pathologic findings.Marked loss of atrial myocardium had occurred.Myocardium had been replaced by adipose and fibrous tissue.The conduction system showed no significant abnormalities.Varying degrees of interstitial and replacement fibrosis were present in the ventricular myocardium.Emery-Dreifuss muscular dystrophy is associated with characteristic pathologic changes in the heart that can cause death in female carriers and affected men.Female carriers should be identified and evaluated because of the risk for sudden cardiac death.

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