Natural History of Dilated Cardiomyopathy in Children

Abstract

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.