Coronavirus Disease 2019 (COVID-19) Pandemic Implications in Pediatric and Adult Congenital Heart Disease.

The corona virus disease -2019 (COVID-19) is a recently described infectious disease caused by the severe acute respiratory syndrome corona virus 2 with significant cardiovascular implications. Given the increased risk for severe COVID-19 observed in adults with underlying cardiac involvement, there is concern that patients with pediatric and congenital heart disease (CHD) may likewise be at increased risk for severe infection. The cardiac manifestations of COVID-19 include myocarditis, arrhythmia and myocardial infarction.

Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality.

The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation (AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology.

Red Blood Cell Transfusion After Stage I Palliation Is Associated With Worse Clinical Outcomes.

Background Packed red blood cell transfusion may improve oxygen content in single-ventricle neonates, but its effect on clinical outcomes after Stage 1 palliation is unknown. Methods and Results Retrospective multicenter analysis of packed red blood cell transfusion exposures in neonates after Stage 1 palliation, excluding those with intraoperative mortality or need for extracorporeal membrane oxygenation. Transfusion practice variability was assessed, and multivariable regression used to identify transfusion risk factors.

Prevalence and Prognostic Association of a Clinical Diagnosis of Depression in Adult Congenital Heart Disease: Results of the Boston Adult Congenital Heart Disease Biobank.

Background In adults with acquired heart disease, depression is common and associated with adverse outcomes. Depression may also be important in adults with congenital heart disease (CHD). Methods and Results We conducted a cohort study of outpatients with CHD, aged ≥18 years, enrolled in a prospective biobank between 2012 and 2017.

Chronic Disease Burden After Congenital Heart Surgery: A 47-Year Population-Based Study With 99% Follow-Up.

Background Postoperative morbidity is an increasingly important outcome measure of patients who have undergone congenital heart surgery (CHS). We examined late postoperative morbidity after CHS on the basis of patients' government-issued medical special reimbursement rights. Methods and Results Between 1953 and 2009, 10 635 patients underwent CHS at <15 years of age in Finland.

The Variability of Preferred Infant Lumbar Puncture Insertion Site Between Novice and Experienced Physicians.

Background: We sought to determine if vertebral interspace selection for performance of infant lumbar puncture (LP) varies between less experienced trainees and more experienced pediatric emergency medicine (PEM) attending physicians.

Methods: We performed an observational prospective study using a convenience sample of infants aged 0 to 12 months presenting to a single emergency department. Trainees with limited LP experience (defined as less than 10 infant LPs performed) marked their preferred LP insertion site with an invisible ultraviolet pen.

Application of Frequent, Spaced Multiple-choice Questions as an Educational Tool in the Pediatric Emergency Department.

Objectives: The objective was to assess the feasibility of using spaced multiple-choice questions (MCQs) to teach residents during their pediatric emergency department (PED) rotation and determine whether this teaching improves knowledge retention about pediatric rashes.

Methods: Residents rotating in the PED from four sites were randomized to four groups: pretest and intervention, pretest and no intervention, no pretest and intervention, and no pretest and no intervention. Residents in intervention groups were automatically e-mailed quizlets with two MCQs every other day over 4 weeks (20 questions total) via an automated e-mail service with answers e-mailed 2 days later.

Value of Troponin Testing for Detection of Heart Disease in Previously Healthy Children.

Background Troponin levels are frequently obtained in pediatric patients, but the benefit remains unclear. Methods and Results This retrospective study included 1993 patients aged 0 to 21 years without history of cardiac disease in whom troponin levels were obtained during clinical evaluation of cardiac and noncardiac presentations. Troponin was elevated (≥0.

Receipt of American Heart Association-Recommended Preconception Health Care Among Privately Insured Women With Congenital Heart Defects, 2007-2013.

Background Our objective was to estimate receipt of preconception health care among women with congenital heart defects (CHD), according to 2017 American Heart Association recommendations, as a baseline for evaluating recommendation implementation. Methods and Results Using 2007 to 2013 IBM MarketScan Commercial Databases, we identified women with CHD diagnosis codes ages 15 to 44 years who became pregnant and were enrolled in health insurance for ≥11 months in the year before estimated conception. We assessed documentation of complete blood count, electrolytes, thyroid-stimulating hormone, liver function, ECG, comprehensive echocardiogram, and exercise stress test, using procedural codes, and outpatient prescription claims for US Food and Drug Administration category D and X cardiac-related medications.

Longitudinal Evaluation of Pain, Flare-Up, and Emotional Health in Fibrodysplasia Ossificans Progressiva: Analyses of the International FOP Registry.

Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare, inherited, connective tissue disease with ∼800 documented cases worldwide. The principal pathological feature of FOP is the transition of skeletal muscle, tendons, ligaments, and fascia into cartilage and bone. This heterotopic ossification (HO) is often preceded by painful soft tissue swellings or flare-ups that may last several months.

Risk Model Development and Validation for Prediction of Coronary Artery Aneurysms in Kawasaki Disease in a North American Population.

Background Accurate prediction of coronary artery aneurysms ( CAAs ) in patients with Kawasaki disease remains challenging in North American cohorts. We sought to develop and validate a risk model for CAA prediction. Methods and Results A binary outcome of CAA was defined as left anterior descending or right coronary artery Z score ≥2.

