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Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy.

  • Singh, Rakesh K1
  • Canter, Charles E2
  • Shi, Ling3
  • Colan, Steven D4
  • Dodd, Debra A5
  • Everitt, Melanie D6
  • Hsu, Daphne T7
  • Jefferies, John L8
  • Kantor, Paul F9
  • Pahl, Elfriede10
  • Rossano, Joseph W11
  • Towbin, Jeffrey A12
  • Wilkinson, James D13
  • Lipshultz, Steven E13
  • 1 Department of Pediatrics, University of California-San Diego and Rady Children's Hospital, San Diego, California. Electronic address: [email protected]
  • 2 Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri.
  • 3 New England Research Institutes, Watertown, Massachusetts.
  • 4 Department of Pediatrics, Boston's Children's Hospital, Boston, Massachusetts.
  • 5 Department of Pediatrics, Vanderbilt University and Monroe Carell Jr. Children's Hospital, Nashville, Tennessee.
  • 6 Department of Pediatrics, Children's Hospital Colorado and University of Colorado, Aurora, Colorado.
  • 7 Department of Pediatrics, Children's Hospital at Montefiore, Bronx, New York.
  • 8 Department of Pediatrics, The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
  • 9 Department of Pediatrics, Stollery Children's Hospital, Edmonton, Alberta, Canada. , (Canada)
  • 10 Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
  • 11 Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
  • 12 Department of Pediatrics, Le Bonheur Children's Hospital, Memphis, Tennessee.
  • 13 Department of Pediatrics, Wayne State University School of Medicine and Children's Hospital of Michigan, Detroit, Michigan.
Published Article
Journal of the American College of Cardiology
Publication Date
Nov 28, 2017
DOI: 10.1016/j.jacc.2017.09.1089
PMID: 29169474


Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation. This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death. Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death. Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64% vs. 63%, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16% vs. 17%) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95% confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death. Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391). Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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