Regionalization or Access to Care? A Joint Pediatric Heart Care Program That Achieves Both: One Program-Two Sites.

Regionalization of care for children with congenital heart disease has been proposed as a method to improve outcomes. This has raised concerns about limiting access to care. We present the details of a joint pediatric heart care program (JPHCP) which utilized regionalization and actually improved access to care.

Defining Expectations for Infants With Hypoplastic Left Heart Syndrome Who Survive Initial Surgical Palliation.

Background: Overall one-year non-mortality outcomes for surgically palliated hypoplastic left heart syndrome (HLHS) patients remain understudied. Using the metric Days Alive and Outside of Hospital (DAOH), the present study sought to characterize expectations for surgically palliated patients' first year of life.

Methods: The Pediatric Health Information System database was used to identify by code all HLHS patients who underwent surgical palliation (Norwood/hybrid and/or heart transplantation [HTx]) during their index neonatal admission and were successfully discharged alive (n  =  2227) and for whom one-year DAOH could be calculated.

Infants Who Require Total Parenteral Nutrition and Paralytics at Time of Heart Transplant Experience Inferior Post-Transplant Mortality.

Infants experience the worst one-year post-heart transplant (HTx) survival of any other pediatric group. Although mechanical ventilatory (MV) requirement at the time of transplant is an established predictor of post-transplant mortality, the impacts of commonly co-utilized support modalities such as total parenteral nutrition (TPN)-dependence and paralytics are understudied. All infant HTx recipients from 2003 to 2020 in both the United Network for Organ Sharing and Pediatric Health Information System databases were identified (n = 1344) and categorized depending upon requirement at the time of transplant-none (59%), MV-only (10%), MV + Paralytics (2%), TPN-dependence-only (15%), MV + TPN (10%), and MV + Paralytics + TPN (4%).

Symptoms of Survivors of Pediatric Hematopoietic Stem Cell Transplant by Age, Sex, and Transplant Type.

The purpose of this study was to describe symptoms experienced by survivors of pediatric hematopoietic stem cell transplant (HSCT), and demographic and treatment-factors associated with ongoing symptomology. Fifty pediatric survivors completed a cross-sectional pilot study. Questionnaires were administered online via REDCap to assess symptoms experienced in the last week.

Surgical Repair of Complete Common Atrioventricular Canal in a Conjoined Twin Following Separation.

Congenital heart disease (CHD) is common in conjoined twins but reports of surgical repair in this population are rare. We present the case of a conjoined twin diagnosed prenatally with a complete common atrioventricular canal defect (CAVC). Due to wound complications from twin separation, definitive repair was deferred and pulmonary arterial banding was performed initially.

Racial Fairness in Precision Medicine: Pediatric Asthma Prediction Algorithms.

Purpose: Quantify and examine the racial fairness of two widely used childhood asthma predictive precision medicine algorithms: the asthma predictive index (API) and the pediatric asthma risk score (PARS).

Design: Apply the API and PARS and evaluate model performance overall and when stratified by race.

Setting: Cincinnati, OH, USA.

Cranial Fasciitis in Children: Expanding the Spectrum of -Associated Clonal Transient Neoplasms.

Cranial fasciitis (CF) is a benign (myo)fibroblastic proliferation of children. Typical presentation consists of a rapidly growing solitary mass on the temporal or parietal cranium in the first 2 years of age. CF is characterized by a rapid growth followed by a relative slowdown and even growth arrest.

Hospital and Professional Charges and Reimbursement Patterns in Pediatric Extracorporeal Membrane Oxygenation.

Background: Extracorporeal membrane oxygenation (ECMO) is an expensive therapeutic modality. We sought to identify the main charge contributors to patient bills and analyze their patterns of reimbursement. We additionally sought to evaluate the impact of 2015 Current Procedural Terminology (CPT) code changes in professional billing for pediatric surgeons.

Teaching the Modified Single-Patch Technique for Complete Atrioventricular Septal Defect.

Understanding the morphology of atrioventricular septal defects and learning the operative strategies for their repair is one of the more difficult tasks for congenital cardiac surgery residents to master. The modified single-patch technique for several reasons lends itself to being a strategy that is relatively easy to teach residents. It has shorter cross-clamp and bypass times than the standard two-patch technique, which allows more time for the instructor to safely take the resident through the case.

