title: Predictors of disease progression in pediatric dilated cardiomyopathy.
abstract: BACKGROUND
  Despite medical advances, children with dilated cardiomyopathy (DCM) remain at high risk of death or need for cardiac transplantation. We sought to identify predictors of disease progression in pediatric DCM.
  
  
  RESULTS
  The Pediatric Heart Network evaluated chronic DCM patients with prospective echocardiographic and clinical data collection during an 18-month follow-up. Inclusion criteria were age <22 years and DCM disease duration >2 months. Patients requiring intravenous inotropic/mechanical support or listed status 1A/1B for transplant were excluded. Disease progression was defined as an increase in transplant listing status, hospitalization for heart failure, intravenous inotropes, mechanical support, or death. Predictors of disease progression were identified using Cox proportional hazards modeling and classification and regression tree analysis. Of the 127 patients, 28 (22%) had disease progression during the 18-month follow-up. Multivariable analysis identified older age at diagnosis (hazard ratio=1.14 per year; P<0.001), larger left ventricular (LV) end-diastolic M-mode dimension z-score (hazard ratio=1.49; P<0.001), and lower septal peak systolic tissue Doppler velocity z-score (hazard ratio=0.81; P=0.01) as independent predictors of disease progression. Classification and regression tree analysis stratified patients at risk of disease progression with 89% sensitivity and 94% specificity based on LV end-diastolic M-mode dimension z-score ≥7.7, LV ejection fraction <39%, LV inflow propagation velocity (color M-mode) z-score <-0.28, and age at diagnosis ≥8.5 months.
  
  
  CONCLUSIONS
  In children with chronic stable DCM, a combination of diagnosis after late infancy and echocardiographic parameters of larger LV size and systolic and diastolic function predicted disease progression.
  
  
  BACKGROUND
  URL: http://www.clinicaltrials.gov. Unique identifier: NCT00123071.
authors.affiliation: University of Utah School of Medicine, Salt Lake City.
authors.forename: Kimberly M
authors.initials: KM
authors.lastname: Molina
authors.forename: Peter
authors.initials: P
authors.lastname: Shrader
authors.forename: Steven D
authors.initials: SD
authors.lastname: Colan
authors.forename: Seema
authors.initials: S
authors.lastname: Mital
authors.forename: Renee
authors.initials: R
authors.lastname: Margossian
authors.forename: Lynn A
authors.initials: LA
authors.lastname: Sleeper
authors.forename: Girish
authors.initials: G
authors.lastname: Shirali
authors.forename: Piers
authors.initials: P
authors.lastname: Barker
authors.forename: Charles E
authors.initials: CE
authors.lastname: Canter
authors.forename: Karen
authors.initials: K
authors.lastname: Altmann
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Radojewski
authors.forename: Elif Seda Selamet
authors.initials: ES
authors.lastname: Tierney
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.forename: Lloyd Y
authors.initials: LY
authors.lastname: Tani
mesh_terms: D000293:Adolescent; D002311:Cardiomyopathy, Dilated / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D016027:Heart Transplantation; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D013997:Time Factors; D016277:Ventricular Function, Left / Q000502:physiology*
keywords: cardiomyopathies; heart transplantation; pediatrics