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Pediatric and adult dilated cardiomyopathy represent distinct pathological entities

Meghna D Patel1, Jayaram Mohan2, Caralin Schneider2

  • 1Department of Pediatrics.

JCI Insight
|July 21, 2017
PubMed

Insights

Pediatric dilated cardiomyopathy (DCM) differs from adult DCM, showing less adverse remodeling. This explains why adult heart failure drugs don't work for children, highlighting the need for new pediatric therapies.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Molecular Biology

Background:

  • Pediatric dilated cardiomyopathy (DCM) is a leading cause for pediatric heart transplantation.
  • Current adult heart failure medications are ineffective in pediatric DCM, despite shared genetic causes.
  • The underlying reasons for this therapeutic disparity remain unclear.

Purpose of the Study:

  • To investigate the distinct pathological and molecular differences between pediatric and adult DCM.
  • To test the hypothesis that pediatric DCM lacks the adverse remodeling characteristic of adult DCM.
  • To provide a mechanistic explanation for the differential response to heart failure therapies.

Main Methods:

  • Analysis of left ventricular (LV) myocardial tissue from pediatric and adult DCM patients and controls.
  • Histopathological examination for cardiomyocyte hypertrophy and myocardial fibrosis.
  • RNA sequencing to compare gene expression profiles between pediatric and adult DCM.

Main Results:

  • Adult DCM showed significant cardiomyocyte hypertrophy and fibrosis, unlike controls.
  • Pediatric DCM exhibited minimal cardiomyocyte hypertrophy and fibrosis compared to controls and adult DCM.
  • RNA sequencing revealed distinct gene expression profiles, with adult DCM showing upregulation of adverse remodeling and immune response transcripts.

Conclusions:

  • Pediatric and adult DCM are distinct pathological entities.
  • The absence of adverse remodeling in pediatric DCM explains treatment failures with adult therapies.
  • New therapeutic strategies are required for pediatric DCM.

Keywords:
Cardiology

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