Ventricular remodeling and survival are more favorable for myocarditis than for idiopathic dilated cardiomyopathy in childhood: an outcomes study from the Pediatric Cardiomyopathy Registry.
BACKGROUND
Myocarditis is a cause of a new-onset dilated cardiomyopathy phenotype in children, with small studies reporting high rates of recovery of left ventricular (LV) function.


RESULTS
The presenting characteristics and outcomes of children with myocarditis diagnosed clinically and with biopsy confirmation (n=119) or with probable myocarditis diagnosed clinically or by biopsy alone (n=253) were compared with children with idiopathic dilated cardiomyopathy (n=1123). Characteristics at presentation were assessed as possible predictors of outcomes. The distributions of time to death, transplantation, and echocardiographic normalization in the biopsy-confirmed myocarditis and probable myocarditis groups did not differ (P≥0.5), but both groups differed significantly from the idiopathic dilated cardiomyopathy group (all P≤0.003). In children with myocarditis, lower LV fractional shortening z-score at presentation predicted greater mortality (hazard ratio, 0.85; 95% confidence interval, 0.73 to 0.98; P=0.03) and greater LV posterior wall thickness predicted transplantation (hazard ratio, 1.17; 95% confidence interval, 1.02 to 1.35; P=0.03). In those with decreased LV fractional shortening at presentation, independent predictors of echocardiographic normalization were presentation with an LV end-diastolic dimension z-score >2 (hazard ratio, 0.36; 95% confidence interval, 0.22 to 0.58; P<0.001) and greater septal wall thickness (hazard ratio, 1.16; 95% confidence interval, 1.01 to 1.34; P=0.04).


CONCLUSIONS
Children with biopsy-confirmed or probable myocarditis had similar proportions of death, transplantation, and echocardiographic normalization 3 years after presentation and better outcomes than those of children with idiopathic dilated cardiomyopathy. 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. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00005391.
Foerster
Susan R
SR
Washington University at St Louis, 1 Children’s Place, St Louis, MO 63110, USA. susanfoerster@msn.com
Canter
Charles E
CE
Cinar
Amy
A
Sleeper
Lynn A
LA
Webber
Steven A
SA
Pahl
Elfriede
E
Kantor
Paul F
PF
Alvarez
Jorge A
JA
Colan
Steven D
SD
Jefferies
John L
JL
Lamour
Jacqueline M
JM
Margossian
Renee
R
Messere
Jane E
JE
Rusconi
Paolo G
PG
Shaddy
Robert E
RE
Towbin
Jeffrey A
JA
Wilkinson
James D
JD
Lipshultz
Steven E
SE
D002311:Cardiomyopathy, Dilated / Q000000981:diagnostic imaging / Q000401:mortality* / Q000503:physiopathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004452:Echocardiography; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009205:Myocarditis / Q000000981:diagnostic imaging / Q000401:mortality* / Q000503:physiopathology* / Q000601:surgery; D012042:Registries; D012189:Retrospective Studies; D013318:Stroke Volume; D013534:Survival; D016896:Treatment Outcome; D016277:Ventricular Function, Left; D020257:Ventricular Remodeling