title: Noncompaction cardiomyopathy in Hirschsprung's disease: a case report.
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.
authors.affiliation: Department of Public Health, Experimental and Forensic Medicine, Unit of Legal Medicine and Forensic Sciences "Antonio Fornari", University of Pavia, Pavia, Italy.
authors.forename: Silvia D
authors.initials: SD
authors.lastname: Visonà
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padova, Italy.
authors.forename: Gaetano
authors.initials: G
authors.lastname: Thiene
authors.affiliation: Department of Pediatrics, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
authors.forename: Savina
authors.initials: S
authors.lastname: Mannarino
authors.affiliation: Department of Pediatrics, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Corana
authors.affiliation: Department of Public Health, Experimental and Forensic Medicine, Unit of Legal Medicine and Forensic Sciences "Antonio Fornari", University of Pavia, Pavia, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Osculati
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padova, Italy. Electronic address: annalisa.angelini@unipd.it.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Angelini
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padova, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Rizzo
mesh_terms: D009202:Cardiomyopathies / Q000150:complications* / Q000235:genetics / Q000473:pathology; D017809:Fatal Outcome; D006627:Hirschsprung Disease / Q000150:complications* / Q000235:genetics / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male
keywords: Hirschsprung's disease; Neural crest; Noncompaction cardiomyopathy