Endocarditis in Bovine Vein Grafts in the Pulmonary Position Placed Surgically & Percutaneously.

Infective endocarditis (IE) is one of the major complications following pulmonary valve replacement (PVR). This analysis hopes to evaluate the incidence, outcomes and possible risk factors of IE associated with trans-catheter and surgical placement of a bovine jugular vein (BJV) graft in the pulmonary position. In this single-center retrospective study, all records of trans-catheter and surgical PVR from 3/2010 to 12/2019 were reviewed.

Differential diagnoses of irritability and temper loss in a 6-year-old.

Irritability and temper loss are common concerns among young children seen for mental health diagnostic evaluations and represent features of nearly a dozen childhood mental health conditions. Thus, careful assessment of irritability and temper loss is essential for informing case conceptualization, diagnosis, and treatment recommendations. The current case study provides a clinical description of a 6-year-old Black male ("Tyler") seen in a tertiary pediatric medical center for concerns related to significant inattention, hyperactivity, impulsivity, oppositional behaviors, irritability, and severe temper loss displayed at home and school.

Antithrombin III infusion improves anticoagulation in congenital diaphragmatic hernia patients on extracorporeal membrane oxygenation.

Purpose: Achieving effective anticoagulation during neonatal extracorporeal membrane oxygenation (ECMO) without increasing the risk of hemorrhage remains challenging. The use of antithrombin III (AT-III) for this purpose has been examined, but studies have been limited to intermittent bolus dosing. We aimed to evaluate the efficacy and safety of an institutionally developed AT-III continuous infusion protocol in neonates receiving ECMO for the treatment of congenital diaphragmatic hernia (CDH).

Short- and Long-Term Outcomes of Prenatally Diagnosed Dandy-Walker Malformation, Vermian Hypoplasia, and Blake Pouch Cyst.

Dandy-Walker continuum, which includes Dandy-Walker malformation, vermian hypoplasia, and Blake pouch cyst, is among the most commonly diagnosed posterior fossa malformation by fetal magnetic resonance imaging (MRI). The objective of our retrospective study was to evaluate fetal and postnatal MRI scan and clinical outcomes. Seventy-two patients were identified; 40 patients had postnatal imaging and follow-up (7 Dandy-Walker malformation, 26 vermian hypoplasia, and 7 Blake pouch cyst).

Pediatric Metastatic Hepatoblastoma With an Mutation and Rhabdoid Cells.

The small cell undifferentiated component of hepatoblastoma is an uncommon histologic component and is distinguished from small cell undifferentiated like pattern (originally called hepatoblastoma and now recognized to be malignant rhabdoid tumor) by the bi-allelic mutations or copy number alterations in the latter. AT-rich interactive domain-containing protein 1A () is a part of the ATP-dependent switch/sucrose non-fermentable complex assembly, but mutations have not been reported as drivers of malignant rhabdoid tumor. mutations in hepatocellular carcinoma are associated with poor prognosis but its significance in hepatoblastoma is unknown.

Risk of Pediatric Cardiac Surgery Increased in Patients Undergoing Tracheal Surgery During the Same Hospitalization.

Background: The impact of complex tracheal surgery (TS) on outcomes of children undergoing cardiac surgery has rarely been investigated in large national databases and could impact anticipated outcomes in those patients.

Methods: The Pediatric Health Information System database was reviewed from January 1, 2005 to December 31, 2014, for pediatric (<18 years) patients undergoing select cardiac surgical procedures using International Classification of Disease-9 procedural coding. Patients were divided based upon having TS (excluding tracheostomy) during the same hospitalization.

Highlights From AES2020, a Virtual American Epilepsy Society Experience.

Due to COVID-19 a live, in-person meeting was not possible for the American Epilepsy Society in 2020. An alternative, virtual event, the AES2020, was held instead. AES2020 was a great success with 4679 attendees from 70 countries.

Transplantation for Congenital Heart Disease: Focus on the Impact of Functionally Univentricular Versus Biventricular Circulation.

Background: Varying single center data exist regarding the posttransplant outcomes of patients with single ventricle circulation, particularly following the Fontan operation. We sought to better elucidate these results in patients with congenital heart disease (CHD) through combining two national databases.

Methods: The United Network for Organ Sharing (UNOS) transplantation database was merged with the Pediatric Health Information System (PHIS), an administrative database with 71% of UNOS patients matched.