Key result
An infant with Beckwith-Wiedemann syndrome presented with supraventricular tachycardia and right atrial nodular lesions composed of hypertrophic myocardial fibers and multinucleated giant cells.
Case Report (n=1)
This case report describes a novel finding of focal giant cell cardiomyopathy in an infant with Beckwith-Wiedemann syndrome, suggesting cardiac cytomegaly as a manifestation of the syndrome's accelerated growth potential.
May indicate cardiac cytomegaly in BWS; hypothesis-generating for giant cell lesions as a syndromic feature.
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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Kapur et al. (1985) conducted a case report in Beckwith-Wiedemann syndrome with focal giant cell cardiomyopathy (n=1). Beckwith-Wiedemann syndrome was evaluated on Histopathological findings of cardiac lesions. An infant with Beckwith-Wiedemann syndrome presented with supraventricular tachycardia and right atrial nodular lesions composed of hypertrophic myocardial fibers and multinucleated giant cells.
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