Non-compaction cardiomyopathy in Hirschsprung's disease: a case report

Non-compaction cardiomyopathy is a rare disorder, often associated with cardiac and non-cardiac malformations. Hirschsprung's disease, a well-known aganglionosis, is associated to congenital heart diseases and has been reported to be due to impairment migration and differentiation of neural crest cells. Here, we present a 8-month-old young boy, who died for cardiogenic shock after surgical resection of the involved bowel segment. The child was affected by both non-compaction cardiomyopathy and Hirschsprung's disease, two entities which can share a common neural crest-derived etiology.

Source: Cardiovascular Pathology - January 2, 2017 Category: Cardiology Authors: Silvia D. Vison à, Gaetano Thiene, Savina Mannarino, Giulia Corana, Antonio Osculati, Annalisa Angelini, Stefania Rizzo Source Type: research