Will Teens Go Red? Low Cardiovascular Disease Awareness Among Young Women.

Background The American Heart Association Go Red for Women campaign has improved awareness of cardiovascular disease ( CVD ) among adult women aged 25 years and older. Little is known about awareness among younger women. Methods and Results We assessed awareness of CVD and prevention efforts among 331 young women aged 15 to 24 years using the American Heart Association National Women's Health Study survey.

Ascending Aorta Size at Birth Predicts White Matter Microstructure in Adolescents Who Underwent Fontan Palliation.

Background In neonates with single ventricle, smaller ascending aorta diameter is associated with cerebral white matter ( WM ) microstructural abnormalities. We sought to determine whether this association persists into adolescence. Methods and Results Ascending aorta Z scores were obtained from first postnatal echocardiogram.

Pediatric Case Exposure During Emergency Medicine Residency.

Objective: While emergency medicine (EM) physicians treat the majority of pediatric EM (PEM) patients in the United States, little is known about their PEM experience during training. The primary objective was to characterize the pediatric case exposure and compare to established EM residency training curricula among EM residents across five U.S.

A Hazardous Materials Educational Curriculum Improves Pediatric Emergency Department Staff Skills.

Objective: Emergency department (ED) providers require competency in responding to hazardous materials (HAZMAT) events. The optimal strategies to teach HAZMAT response principles to ED providers and to ensure skill retention are not known. Our aim was to design, implement, and evaluate a multifaceted, interprofessional educational curriculum for pediatric ED staff to improve their skills, knowledge, and confidence in responding to a HAZMAT event.

Does Point-of-care Ultrasound Affect Patient and Caregiver Satisfaction for Children Presenting to the Pediatric Emergency Department?

Objectives: Point-of-care ultrasound (POCUS) may facilitate the diagnosis and management of children for various conditions. Integration of POCUS into clinical care requires hands-on training; however, providers may be reluctant to perform educational ultrasounds to improve their skills, as it is a procedure without direct clinical benefit to the patient and due to concerns that it may lower a family's overall satisfaction. We sought to evaluate whether the use of POCUS changed overall patient/caregiver satisfaction in a pediatric emergency department (ED) visit.

Focusing in on use of pharmacokinetic profiles in routine hemophilia care.

Background: Emergence of population pharmacokinetic models for prediction of individual pharmacokinetic (PK) profiles facilitates individualization of prescribed prophylactic therapy for patients with hemophilia A and B and may have a favorable impact on clinical outcomes and annual factor utilization. How providers approach the integration and application of these data into routine clinical practice is not clear.

Objective: To explore the potential application of and barriers to incorporating PK profiles into current hemophilia prophylaxis decision making.

Performing and interpreting individual pharmacokinetic profiles in patients with Hemophilia A or B: Rationale and general considerations.

Objectives: In a separate document, we have provided specific guidance on performing individual pharmacokinetic (PK) studies using limited samples in persons with hemophilia with the goal to optimize prophylaxis with clotting factor concentrates. This paper, intended for clinicians, aims to describe how to interpret and apply PK properties obtained in persons with hemophilia.

Methods: The members of the Working Party on population PK (PopPK) of the ISTH SSC Subcommittee on Factor VIII and IX and rare bleeding disorders, together with additional hemophilia and PK experts, completed a survey and ranking exercise whereby key areas of interest in the field were identified.

Graph theory analysis of cortical thickness networks in adolescents with d-transposition of the great arteries.

Objective: Adolescents with d-transposition of the great arteries (d-TGA) who had the arterial switch operation in infancy have been found to have structural brain differences compared to healthy controls. We used cortical thickness measurements obtained from structural brain MRI to determine group differences in global brain organization using a graph theoretical approach.

Methods: Ninety-two d-TGA subjects and 49 controls were scanned using one of two identical 1.

Management and outcomes of congenital chylothorax in the neonatal intensive care unit: A case series.

Importance: Congenital chylothorax is a rare condition with pulmonary and multiorgan system effects, for which there are no standardized treatment recommendations. Collective review of known cases offers some conclusions and suggestions for treatment.

Objective: The aim of this study was to present a case series of 5 patients who were treated in the neonatal intensive care unit with chylothorax.

A Randomized Educational Interventional Trial of Spaced Education During a Pediatric Rotation.

Objective: Spacing of educational material over time has been shown to improve learning efficiency and long-term knowledge retention. We evaluated the impact of adding a spaced education curriculum to a month-long pediatric rotation.

Methods: This was a randomized controlled educational intervention trial of residents on a rotation in a pediatric emergency department.

Longitudinal MRI quantification of muscle degeneration in Duchenne muscular dystrophy.

Objective: The aim of this study was to evaluate the usefulness of magnetic resonance imaging (MRI) in detecting the progression of Duchenne muscular dystrophy (DMD) by quantification of fat infiltration (FI) and muscle volume index (MVI, a residual-to-total muscle volume ratio).

Methods: Twenty-six patients (baseline age: 5-12 years) with genetically proven DMD were longitudinally analyzed with lower limb 3T MRI, force measurements, and functional tests (Gowers, 10-m time, North Star Ambulatory Assessment, 6-min walking test). Five age-matched controls were also examined, with a total of 85 MRI studies.