Ventricular remodeling after Myocarditis vs. Dilated Cardiomyopathy in Children

Circulatation: Heart Failure 2010

Published online Sep 10, 2010

Ventricular Remodeling and Survival are More Favorable for Myocarditis Than For Idiopathic Dilated Cardiomyopathy in Childhood: An Outcomes Study from the Pediatric Cardiomyopathy Registry

Susan R. Foerster; Charles E. Canter; Amy Cinar; Lynn A. Sleeper; Steven A. Webber; Elfriede Pahl;Paul F. Kantor; Jorge A. Alvarez; Steven D. Colan; John L. Jefferies; Jacqueline M. Lamour;Renee Margossian; Jane E. Messere;Paolo G. Rusconi; Robert E. Shaddy; Jeffrey A. Towbin;James D. Wilkinson and Steven E. Lipshultz

Background—Myocarditis is one cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function.

Methods and Results—The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (BCM, n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (PM, n=253) were compared to children with idiopathic dilated cardiomyopathy (IDCM, n=1123). Characteristics at presentation were assessed as possible predictors of outcomes. The distributions of time to death, transplantation, and echocardiographic normalization in the BCM and PM groups did not differ (P0.5), but both groups differed significantly from the IDCM group (all P0.003). In children with myocarditis,lower LVFS z-score at presentation predicted greater mortality (hazard ratio [HR]=0.85, 95% CI 0.73-0.98, P=0.03) and greater LV posterior wall thickness predicted transplantation (HR=1.17, 95% CI 1.02-1.35, P=0.03). In those with decreased LVFS at presentation, independent predictors of echocardiographic normalization were presentation with an LVEDD z-score >2 (HR=0.36, 95% CI 0.22-0.58, P <0.001)> (HR=1.16, 95% CI 1.01-1.34, P=0.04).

Conclusions—Children with biopsy-confirmed or probable myocarditis had similar proportions of death, transplant, and echocardiographic normalization 3 years after presentation and better outcomes than those of children with IDCM. In children with myocarditis who had impaired LV ejection at presentation, rates of echocardiographic normalization were greater in those without LV dilation and in those with greater septal wall thickness at presentation.