Focal Giant Cell Cardiomyopathy with Beckwith-Wiedemann Syndrome |
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Authors: | Sudesh Kapur Karen S. Kuehl Frank M. Midgely Roma S. Chandra |
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Affiliation: | a Departments of Pathology, Cardiology, and Surgery, Children's Hospital National Medical Center and George Washington, University School of Medicine, Washington, DC |
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Abstract: | Cardiac involvement in Beckwith-Wiedemann syndrome is mostly limited to mild cardiomegaly. Although these patients have visceromegaly, macroglossia, gigantism, and adrenal cytomegaly, no significant myocardial changes have been described. An infant with dysmorphic features of this syndrome had supraventricular tachycardia since birth. Nodular lesions were present in the right atrium. Morphologically these lesions were composed of hypertrophic myocardial fibers admixed with multinucleated giant cells of myogenic origin. The exact nature of these lesions remains undetermined. It is postulated that hypertrophic myocardial cells may represent cardiac cytomegaly as a manifestation of the accelerated growth potential of cells seen with this syndrome. |
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Keywords: | Beckwith-Wiedemann syndrome cardiomyopathy giant cell |
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