title: Long-term outcomes of dilated cardiomyopathy diagnosed during childhood: results from a national population-based study of childhood cardiomyopathy.
abstract: BACKGROUND
  Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy.
  
  
  RESULTS
  The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings. The primary study end point included time to the combined outcome of death or cardiac transplantation. There were 175 patients 0 to <10 years of age at the time of diagnosis. Survival free from death or transplantation was 74% (95% confidence interval, 67-80) 1 year after diagnosis, 62% (95% confidence interval, 55-69) at 10 years, and 56% (95% confidence interval, 46-65) at 20 years. In multivariable analysis, age at diagnosis <4 weeks or >5 years, familial cardiomyopathy, and lower baseline left ventricular fractional shortening Z score were associated with increased risk of death or transplantation, as was lower left ventricular fractional shortening Z score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline left ventricular fractional shortening Z score, higher left ventricular fractional shortening Z score during follow-up, and greater improvement in left ventricular fractional shortening Z score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At the latest follow-up, 100 of 104 of survivors (96%) were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy.
  
  
  CONCLUSIONS
  Death or transplantation occurred in 26% of patients with childhood dilated cardiomyopathy within 1 year of diagnosis and ~1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy, and severity of left ventricular dysfunction. The majority of surviving subjects are well and free of cardiac medication.
authors.affiliation: From The Royal Children's Hospital, Melbourne, Victoria, Australia (P.M.A.A., A.M.D., R.G.W.); Murdoch Children's Research Institute, Melbourne, Victoria, Australia (P.M.A.A., K.J.L., A.M.D., I.K., R.G.W.); Royal Brompton Hospital and the National Heart and Lung Institute, Imperial College, London, UK (P.E.F.D.); University of Texas Southwestern Medical Center, Dallas (A.W.N.); University of Melbourne, Melbourne, Victoria, Australia (K.J.L.); Children's Hospital at Westmead, Sydney, New South Wales, Australia (C.T., G.F.S.); Boston Children's Hospital and Harvard Medical School, Boston, MA (S.D.C.); Women's and Children's Hospital, Adelaide, South Australia, Australia (T.R.); Princess Margaret Hospital, Perth, Western Australia, Australia (J.R.); and University of Queensland, Brisbane, Queensland, Australia (R.J.).
authors.forename: Peta M A
authors.initials: PM
authors.lastname: Alexander
authors.forename: Piers E F
authors.initials: PE
authors.lastname: Daubeney
authors.forename: Alan W
authors.initials: AW
authors.lastname: Nugent
authors.forename: Katherine J
authors.initials: KJ
authors.lastname: Lee
authors.forename: Christian
authors.initials: C
authors.lastname: Turner
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.forename: Terry
authors.initials: T
authors.lastname: Robertson
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Davis
authors.forename: James
authors.initials: J
authors.lastname: Ramsay
authors.forename: Robert
authors.initials: R
authors.lastname: Justo
authors.forename: Gary F
authors.initials: GF
authors.lastname: Sholler
authors.forename: Ingrid
authors.initials: I
authors.lastname: King
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
mesh_terms: D001315:Australia / Q000453:epidemiology; D002311:Cardiomyopathy, Dilated / Q000188:drug therapy / Q000401:mortality* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011237:Predictive Value of Tests; D012307:Risk Factors; D016019:Survival Analysis; D018487:Ventricular Dysfunction, Left / Q000188:drug therapy / Q000401:mortality* / Q000601:surgery*; D020257:Ventricular Remodeling / Q000502:physiology
keywords: cardiomyopathies; follow-up studies; mortality; pediatrics; ventricular remodeling