title: Recovery of echocardiographic function in children with idiopathic dilated cardiomyopathy: results from the pediatric cardiomyopathy registry.
abstract: OBJECTIVE
  This study sought to determine the incidence and predictors of recovery of normal echocardiographic function among children with idiopathic dilated cardiomyopathy (DCM).
  
  
  BACKGROUND
  Most children with idiopathic DCM have poor outcomes; however, some improve.
  
  
  METHODS
  We studied children <18 years of age from the Pediatric Cardiomyopathy Registry who had both depressed left ventricular (LV) function (fractional shortening or ejection fraction z-score <-2) and LV dilation (end-diastolic dimension [LVEDD] z-score >2) at diagnosis and who had at least 1 follow-up echocardiogram 30 days to 2 years from the initial echocardiogram. We estimated the cumulative incidence and predictors of normalization.
  
  
  RESULTS
  Among 868 children who met the inclusion criteria, 741 (85%) had both echocardiograms. At 2 years, 22% had recovered normal LV function and size; 51% had died or undergone heart transplantation (median, 3.2 months), and 27% had persistently abnormal echocardiograms. Younger age (hazard ratio [HR]: 0.92; 95% confidence interval [CI]: 0.88 to 0.97) and lower LVEDD z-score (HR: 0.78; 95% CI: 0.70 to 0.87) independently predicted normalization. Nine children (9%) with normal LV function and size within 2 years of diagnosis later underwent heart transplantation or died.
  
  
  CONCLUSIONS
  Despite marked LV dilation and depressed function initially, children with idiopathic DCM can recover normal LV size and function, particularly those younger and with less LV dilation at diagnosis. Investigations related to predictors of recovery, such as genetic associations, serum markers, and the impact of medical therapy or ventricular unloading with assist devices are important next steps. Longer follow-up after normalization is warranted as cardiac failure can recur. (Pediatric Cardiomyopathy Registry; NCT00005391).
authors.affiliation: Primary Children's Medical Center, Salt Lake City, Utah. Electronic address: Melanie.everitt@imail.org.
authors.forename: Melanie D
authors.initials: MD
authors.lastname: Everitt
authors.affiliation: New England Research Institutes, Inc., Watertown, Massachusetts.
authors.forename: Lynn A
authors.initials: LA
authors.lastname: Sleeper
authors.affiliation: New England Research Institutes, Inc., Watertown, Massachusetts.
authors.forename: Minmin
authors.initials: M
authors.lastname: Lu
authors.affiliation: Washington University, St. Louis, Missouri.
authors.forename: Charles E
authors.initials: CE
authors.lastname: Canter
authors.affiliation: Ann & Robert H. Lurie Children's Hospital, Chicago, Illinois.
authors.forename: Elfriede
authors.initials: E
authors.lastname: Pahl
authors.affiliation: Department of Pediatrics, Miller School of Medicine, University of Miami, Miami, Florida.
authors.forename: James D
authors.initials: JD
authors.lastname: Wilkinson
authors.affiliation: Columbia University, New York, New York.
authors.forename: Linda J
authors.initials: LJ
authors.lastname: Addonizio
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Towbin
authors.affiliation: University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Joseph
authors.initials: J
authors.lastname: Rossano
authors.affiliation: Children's Hospital at Montefiore, Bronx, New York.
authors.forename: Rakesh K
authors.initials: RK
authors.lastname: Singh
authors.affiliation: Children's Hospital at Montefiore, Bronx, New York.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Lamour
authors.affiliation: Vanderbilt University, Nashville, Tennessee.
authors.forename: Steven A
authors.initials: SA
authors.lastname: Webber
authors.affiliation: Boston Children's Hospital, Boston, Massachusetts.
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.affiliation: Boston Children's Hospital, Boston, Massachusetts.
authors.forename: Renee
authors.initials: R
authors.lastname: Margossian
authors.affiliation: University of Alberta, Stollery Children's Hospital, Edmonton, Alberta, Canada.
authors.forename: Paul F
authors.initials: PF
authors.lastname: Kantor
authors.affiliation: Children's Hospital at Montefiore, Bronx, New York.
authors.forename: John L
authors.initials: JL
authors.lastname: Jefferies
authors.affiliation: Columbia University, New York, New York.
authors.forename: Steven E
authors.initials: SE
authors.lastname: Lipshultz
mesh_terms: D000293:Adolescent; D017677:Age Distribution; D002311:Cardiomyopathy, Dilated / Q000151:congenital / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D020127:Recovery of Function; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D013318:Stroke Volume; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000401:mortality / Q000503:physiopathology; D020257:Ventricular Remodeling / Q000502:physiology*
keywords: cardiomyopathy; echocardiography; heart failure; pediatrics