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Abstract Background Proteases that degrade the amyloid ß-protein (Aß) have emerged as key players in the etiology and potential treatment of Alzheimer’s disease (AD), but it is unlikely that all such proteases have been identified. To discover new Aß-degrading proteases (AßDPs), we conducted an unbiased, genome-scale, functional cDNA screen designed to identify proteases capable of lowering net Aß levels produced by cells, which were subsequently characterized for Aß-degrading activity using an array of downstream assays. Results The top hit emerging from the screen was ß-site amyloid precursor protein-cleaving enzyme 2 (BACE2), a rather unexpected finding given the well-established role of its close homolog, BACE1, in the production of Aß. BACE2 is known to be capable of lowering Aß levels via non-amyloidogenic processing of APP. However, in vitro, BACE2 was also found to be a particularly avid AßDP, with a catalytic efficiency exceeding all known AßDPs except insulin-degrading enzyme (IDE). BACE1 was also found to degrade Aß, albeit ~150-fold less efficiently than BACE2. Aß is cleaved by BACE2 at three peptide bonds—Phe19-Phe20, Phe20-Ala21, and Leu34-Met35—with the latter cleavage site being the initial and principal one. BACE2 overexpression in cultured cells was found to lower net Aß levels to a greater extent than multiple, well-established AßDPs, including neprilysin (NEP) and endothelin-converting enzyme-1 (ECE1), while showing comparable effectiveness to IDE. Conclusions This study identifies a new functional role for BACE2 as a potent AßDP. Based on its high catalytic efficiency, its ability to degrade Aß intracellularly, and other characteristics, BACE2 represents a particulary strong therapeutic candidate for the treatment or prevention of AD.

Abstract Background Increasing the cultural competence of physicians and other health care providers has been suggested as one mechanism for reducing health disparities by improving the quality of care across racial/ethnic groups. While cultural competency training for physicians is increasingly promoted, relatively few studies evaluating the impact of training have been published. Methods We recruited 53 primary care physicians at 4 diverse practice sites and enrolled 429 of their patients with diabetes and/or hypertension. Patients completed a baseline survey which included a measure of physician culturally competent behaviors. Cultural competency training was then provided to physicians at 2 of the sites. At all 4 sites, physicians received feedback in the form of their aggregated cultural competency scores compared to the aggregated scores from other physicians in the practice. The primary outcome at 6 months was change in the Patient-Reported Physician Cultural Competence (PRPCC) score; secondary outcomes were changes in patient trust, satisfaction, weight, systolic blood pressure, and glycosylated hemoglobin. Multiple analysis of variance was used to control for differences patient characteristics and baseline levels of the outcome measure between groups. Results Patients had a mean of 2.8 + 2.2 visits to the study physician during the study period. Changes in all outcomes were similar in the "Training + Feedback" group compared to the "Feedback Only" group (PRPCC: 3.7 vs.1.8; trust: -0.7 vs. -0.2 ; satisfaction: 1.9 vs. 2.5; weight: -2.5 lbs vs. -0.7 lbs; systolic blood pressure: 1.7 mm Hg vs. 0.1 mm Hg; glycosylated hemoglobin 0.02% vs. 0.07%; p = NS for all). Conclusion The lack of measurable impact of physician training on patient-reported and disease-specific outcomes in the current has several possible explanations, including the relatively limited nature of the intervention. We hope that the current study will help provide a basis for future studies, using more intensive interventions with different provider groups.

