Neonatal Cardiac Dilation and Dysfunction: Time to Look in the Genes.

Cardiomyopathy is a disease of the myocardium that can present with ventricular hypertrophy, dilation, or dysfunction and subsequently result in clinical heart failure. Dilated cardiomyopathy is the most common phenotype; however, it can be difficult to differentiate from myocarditis, particularly in neonates. Important causes of ventricular dysfunction in the pediatric population range from primary cardiomyopathies that affect the structure or function of the myocardium to systemic diseases that lead to secondary myocardial injury.

Methylprednisolone for Infant Heart Surgery: Subpopulation Analyses of a Randomized Controlled Trial.

Objectives: Evaluate benefits and harms of prophylactic intraoperative methylprednisolone in subpopulations undergoing infant heart surgery.

Design: Subpopulation analyses of The Steroids to Reduce Systemic Inflammation after Infant Heart Surgery (STRESS) trial, a double-blind randomized placebo-controlled trial.

Setting: Twenty-four congenital heart centers.

Reexploring the STRESS Trial: Subgroup Postoperative Outcomes Following Methylprednisolone for Infant Heart Surgery.

Unlabelled: Objective Assess the association between intraoperative methylprednisolone and specific postoperative outcomes among subgroups undergoing infant heart surgery.

Design: Subpopulation analyses of The Steroids to Reduce Systemic Inflammation after Infant Heart Surgery trial, a double-blind randomized placebo-controlled trial.

Setting: 24 congenital heart centers.

Pulmonary Veins: Not Always Where You Expect Them.

• Multimodality imaging is important in patients with ACHD and challenging anatomy. • Complex congenital anatomy and physiology requires multidisciplinary discussions. • Image-guided percutaneous intervention may be warranted after TAPVR repair.

Assessment of the CLOT (children's likelihood of thrombosis) real-time risk prediction model of hospital-associated venous thromboembolism in children with congenital heart disease.

Background: Children with congenital heart disease (CHD) are at high risk for hospital-associated venous thromboembolism (HA-VTE). The children's likelihood of thrombosis (CLOT) trial validated a real-time predictive model for HA-VTE using data extracted from the EHR for pediatric inpatients. We tested the hypothesis that addition of CHD specific data would improve model prediction in the CHD population.

Comprehensive cardiac magnetic resonance T1, T2, and extracellular volume mapping to define Duchenne cardiomyopathy.

Background: Cardiomyopathy is the leading cause of death in Duchenne muscular dystrophy (DMD). Cardiac magnetic resonance (CMR) parametric mapping sequences offer insights into disease pathophysiology. We propose a novel approach by leveraging T2 mapping in conjunction with T1 and extracellular volume (ECV) mapping to perform a virtual myocardial biopsy.

Continuous Murmur in a Child: Sometimes It Is a Zebra.

• Most AVFs present with a continuous murmur with radiation to the back. • There is little evidence to guide management of thoracic AVF. • Management options include surgical repair, embolization, or conservative management.

Association of cardiovascular magnetic resonance diastolic indices with arrhythmia in repaired Tetralogy of Fallot.

Background: Patients with repaired Tetralogy of Fallot (rTOF) experience a high burden of long-term morbidity, particularly arrhythmias. Cardiovascular magnetic resonance (CMR) is routinely used to assess ventricular characteristics but the relationship between CMR diastolic function and arrhythmia has not been evaluated. We hypothesized in rTOF, left ventricular (LV) diastolic dysfunction on CMR would correlate with arrhythmias and mortality.

Machine Learning to Predict Interstage Mortality Following Single Ventricle Palliation: A NPC-QIC Database Analysis.

There is high risk of mortality between stage I and stage II palliation of single ventricle heart disease. This study aimed to leverage advanced machine learning algorithms to optimize risk-prediction models and identify features most predictive of interstage mortality. This study utilized retrospective data from the National Pediatric Cardiology Quality Improvement Collaborative and included all patients who underwent stage I palliation and survived to hospital discharge (2008-2019).

Influence of CYP2D6 genetic variation on adverse events with propafenone in the pediatric and young adult population.

Propafenone is an antiarrhythmic drug metabolized primarily by cytochrome P450 2D6 (CYP2D6). In adults, propafenone adverse events (AEs) are associated with CYP2D6 poor metabolizer status; however, pediatric data are lacking. Subjects were tested for 10 CYP2D6 allelic variants and copy number status, and activity scores assigned to each genotype.