Posterior reversible encephalopathy syndrome in a pediatric heart transplant recipient with coarctation of aorta.

Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiographic syndrome reported in children with hypertension due to renal diseases, immunosuppression after solid organ transplant, cytotoxic agents for chemotherapy, and many others rare instances. We described PRES in a 6-year-old child with hypertension secondary to an incidental postoperative coarctation of the aorta after heart transplantation (HT). Her blood pressure was well controlled with amlodipine during the outpatient visits and home monitoring of blood pressure, but she had hypertension when presented with neurological symptoms.

Heart Failure with Preserved Ejection Fraction in Children.

Diastolic dysfunction (DD) refers to abnormalities in the mechanical function of the left ventricle (LV) during diastole. Severe LVDD can cause symptoms and the signs of heart failure (HF) in the setting of normal or near normal LV systolic function and is referred to as diastolic HF or HF with preserved ejection fraction (HFpEF). Pediatric cardiologists have long speculated HFpEF in children with congenital heart disease and cardiomyopathy.

Racial and Ethnic Disparity in Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 in Mississippi, USA.

We aimed to study the disparity in the clinical profile and outcomes of hospitalized Multisystem Inflammatory Syndrome in Children (MIS-C) patients at our center. The second goal was to examine the temporal association with preceding SARS-CoV-2 infection by race/ethnicity in our community in Mississippi. We found the racial disparity in the prevalence of MIS-C exceeded its temporal association with SARS-CoV-2 infections.

Trends in Contemporary Use of Ventricular Assist Devices in Children Awaiting Heart Transplantation and Their Outcomes by Race/Ethnicity.

This retrospective study included children aged ≤18 years who had durable ventricular assist devices (VADs) as a bridge to transplantation from the United Network Organ Sharing (UNOS) database between 2011 and 2020. We evaluated 90 day waitlist mortality and 1 year posttransplant mortality after VAD implantation in children stratified by race/ethnicity: Black, White, and Others. The VAD was used in a higher proportion of Black children listed for heart transplantation (HT) (26%) versus Other (25%) versus White (22%); p < 0.

Telehealth: Opportunities to Improve Access, Quality, and Cost in Pediatric Care.

The use of telehealth technology to connect with patients has expanded significantly over the past several years, particularly in response to the global coronavirus disease 2019 pandemic. This technical report describes the present state of telehealth and its current and potential applications. Telehealth has the potential to transform the way care is delivered to pediatric patients, expanding access to pediatric care across geographic distances, leveraging the pediatric workforce for care delivery, and improving disparities in access to care.

SARS-CoV-2 infection induced thyroid storm and heart failure in an adolescent girl.

We report a case of thyroid storm precipitated by SARS-CoV-2 infection in an adolescent girl with a history of Graves disease and dilated cardiomyopathy. This case highlights that SARS-CoV-2 infection can potentially trigger a thyrotoxicosis crisis and acute decompensated heart failure in a patient with underlying thyroid disease and myocardial dysfunction even in the absence of multi-system inflammatory syndrome in children. We systematically reviewed the thyrotoxicosis cases with SARS-CoV-2 infection and described its impact on pre-existing dilated cardiomyopathy.

Telehealth: Improving Access to and Quality of Pediatric Health Care.

All children and adolescents deserve access to quality health care regardless of their race/ethnicity, health conditions, financial resources, or geographic location. Despite improvements over the past decades, severe disparities in the availability and access to high-quality health care for children and adolescents continue to exist throughout the United States. Economic and racial factors, geographic maldistribution of primary care pediatricians, and limited availability of pediatric medical subspecialists and pediatric surgical specialists all contribute to inequitable access to pediatric care.

Myocarditis and Pericarditis Following mRNA COVID-19 Vaccination: What Do We Know So Far?

This is a cross-sectional study of 29 published cases of acute myopericarditis following COVID-19 mRNA vaccination. The most common presentation was chest pain within 1-5 days after the second dose of mRNA COVID-19 vaccination. All patients had an elevated troponin.

Current and Future Drug and Device Therapies for Pediatric Heart Failure Patients: Potential Lessons from Adult Trials.

This review discusses the potential drug and device therapies for pediatric heart failure (HF) due to reduced systolic function. It is important to realize that most drugs that are used in pediatric HF are extrapolated from adult cardiology practices or consensus guidelines based on expert opinion rather than on evidence from controlled clinical trials. It is difficult to conclude whether the drugs that are well established in adult HF trials are also beneficial for children because of tremendous heterogeneity in the mechanism of HF in children and variations in the pharmacokinetics and pharmacodynamics of drugs from birth to adolescence.

Workforce Trends and Analysis of Selected Pediatric Subspecialties in the United States.

Objective: To update pediatric subspecialty workforce data to support evidence-based legislation and public policy decisions by replicating the American Academy of Pediatrics' 1998 Future of Pediatric Education (FOPE II) workforce survey.

Methods: A descriptive and comparative analysis of survey responses from 9950 US pediatric subspecialists who completed an electronic survey.

Results: Pediatric subspecialists are working fewer hours and spending less of their time in direct patient care than they did in 1998 but the mean hours worked differs significantly according to subspecialty.

Externally applied compression therapy for Fontan patients.

