Cardiac Transcriptome Remodeling And Impaired Bioenergetics in Single-Ventricle Congenital Heart Disease

Overview

Journal JACC Basic Transl Sci

Date 2023 Apr 10

PMID 37034285

Authors

Anastacia M Garcia

Lee S Toni

Carissa A Miyano

Genevieve C Sparagna

Raleigh Jonscher

Elisabeth K Phillips

Anis Karimpour-Fard

Hailey L Chapman

Angela N Baybayon-Grandgeorge

Ashley E Pietra

Emma Selner

Kathryn C Chatfield

Brian L Stauffer

Carmen C Sucharov

Shelley D Miyamoto

Affiliations

Soon will be listed here.

Abstract

The mechanisms responsible for heart failure in single-ventricle congenital heart disease are unknown. Using explanted heart tissue, we showed that failing single-ventricle hearts have dysregulated metabolic pathways, impaired mitochondrial function, decreased activity of carnitine palmitoyltransferase activity, and altered functioning of the tricarboxylic acid cycle. Interestingly, nonfailing single-ventricle hearts demonstrated an intermediate metabolic phenotype suggesting that they are vulnerable to development of heart failure in the future. Mitochondrial targeted therapies and treatments aimed at normalizing energy generation could represent a novel approach to the treatment or prevention of heart failure in this vulnerable group of patients.

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