Natural History of Dilated Cardiomyopathy in Children

Background: the long-term progression of idiopathic dilated cardiomyopathy (dcm) in pediatric patients compared with adult patients has not been previously characterized. in this study,we compared outcome and long-term progression of pediatric and adult dcm populations.methods and results: between 1988 and 2014,927 dcm patients were consecutively enrolled. the pediatric population (aged <18 years at enrollment) included 47 participants (5.1%). at presentation,the pediatric population compared with adult patients had a significantly increased occurrence of familial forms (p=0.03),shorter duration of heart failure (p=0.04),lower systolic blood pressure (p=0.01),decreased presence of left bundle-branch block (p=0.001),and increased left ventricular ejection fraction (p=0.03). despite these baseline differences,long-term longitudinal trends of new york heart association class iii to iv,left ventricular dimensions,left ventricular ejection fraction,and restrictive filling pattern were similar between the 2 populations. regarding survival analysis,because of the size difference between the 2 populations,we compared the pediatric population with a sample of adult patients randomly matched using the above-mentioned baseline differences in a 3:1 ratio (141 adult versus 47 pediatric patients). during a median follow-up of 110 months,survival free from heart transplantation was significantly lower among pediatric patients compared with adults (p<0.001). furthermore,pediatric age (ie,<18 years) was found to be associated with an increasing risk of both death from pump failure and life-threatening arrhythmias.conclusions: despite the pediatric dcm population having higher baseline left ventricular ejection fraction and similar long-term echocardiographic progression compared with the adult dcm population,the pediatric dcm patients had worse cardiovascular prognosis. © 2016 the authors. published on behalf of the american heart association,inc.,by wiley blackwell.