Utilisation of a post-Fontan management pathway reduces chest tube drainage and hospital readmission rates.

Background: Complications following the Fontan procedure include prolonged pleural drainage and readmission for effusions. To address these complications, a post-Fontan management pathway was implemented with primary goals of reducing chest tube duration/reinsertion rates and decreasing hospital length of stay and readmissions.

Methods: Fontan patients were identified by retrospective chart review (2017-2019) to obtain baseline data for chest tube duration/reinsertion rates, hospital length of stay, and readmission rates for effusion.

Postoperative Morbidity and Interstage Hemodynamics Following Stage I Palliation in Patients with Turner Syndrome and Hypoplastic Left Heart Syndrome.

Background: Turner syndrome (TS) is associated with left-sided cardiac lesions, including hypoplastic left heart syndrome (HLHS). Mortality as high as 80-90% has been reported following stage I single-ventricle palliation (S1P) in patients with TS and HLHS (TS + HLHS). The specific factors that relate to poor outcomes are not well understood.

Cardiac magnetic resonance assessment of acute rejection and cardiac allograft vasculopathy in pediatric heart transplant.

Background: In pediatric heart transplant (PHT), cardiac catheterization with endomyocardial biopsy (EMB) is standard for diagnosing acute rejection (AR) and cardiac allograft vasculopathy (CAV) but is costly and invasive.

Objectives: To evaluate the ability of cardiac magnetic resonance (CMR) to noninvasively identify differences in PHT patients with AR and CAV.

Methods: Patients were enrolled at three children's hospitals.

Reference data for left ventricular filling and atrial function in children using cardiovascular magnetic resonance.

Background: Diastolic dysfunction is associated with morbidity and mortality in multiple pediatric disease processes. Cardiovascular magnetic resonance (CMR) provides a non-invasive method of studying left ventricular (LV) diastolic dysfunction through the assessment of LV filling curves and left atrial (LA) volume and function. However, there are no normative data for LV filling curves and the standard method is time-intensive.

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.

Precision Medicine in Pediatric Cardiology.

Precision medicine is a developing strategy for individualized treatment of a wide range of diseases. Congenital heart disease is the most common of all congenital defects and carries a high degree of variability in outcomes because of unidentified causes. Advances have identified individual genetic and environmental factors that have helped understand variations in morbidity and mortality in pediatric cardiology.

Association between nitric oxide synthase 3 genetic variant and acute kidney injury following pediatric cardiac surgery.

Background: Acute kidney injury (AKI) complicates 30% to 50% of cardiac surgeries in pediatric patients. Genetic variants that affect renal blood flow and inflammation have been associated with AKI after cardiac surgery in diverse populations of adults but have not been studied in children. The objective of this study is to test the hypothesis that common candidate genetic variants are associated with AKI following pediatric cardiac surgery.

Transient fetal atrioventricular block: A series of four cases and approach to management.

Introduction: Fetal atrioventricular block (AVB) is a failure of conduction from atria to ventricles. Immune- and nonimmune-mediated forms occur, especially in association with congenital heart disease. Second-degree (2°) AVB may be reversible with dexamethasone and intravenous immunoglobulin in immune-mediated disease.

The Association of Fetal Congenital Cardiac Defects and Placental Vascular Malperfusion.

Objectives: Abnormal early angiogenesis appears to impact both placental disorders and fetal congenital heart defects (CHD). We sought to assess the association of placental perfusion defects (PPD) and fetal (CHD).

Methods: Singleton pregnancies with isolated severe fetal CHD were compared to controls without congenital anomalies or maternal malperfusion (MVM) risk factors.

Appropriate use of echocardiography for palpitations in paediatric cardiology clinics.

Objectives: Identify diagnostic yield and frequency of echocardiograms for palpitation-related indications at outpatient paediatric cardiology clinics in relation to the 2014 ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE appropriate use criteria for Initial Transthoracic Echocardiography in Outpatient Paediatric Cardiology.

Study Design: A single-centre, retrospective study of children presenting for evaluation of a chief complaint of palpitations to outpatient paediatric cardiology clinics from 2015 to 2017. Palpitations were defined as an unpleasant sensation of rapid, irregular, and/or forceful beating of the heart.

Serial block face-scanning electron microscopy: a method to study retinal degenerative phenotypes.

Retinal degenerative conditions can vary in their clinical features and often present with subtle phenotypic features before the onset of clinically overt disease. To capture these isolated events that precipitate disease, large representative areas of the retina must be imaged at high resolution. Compared to light microscopic methods, traditional electron microscopy can provide images at sufficient resolution to detect subtle pathologic changes in the retina, but are limited to the area being surveyed.

Sequencing from dried blood spots in infants with "false positive" newborn screen for MCAD deficiency.

Background: Newborn screening (NBS) for medium chain acyl-CoA dehydrogenase deficiency (MCADD), one of the most common disorders identified, uses measurement of octanoylcarnitine (C8) from dried blood spots. In the state of Ohio, as in many places, primary care providers, with or without consultation from a metabolic specialist, may perform "confirmatory testing", with the final diagnostic decision returned to the state. Confirmatory testing may involve measurement of metabolites, enzyme analysis, mutation screening, or sequencing.