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Journal Article
End-stage cardiac failure caused by isolated ventricular non-compaction: cardiac transplantation in a 5-year-old boy.
Isolated ventricular non-compaction (IVNC) is a rare congenital cardiomyopathy characterized by arrest in endomyocardial morphogenesis. Presenting features in children include systolic and diastolic ventricular dysfunction, cardiac arrhythmias, and thromboembolism. End-stage cardiac failure necessitating cardiac transplantation is described in adults. We report the unique case of a 5-year-old boy with IVNC and end-stage heart failure who underwent cardiac transplantation. Diagnostic echocardiographic features, gross cardiac morphology, and histopathologic findings are illustrated.
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