Congenital Heart Disease Studies

The Fontan EV Score: A Circulating Extracellular Vesicle-Based Risk Stratification Tool for Fontan-Associated Liver Disease

This paper develops the Fontan EV Score, a non-invasive biomarker using circulating extracellular vesicles to predict Fontan-associated liver disease severity, outperforming existing serological indices.

Altered Myocardial Structure in Post-natal Tetralogy of Fallot: A Substrate for Interpretation of Ventricular Function and Dysfunction?

This study uses HiP-CT to show altered myocardial structure in pediatric ToF hearts, revealing structural changes that align with clinical observations of ventricular dysfunction.

Serum Proteomic Profiling Implicates a Dysregulated Neurohormonal-Inflammatory Axis in Post-Fontan Tachycardia

This study uses serum proteomics and machine learning to identify a seven-protein panel predictive of post-Fontan tachycardia, implicating a dysregulated neurohormonal-inflammatory axis.

Optimized Biomechanical Design of a Pulsatile Fontan Conduit for Congenital Heart Palliation

This paper develops a biomechanical framework to optimize the design of a pulsatile Fontan conduit for congenital heart defect palliation, focusing on microstructure and material properties to achieve desired hemodynamic performance.

The Heterogeneous Nature of Atrioventricular Conduction Tissues in Tetralogy of Fallot demonstrated by Hierarchical Phase-contrast Tomography - redefining the anatomic substrate

This paper uses HiP-CT to analyze the atrioventricular conduction system in ToF specimens, revealing structural anomalies and surgical risks.

Pulmonary Vascular Endothelial Dysfunction is Induced by Non-Pulsatile Pulmonary Blood Flow in an Ovine Classic Glenn Model

This study shows that non-pulsatile pulmonary blood flow after a Glenn procedure causes endothelial dysfunction in lambs, independent of hypoxemia and polycythemia.

Reduced cerebrovascular reactivity in youth with complex congenital heart defects

This study finds persistent cerebrovascular reactivity deficits in youth with complex congenital heart defects compared to controls, with associations with cognitive performance.

Genome Sequencing is Critical for Forecasting Outcomes Following Congenital Cardiac Surgery

This study uses AI to show that genome sequencing can predict adverse outcomes after congenital heart defect surgery by identifying damaging genotypes in specific genes.

Pulsatile flow dynamics determine pulmonary arterial architecture

The paper investigates how loss of pulsatile flow affects pulmonary vascular structure in SV-CHD patients and identifies PDGFB as a key mediator of vascular remodeling.

Impact of Serum Circulating Factors and PDE5 Inhibitor Therapy on Cardiomyocyte Metabolism in Single Ventricle Heart Disease

This study investigates how serum factors and PDE5 inhibitor therapy affect cardiomyocyte metabolism in single ventricle heart disease, finding that PDE5 inhibitors improve mitochondrial function and metabolic intermediates.

Genetic, demographic and clinical variables act synergistically to impact neurodevelopmental outcomes in children with single ventricle heart disease

The paper examines how genetic, demographic, and clinical factors interact to influence neurodevelopmental outcomes in children with single ventricle heart disease.

Enhancing Neurocognitive Outcome Prediction in Congenital Heart Disease Patients: The Role of Brain Age Biomarkers and Beyond

This study shows that adding a brain age biomarker improves predictions of neurocognitive outcomes in CHD patients using demographic, socioeconomic, and genetic factors.

Computational Fontan Analysis: Preserving accuracy while expediting workflow

This study validates computational fluid dynamics simulations for Fontan hemodynamics using in vitro experiments, showing mesh size affects accuracy but not all metrics.

Experimental-based Computational Modeling Distinguishes Early Cardiac Outflow Tract Compensation Mechanisms

The paper combines experimental and computational modeling to study how abnormal hemodynamics affect cardiac outflow tract development and identify compensation mechanisms.

Abnormal microstructural development of the cerebral cortex in neonates with congenital heart disease is associated with impaired cerebral oxygen delivery

The paper investigates microstructural cortex development in neonates with congenital heart disease and finds impaired oxygen delivery correlates with abnormal microstructure.

Left planum temporale growth predicts language development in newborns with congenital heart disease

The paper investigates if brain growth rates in newborns with congenital heart disease correlate with language development at 12 months, finding a significant link between left planum temporale growth and language scores.

Metformin Intervention Prevents Cardiac Dysfunction in a Murine Model of Adult Congenital Heart Disease

This study shows that metformin prevents cardiac dysfunction in a mouse model of adult congenital heart disease under metabolic stress.