Monday, January 18, 2016

Pediatric cardiac transplantation for Non-dilated cardiomyopathies

Linda J. Addonizio, Progress in Pediatric Cardiology, Available online 16 January 2016, ISSN 1058-9813, doi:10.1016/j.ppedcard.2016.01.006.

In data from the Pediatric Cardiomyopathy Registry, the idiopathic and familial types comprised 74% of the total cases of hypertrophic cardiomyopathy with inborn errors of metabolism comprising 9%(34% of these had Pompe disease), malformation syndromes 9% (78% had Noonan Syndrome) and neuromuscular disorders 8% (88% Friedreich ataxia). Children with inborn errors of metabolism and malformation syndromes have significantly worse outcomes than children with either idiopathic or neuromuscular disease as an etiology for their hypertrophic cardiomyopathy.


 Pediatric cardiac transplantation for Non-dilated cardiomyopathies