Focal giant-cell cardiomyopathy.
A newborn with supraventricular arrhythmia diagnosed during fetal life was found at autopsy to have a small nodule with hypertrophic and multinucleated myocardial cells in the atrial septum, near the atrioventricular node. There were no stigmata of Beckwith-Wiedemann syndrome or tuberous sclerosis. This focal giant-cell cardiomyopathy has been described only once, in an infant with Beckwith-Wiedemann syndrome. It is suggested that these dysplastic myocardial cells may be responsible for the atrial arrhythmia and that this hamartomatous lesion has the potential for malignant transformation.
Drut
R M
RM
Department of Pathology, Hospital de Niños, La Plata, Argentina.
Drut
R
R
D001145:Arrhythmias, Cardiac / Q000151:congenital; D001344:Autopsy; D009202:Cardiomyopathies / Q000150:complications / Q000151:congenital* / Q000473:pathology; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007710:Klebsiella Infections / Q000150:complications; D018805:Sepsis / Q000150:complications