Background: Limited therapeutic options are available for Fontan patients with dysfunctional or failing single ventricle physiology. This study describes the evaluation of an alternative, non-invasive, at-home therapeutic compression treatment for Fontan patients. Our hypothesis is that routinely administered, externally applied compression treatments to the lower extremities will augment systemic venous return, improve ventricular preload, and thus enhance cardiac output in Fontan patients.

Mechanical Circulatory Support Devices for Pediatric Patients With Congenital Heart Disease.

The use of mechanical circulatory support (MCS) devices is a viable therapeutic treatment option for patients with congestive heart failure. Ventricular assist devices, cavopulmonary assist devices, and total artificial heart pumps continue to gain acceptance as viable treatment strategies for both adults and pediatric patients as bridge-to-transplant, bridge-to-recovery, and longer-term circulatory support alternatives. We present a review of the current and future MCS devices for patients having congenital heart disease (CHD) with biventricular or univentricular circulations.

The Use of Telemedicine to Address Access and Physician Workforce Shortages.

The use of telemedicine technologies by primary care pediatricians, pediatric medical subspecialists, and pediatric surgical specialists (henceforth referred to as "pediatric physicians") has the potential to transform the practice of pediatrics. The purpose of this policy statement is to describe the expected and potential impact that telemedicine will have on pediatric physicians' efforts to improve access and physician workforce shortages. The policy statement also describes how the American Academy of Pediatrics can advocate for its members and their patients to best use telemedicine technologies to improve access to care, provide more patient- and family-centered care, increase efficiencies in practice, enhance the quality of care, and address projected shortages in the clinical workforce.

Three-dimensional laser flow measurements of a patient-specific fontan physiology with mechanical circulatory assistance.

Mechanical assistance of the Fontan circulation is hypothesized to enhance ventricular preload and improve cardiac output; however, little is known about the fluid dynamics. This study is the first to investigate the three-dimensional flow conditions of a blood pump in an anatomic Fontan. Laser measurements were conducted having an axial flow impeller in the inferior vena cava.

Stereo-particle image velocimetry measurements of a patient-specific Fontan physiology utilizing novel pressure augmentation stents.

Single ventricle anomalies are a challenging set of congenital heart defects that require lifelong clinical management due to progressive decline of cardiovascular function. Few therapeutic devices are available for these patients, and conventional blood pumps are not designed for the unique anatomy of the single ventricle physiology. To address this unmet need, we are developing an axial flow blood pump with a protective cage or stent for Fontan patients.

Experimental measurements of energy augmentation for mechanical circulatory assistance in a patient-specific Fontan model.

A mechanical blood pump specifically designed to increase pressure in the great veins would improve hemodynamic stability in adolescent and adult Fontan patients having dysfunctional cavopulmonary circulation. This study investigates the impact of axial-flow blood pumps on pressure, flow rate, and energy augmentation in the total cavopulmonary circulation (TCPC) using a patient-specific Fontan model. The experiments were conducted for three mechanical support configurations, which included an axial-flow impeller alone in the inferior vena cava (IVC) and an impeller with one of two different protective stent designs.

Pneumatically-driven external pressure applicator to augment Fontan hemodynamics: preliminary findings.

Background: This study investigated the application of circumferentially applied, external pressure to the lower extremities as a preventative measure and long-term clinical treatment strategy for Fontan patients.

Objective: We hypothesized that the application of circumferential pressure to the lower limbs will augment venous return and thus cardiac output.

Methods: Two patients (an extra-cardiac and intra-atrial Fontan) were evaluated.

Dual-pump support in the inferior and superior vena cavae of a patient-specific fontan physiology.

The implementation of simultaneous mechanical cavopulmonary assistance having blood pumps located in both of the vena cavae is investigated as an approach to treating patients with an ailing Fontan physiology. Identical intravascular blood pumps are employed to model the hemodynamic support of a patient-specific Fontan. Pressure flow characteristics, energy gain calculations, and blood damage analyses are assessed for each model.

A viable therapeutic option: mechanical circulatory support of the failing Fontan physiology.

A blood pump specifically designed to augment flow from the great veins through the lungs would ameliorate the poor physiology of the failing univentricular circulation and result in a paradigm shift in the treatment strategy for Fontan patients. This study is the first to examine mechanical cavopulmonary assistance with a blood pump in the inferior vena cava (IVC) and hepatic blood flow. Five numerical models of mechanical cavopulmonary assistance were investigated using a three-dimensional, reconstructed, patient-specific Fontan circulation from magnetic resonance imaging data.

Steady flow analysis of mechanical cavopulmonary assistance in MRI-derived patient-specific fontan configurations.

This numerical study examined the performance of an intravascular axial flow blood pump for mechanical hemodynamic support of patients in the setting of Fontan failure, which presently has few treatment options. Three anatomically accurate geometries of the total cavopulmonary connection (TCPC) were generated using patients' magnetic resonance imaging data. These patient-specific geometries, as well as an idealized version with cylindrical vessels, were computationally analyzed with and without a pump in the inferior vena cava.

Controlled pitch-adjustment of impeller blades for an intravascular blood pump.

Thousands of mechanical blood pumps are currently providing circulatory support, and the incidence of their use continues to increase each year. As the use of blood pumps becomes more pervasive in the treatment of those patients with congestive heart failure, critical advances in design features to address known limitations and the integration of novel technologies become more imperative. To advance the current state-of-the-art in blood pump design, this study investigates the inclusion of pitch-adjusting blade features in intravascular blood pumps as a means to increase energy transfer; an approach not explored to date.