Funding outcome

Congenital heart defects (CHD) are the most prevalent birth defects in Canada, affecting 1 in every 100 newborn babies. Despite progress in diagnosis and surgical interventions, the risk of developing chronic heart failure remains high in CHD patients. CHD occurs when the heart structures do not develop normally due to genetic defects. We have previously shown that children with CHD also have an altered energy metabolism profile. These metabolic changes impact the hearts' ability to produce sufficient energy for its pumping function, as well as its response to stress and growth signals. This study aims to identify new mechanisms contributing to adverse metabolic changes in newborn hearts with CHD. Fetal hearts metabolize glucose via glycolysis rather than fat, which produces more energy. After birth, the heart's fuel preference shifts from glucose to fat to fulfill the increasing energy demand. However, this transition is impaired in infants with CHD. As a result, the heart produces less energy despite the increased demand. The current study aims to understand the mechanisms that disrupt the energy metabolism maturation resulting in a maintained fetal-like metabolic phenotype in newborn hearts with CHD. We will use both surgical heart tissues obtained from infants undergoing heart surgery due to CHD, as well as cardiac cells under fetal-like and newborn conditions. We will investigate the modifications of glucose metabolism enzymes and determine whether these alterations contribute to impaired energy metabolism and adverse remodelling in newborn hearts with CHD. This will help to develop new cardioprotective strategies to improve health outcomes in children with CHD.