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Noncompaction cardiomyopathy in Hirschsprung's disease: a case report
Silvia D Visonà1, Gaetano Thiene2, Savina Mannarino3
1Department of Public Health, Experimental and Forensic Medicine, Unit of Legal Medicine and Forensic Sciences "Antonio Fornari", University of Pavia, Pavia, Italy.
Abstract:
Noncompaction cardiomyopathy is a rare disorder, often associated with cardiac and noncardiac malformations. Hirschsprung's disease, a well-known aganglionosis, is associated with congenital heart diseases and has been reported to be due to impairment migration and differentiation of neural crest cells. Here, we present an 8-month-old male infant who died for cardiogenic shock after surgical resection of the involved bowel segment. The child was affected by both noncompaction cardiomyopathy and Hirschsprung's disease, two entities which can share a common neural crest-derived etiology.
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