Objective: To retrospectively assess the prevalence of secondary overtriage (SO) within a rural regional Appalachian health care system., Methods: Trauma registry data was extracted for all trauma activation transfer patients from 2017 to 2022. Transferred patients were then stratified into two groups, non-secondary overtriage (non-SO) or SO. Patients were considered SO if they met three criteria following transfer: an Injury Severity Score (ISS) of less than 15, no required operative intervention, and discharge within 48 hours of arrival. Descriptive statistics were compared for age, length of stay (LOS), ICU LOS, and ISS. Surgical subspecialty consultations were compared between the two groups. Patients in the SO group were further assessed by body region of injury and Abbreviated Injury Score (AIS)., Results: Among 3,291 trauma activation transfer patients, 43% (1,407) were considered SO transfers. Patients in the SO group were significantly younger, had shorter average hospital and ICU LOS, and lower ISS compared to the non-SO group. Additionally, 25.7% of patients in the SO group had injuries to the head or neck of which 8.96% have an AIS ≥3. 21% of patients had injuries to the face, with 0.14% having an AIS ≥3., Conclusions: 43% of transfer patients in this study met our definition of SO. Although no optimal rate of SO has been universally established, limiting SO stands to benefit both patients and trauma systems. This study highlights how institutional analysis of transfer patients may help inform transfer protocols to reduce secondary overtriage and overutilization of scarce resources., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Idiopathic acute rectal necrosis (IARN) is a rare condition due to a robust rectal blood supply. This report describes an 83-year-old man presenting with septic shock due to distal sigmoid and complete rectal necrosis with perforation. He underwent emergent exploratory laparotomy, sigmoid and proximal rectum resection, and end sigmoid colostomy creation with delayed distal rectal evaluation. Bedside proctoscopy revealed pale, viable-appearing distal rectal mucosa on postoperative day 3. The patient had a protracted, complicated hospital stay but required no further operative intervention. Subsequent colostomy reversal was done 8 months postoperatively, and the patient did well and has been discharged with normal gastrointestinal function. Our successful conservative operative management of IARN deviates from previously described management in the literature which is emergent abdominoperineal resection. This conservative surgical strategy appears to have contributed to the patient's positive outcomes, highlighting the importance of considering a similar approach for future IARN cases., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Many medical schools incorporate longitudinal clerkships, which promote continuity and may offer early clinical exposure during the preclinical curriculum. However, the mechanisms of near-peer learning and how it contributes to the development of clinical skills in longitudinal clinical experiences are less clear. The authors explored how peer-to-peer interactions among medical students influenced their developmental trajectories from nascent clinicians to more seasoned practitioners capable of juggling dual roles of clinical care and clinical supervision within longitudinal clerkships., Method: The Education-Centered Medical Home (ECMH) at Northwestern University Feinberg School of Medicine is a longitudinal clerkship that represents an ideal setting to explore peer learning. At ECMH, continuity is established across all 4 years of medical school among small groups of students from each year, a preceptor, and a panel of outpatients. The authors conducted 6 focus groups and 9 individual interviews between March 2021 and February 2023 with medical students from all years. Using constructivist grounded theory, the authors collected and analyzed data iteratively using constant comparison to identify themes and explore their relationships., Results: Within ECMH, peer relationships fostered an informal learning culture that enabled meaningful peer interactions while reinforcing the established culture. The authors identified 3 essential learning practices between senior and junior medical students: preparing for patient encounters, shifting roles dynamically during the joint encounter, and debriefing encounters afterward. These practices strengthened learning relationships and supported students' developmental trajectories., Conclusions: Longitudinal peer learning relationships enabled meaningful peer interaction that influenced medical students' clinical development and capability for clinical supervision. Mutual trust, familiarity, and continuity facilitate targeted feedback practices and growth at the edge of junior students' capabilities. Optimizing this peer learning environment and seeking new opportunities to use longitudinal peer learning in clinical environments could promote psychological safety and professional identity formation for medical students., (Copyright © 2024 the Association of American Medical Colleges.)

