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

被引：7

作者：

Garcia, Anastacia M. ^{[1
]}

Toni, Lee S. ^{[2
]}

Miyano, Carissa A. ^{[2
]}

Sparagna, Genevieve C. ^{[2
]}

Jonscher, Raleigh ^{[2
]}

Phillips, Elisabeth K. ^{[2
]}

Karimpour-Fard, Anis ^{[2
,3
]}

Chapman, Hailey L. ^{[1
]}

Baybayon-Grandgeorge, Angela N. ^{[2
]}

Pietra, Ashley E. ^{[1
]}

Selner, Emma ^{[1
]}

Chatfield, Kathryn C. ^{[1
]}

Stauffer, Brian L. ^{[2
,4
]}

Sucharov, Carmen C. ^{[2
,6
]}

Miyamoto, Shelley D. ^{[1
,5
]}

机构：

[1] Univ Colorado Anschutz Med Campus, Childrens Hosp Colorado, Dept Pediat, Div Cardiol, Aurora, CO USA

[2] Univ Colorado Anschutz Med Campus, Dept Med, Div Cardiol, Aurora, CO USA

[3] Univ Colorado Anschutz Med Campus, Dept Pharmacol, Aurora, CO USA

[4] Denver Hlth & Hosp Author, Dept Med, Div Cardiol, Denver, CO USA

[5] Univ Colorado Anschutz Med Campus, Childrens Hosp Colorado, Dept Pediat, Div Cardiol, 13123 East 16th Ave,B100, Aurora, CO 80045 USA

[6] Univ Colorado Anschutz Med Campus, Dept Med, Div Cardiol, 12700 East 19th Ave,B139, Aurora, CO 80045 USA

来源：

JACC-BASIC TO TRANSLATIONAL SCIENCE | 2023年 / 8卷 / 03期

基金：

美国国家卫生研究院;

关键词：

cardiac metabolism; cardiometabolic remodeling; heart failure; hypoplastic left heart syndrome; mitochondria; oxidative phosphorylation; single ventricle; AMINO-ACIDS; FAILURE; CARDIOLIPIN; MORPHOLOGY; OUTCOMES; IMPACT; TRANSPOSITION; PALLIATION; PREDICTORS; METABOLISM;

D O I：

10.1016/j.jacbts.2022.09.013

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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. (J Am Coll Cardiol Basic Trans Science 2023;8:258-279) (c) 2023 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

引用

页码：258 / 279

页数：22

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