Natural History of Dilated Cardiomyopathy in Children

J Am Heart Assoc. 2016 Jun 30;5(7):e003450. doi: 10.1161/JAHA.116.003450.


Background: The long-term progression of idiopathic dilated cardiomyopathy (DCM) in pediatric patients compared with adult patients has not been previously characterized. In this study, we compared outcome and long-term progression of pediatric and adult DCM populations.

Methods and results: Between 1988 and 2014, 927 DCM patients were consecutively enrolled. The pediatric population (aged <18 years at enrollment) included 47 participants (5.1%). At presentation, the pediatric population compared with adult patients had a significantly increased occurrence of familial forms (P=0.03), shorter duration of heart failure (P=0.04), lower systolic blood pressure (P=0.01), decreased presence of left bundle-branch block (P=0.001), and increased left ventricular ejection fraction (P=0.03). Despite these baseline differences, long-term longitudinal trends of New York Heart Association class III to IV, left ventricular dimensions, left ventricular ejection fraction, and restrictive filling pattern were similar between the 2 populations. Regarding survival analysis, because of the size difference between the 2 populations, we compared the pediatric population with a sample of adult patients randomly matched using the above-mentioned baseline differences in a 3:1 ratio (141 adult versus 47 pediatric patients). During a median follow-up of 110 months, survival free from heart transplantation was significantly lower among pediatric patients compared with adults (P<0.001). Furthermore, pediatric age (ie, <18 years) was found to be associated with an increasing risk of both death from pump failure and life-threatening arrhythmias.

Conclusions: Despite the pediatric DCM population having higher baseline left ventricular ejection fraction and similar long-term echocardiographic progression compared with the adult DCM population, the pediatric DCM patients had worse cardiovascular prognosis.

Keywords: cardiomyopathy; death; dilated; echocardiography; heart failure; pediatrics; sudden.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Arrhythmias, Cardiac / epidemiology
  • Blood Pressure
  • Bundle-Branch Block / epidemiology
  • Cardiomyopathy, Dilated / epidemiology
  • Cardiomyopathy, Dilated / genetics
  • Cardiomyopathy, Dilated / physiopathology*
  • Cardiomyopathy, Dilated / therapy
  • Child
  • Death, Sudden, Cardiac / epidemiology
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Heart Failure / epidemiology*
  • Heart Transplantation*
  • Humans
  • Italy / epidemiology
  • Male
  • Middle Aged
  • Prognosis
  • Registries*
  • Stroke Volume
  • Time Factors

Supplementary concepts

  • Idiopathic dilation cardiomyopathy