The overall focus of my current research program is to better understand the unique adaptations governing pathological cardiac remodeling and the progression to heart failure in pediatric patients with complex congenital heart disease (CHD), including hypoplastic left heart syndrome (HLHS) and other single ventricle defects. The ultimate goal of my research is to develop the critical knowledgebase and infrastructure necessary to identify efficacious therapies for improving outcomes in this vulnerable group.

Through our publications we have demonstrated that the heart of patients with single ventricle defects are typified by unique molecular, genetic, and functional changes when compared to the normal heart. By using human cardiac tissue and blood samples in combination with cell and animal models, we have evaluated both the basic biology of complex CHD and interventions that have the potential to address the decline in myocardial function. Currently, a major focus of the lab is to elucidate the molecular mechanisms involved in the modulation of cardiac energy metabolism in single ventricle heart disease. This work will be important for the identification and development of drug therapies that improve cardiac function and enhance transplant-free survival in this population. Additionally, we are actively investigating the use of peripheral blood mononuclear cells (PBMCs) as noninvasive molecular biomarkers in four distinct but complimentary capacities: 1) as a surrogate of the myocardial metabolic environment and presence of HF, 2) as a readout of inflammatory and immune remodeling, 3) for risk stratification and prediction of clinical outcomes, and 4) as a platform to evaluate therapeutic efficacy of novel drug targets to treat complex CHD pathology.