Outcome Predictors for Pediatric Dilated Cardiomyopathy: A Systematic Review

Abstract

Dilated cardiomyopathy comprises the largest group of pediatric cardiomyopathy functional types and is the most common indication for heart transplant in children over 5 years old. Prognostic factors for this condition have long been sought by many researchers. In a systematic review of these factors, we found 32 relevant articles published since 1976. Four studies report finding no predictive factors. In the remaining 28 studies, several factors indicating better prognosis stand out across multiple articles: younger age at diagnosis, higher left-ventricular fractional shortening and ejection fraction, and the presence of myocarditis. Results for other factors conflict across studies: severe mitral regurgitation, arrhythmias, and a family history of cardiomyopathy. Elevated left-ventricular end diastolic pressure was statistically significant in two studies, but it may be of limited utility as a result of its invasiveness. Although most children have congestive heart failure at presentation, only two studies found it to be a significant predictor of mortality. The largest study of this factor qualified the increased risk to 1 year after presentation. Other significant predictors that have not been analyzed or reported by more than one study group, are right ventricular failure and impaired cardiac adrenergic innervation, as detected by radiolabeled meta-iodobenzylguanidine imaging. Although 1- and 5-year survival rates have steadily improved, as more children with DCM receive cardiac transplants, event-free survival rates (the absence of "heart death" resulting in death or transplant) are similar to those from decades ago. A unified risk algorithm may assist in clinical decision-making but requires more studies. Other studies are needed to assess the post-transplant survival experience.