Noncompaction cardiomyopathy in Hirschsprung's disease: a case report

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.

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