Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy.

TitleSurvival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy.
Publication TypeJournal Article
Year of Publication2017
AuthorsSingh RK, Canter CE, Shi L, Colan SD, Dodd DA, Everitt MD, Hsu DT, Jefferies JL, Kantor PF, Pahl E, Rossano JW, Towbin JA, Wilkinson JD, Lipshultz SE
Corporate AuthorsPediatric Cardiomyopathy Registry Investigators
JournalJ Am Coll Cardiol
Date Published2017 Nov 28
KeywordsAdolescent, Aged, Cardiomyopathy, Dilated, Child, Child, Preschool, Cohort Studies, Female, Follow-Up Studies, Heart Transplantation, Heart Ventricles, Humans, Infant, Male, Middle Aged, Multivariate Analysis, Proportional Hazards Models, Time Factors, Treatment Outcome, Ventricular Remodeling

BACKGROUND: Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation.

OBJECTIVES: This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death.

METHODS: 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.

RESULTS: 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.

CONCLUSIONS: 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).

Alternate JournalJ. Am. Coll. Cardiol.
PubMed ID29169474