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.