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Idiopathic restrictive cardiomyopathy in children

L M Russo1, S A Webber

  • 1Division of Cardiology, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, Pennsylvania 15213, USA. linda.russo@chp.edu

Heart (British Cardiac Society)
|August 17, 2005
PubMed

Insights

Pediatric idiopathic restrictive cardiomyopathy has a poor prognosis without transplantation. Lower cardiac filling pressures and left atrial to aortic root ratio at diagnosis correlate with longer survival in children with this condition.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Research
  • Restrictive Cardiomyopathy

Background:

  • Idiopathic restrictive cardiomyopathy (iRC) is a rare and severe condition in children.
  • Understanding its natural history and prognostic factors is crucial for management.

Purpose of the Study:

  • To define the natural history of iRC in a pediatric population.
  • To identify factors predictive of outcome in children diagnosed with iRC.

Main Methods:

  • Retrospective analysis of pediatric patients diagnosed with iRC between 1970 and 2002.
  • Evaluation of demographic data, clinical presentation, echocardiographic and hemodynamic findings, survival, and cause of death.

Main Results:

  • Survival probabilities at 1, 5, and 10 years were 80.5%, 39%, and 20%, respectively.
  • Median survival without transplantation was 2.2 years.
  • Lower right and left ventricular end-diastolic pressures and a lower left atrial to aortic root dimension ratio (LA:Ao) at diagnosis were significantly correlated with longer survival.

Conclusions:

  • Pediatric iRC carries a very poor prognosis without heart transplantation.
  • Cardiac filling pressures and LA:Ao ratio at diagnosis are important predictors of survival.
  • Symptoms at presentation did not influence survival time.
Abstract

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