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Isolation of Endocardial and Coronary Endothelial Cells from the Ventricular Free Wall of the Rat Heart
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Cardiac Transcriptome Remodeling and Impaired Bioenergetics in Single-Ventricle Congenital Heart Disease

Anastacia M Garcia1, Lee S Toni2, Carissa A Miyano2

  • 1Division of Cardiology, Department of Pediatrics, University of Colorado Anschutz Medical Campus, Children's Hospital Colorado, Aurora, Colorado, USA.

JACC. Basic to Translational Science
|April 10, 2023
PubMed

Insights

Heart failure in single-ventricle disease involves metabolic and mitochondrial dysfunction. Nonfailing hearts show vulnerability, suggesting targeted energy generation therapies could prevent future heart failure.

Area of Science:

  • Cardiology
  • Biochemistry
  • Mitochondrial Biology

Background:

  • Single-ventricle congenital heart disease (SV-CHD) is a complex condition.
  • The mechanisms leading to heart failure in SV-CHD remain poorly understood.
  • Understanding metabolic alterations is crucial for developing effective treatments.

Purpose of the Study:

  • To investigate the metabolic and mitochondrial mechanisms underlying heart failure in SV-CHD.
  • To identify potential therapeutic targets for preventing or treating heart failure in this patient population.

Main Methods:

  • Analysis of explanted heart tissue from patients with SV-CHD.
  • Assessment of metabolic pathways, including the tricarboxylic acid cycle.
  • Evaluation of mitochondrial function and carnitine palmitoyltransferase activity.

Main Results:

  • Failing single-ventricle hearts exhibit dysregulated metabolic pathways.
  • Impaired mitochondrial function and decreased carnitine palmitoyltransferase activity were observed.
  • Nonfailing single-ventricle hearts showed an intermediate metabolic phenotype, indicating future vulnerability.

Conclusions:

  • Metabolic and mitochondrial dysfunction are key contributors to heart failure in SV-CHD.
  • Nonfailing hearts in SV-CHD are metabolically vulnerable.
  • Mitochondrial-targeted therapies aimed at normalizing energy generation offer a novel treatment strategy.

Keywords:
cardiac metabolismcardiometabolic remodelingheart failurehypoplastic left heart syndromemitochondriaoxidative phosphorylationsingle ventricle

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