Background: This study analyzed the overall incidence of delirium, comorbid conditions, injury patterns, and pharmacological risk factors for the development of delirium in an alert, geriatric trauma population., Methods: IRB-approved, prospective, consecutive cohort series at two Southeastern Level 1 trauma centers from June 11 to August 15, 2023. Delirium was assessed using the Confusion Assessment Method (CAM) score. Comorbidities and medications were detailed from electronic medical records. Inclusion criteria: age ≥55, GCS ≥14, and ICU admission for trauma. Patients on a ventilator were excluded. Data was analyzed using SPSS version 28 (Armonk, NY: IBM Corp)., Results: In total, 196 patients met inclusion criteria. Incidences of delirium for Hospital 1 (n = 103) and Hospital 2 (n = 93) were 15.5% and 12.9%, respectively, with an overall incidence of 14.3% and with no statistical differences between hospitals ( P = .599). CAD, CKD, dementia, stroke history, and depression were statistically significant risk factors for developing delirium during ICU admission. Inpatient SSRI/SNRIs, epinephrine/norepinephrine, and lorazepam were significant risk factors. Injury patterns, operative intervention, and use of lidocaine infusions and gabapentin were not statistically significant in delirium development. Using binary linear regression (BLR) analysis, independent risk factors for delirium were dementia, any stage CKD, home SSRI/SRNI prescription, any spine injury and cerebrovascular disease, or injury., Discussion: Comorbidities of CAD, CHF, CKD, and depression, and these medications: home lorazepam and ICU epinephrine/norepinephrine statistically are more common in patients developing delirium. Dementia, CKD, home SSRI/SRNI and stroke/cerebrovascular disease/injury, and spine injuries are independent predictors by BLR., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Every 10 years, the American Heart Association (AHA) Emergency Cardiovascular Care Committee establishes goals to improve survival from cardiac arrest. These goals align with broader AHA Impact Goals and support the AHA's advocacy efforts and strategic investments in research, education, clinical care, and quality improvement programs. This scientific statement focuses on 2030 AHA emergency cardiovascular care priorities, with a specific focus on bystander cardiopulmonary resuscitation, early defibrillation, and neurologically intact survival. This scientific statement also includes aspirational goals, such as establishing cardiac arrest as a reportable disease and mandating reporting of standardized outcomes from different sources; advancing recognition of and knowledge about cardiac arrest; improving dispatch system response, availability, and access to resuscitation training in multiple settings and at multiple time points; improving availability, access, and affordability of defibrillators; providing a focus on early defibrillation, in-hospital programs, and establishing champions for debriefing and review of cardiac arrest events; and expanding measures to track outcomes beyond survival. The ability to track and report data from these broader aspirational targets will potentially require expansion of existing data sets, development of new data sets, and enhanced integration of technology to collect process and outcome data, as well as partnerships of the AHA with national, state, and local organizations. The COVID-19 (coronavirus disease 2019) pandemic, disparities in COVID-19 outcomes for historically excluded racial and ethnic groups, and the longstanding disparities in cardiac arrest treatment and outcomes for Black and Hispanic or Latino populations also contributed to an explicit focus and target on equity for the AHA Emergency Cardiovascular Care 2030 Impact Goals.

Between 0.25% and 3% of admissions to the NICU, PICU, and PCICU receive cardiopulmonary resuscitation (CPR). Most CPR events occur in patients <1 year old. The incidence of CPR is 10 times higher in the NICU than at birth. Therefore, optimizing the approach to CPR in hospitalized neonates and infants is important. At birth, the resuscitation of newborns is performed according to neonatal resuscitation guidelines. In older infants and children, resuscitation is performed according to pediatric resuscitation guidelines. Neonatal and pediatric guidelines differ in several important ways. There are no published recommendations to guide the transition from neonatal to pediatric guidelines. Therefore, hospitalized neonates and infants can be resuscitated using neonatal guidelines, pediatric guidelines, or a hybrid approach. This report summarizes the current neonatal and pediatric resuscitation guidelines, considers how to apply them to hospitalized neonates and infants, and identifies knowledge gaps and future priorities. The lack of strong scientific data makes it impossible to provide definitive recommendations on when to transition from neonatal to pediatric resuscitation guidelines. Therefore, it is up to health care teams and institutions to decide if neonatal or pediatric guidelines are the best choice in a given location or situation, considering local circumstances, health care team preferences, and resource limitations., (Copyright © 2024 by the American Academy of Pediatrics.)

Sunken Skin Flap Syndrome (or Syndrome of the Trephined) following a head trauma is rare, but most often results from complications after decompressive craniectomy. This syndrome is most often characterized by neurological dysfunction that improves with cranioplasty. Early diagnosis and treatment are critically important to long term neurological improvement. This is a case report of a 49-year-old male who fell down a flight of stairs and was found unresponsive. Initial imaging revealed extensive head trauma. Neurosurgery performed an emergency decompressive craniectomy, but his post-operative course was complicated by the development of sunken flap syndrome one month after his initial surgery, diagnosed by an acute neurological decline and emergent CT imaging. A review of the literature indicates that this is a rarely documented finding, and this case report discusses the critical components of diagnosis and treatment of this unusual and potentially lethal condition.

Simulation in health-care professions has grown in the last few decades. We provide an overview of the history of simulation in other fields, the trajectory of simulation in health professions education, and research in medical education, including the learning theories and tools to assess and evaluate simulation programs. We also propose future directions for simulation and research in health professions education., Competing Interests: Disclosure M.E. McBride is a paid consultant for the American Heart Association., (Copyright © 2022 Elsevier Inc. All rights reserved.)

The number of adults with congenital heart disease is rapidly increasing, resulting in more emergency care needs of this unique population. Concomitantly, the number of physicians trained in adult congenital heart disease (ACHD) care is insufficient, leading to physicians with limited experience assuming primary responsibility for the management of acute illness. We developed a simulation-based curriculum consisting of three cases and linked assessment instruments for fellows in multiple training programs to enhance their experience in this growing field. A 40-min asynchronous didactic presentation on ACHD emergencies was provided between pretests and posttests. Each participant was given checklist, global, and timeliness scores, and a second rater scored a subset to assess inter-rater reliability. Twenty-two participants across multiple disciplines completed the study. Our results demonstrate a significant and meaningful improvement in checklist scores, as well as a significant improvement in the secondary measures of global and efficiency performance from the first simulation to the second. Comfort levels for trainees improved significantly on post-test surveys. Inter-rater reliability was greater than 0.6 for all assessments. In conclusion, our novel simulation-based educational curriculum improved trainee performance in managing emergencies in adults with congenital heart disease, and we provide validity evidence for use of our checklist in training fellows for formative feedback., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

This manuscript is one component of a larger series of articles produced by the Neonatal Cardiac Care Collaborative that are published in this supplement of Pediatrics. In this review article, we summarize the contemporary physiologic principles, evaluation, and management of acute care issues for neonates with complex congenital heart disease. A multidisciplinary team of authors was created by the Collaborative's Executive Committee. The authors developed a detailed outline of the manuscript, and small teams of authors were assigned to draft specific sections. The authors reviewed the literature, with a focus on original manuscripts published in the last decade, and drafted preliminary content and recommendations. All authors subsequently reviewed and edited the entire manuscript until a consensus was achieved. Topics addressed include cardiopulmonary interactions, the pathophysiology of and strategies to minimize the development of ventilator-induced low cardiac output syndrome, common postoperative physiologies, perioperative bleeding and coagulation, and common postoperative complications., (Copyright © 2022 by the American Academy of Pediatrics.)

Pediatric Cardiac Critical Care (PCCC) is a challenging discipline where decisions require a high degree of preparation and clinical expertise. In the modern era, outcomes of neonates and children with congenital heart defects have dramatically improved, largely by transformative technologies and an expanding collection of pharmacotherapies. Exponential advances in science and technology are occurring at a breathtaking rate, and applying these advances to the PCCC patient is essential to further advancing the science and practice of the field. In this article, we identified and elaborate on seven key elements within the PCCC that will pave the way for the future., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Pollak, Feinstein, Mannarino, McBride, Mendonca, Keizman, Mishaly, van Leeuwen, Roeleveld, Koers and Klugman.)

This article aims to provide guidance to health care workers for the provision of basic and advanced life support to children and neonates with suspected or confirmed coronavirus disease 2019 (COVID-19). It aligns with the 2020 American Heart Association Guidelines for Cardiopulmonary Resuscitation (CPR) and Emergency Cardiovascular care while providing strategies for reducing risk of transmission of severe acute respiratory syndrome coronavirus 2 to health care providers. Patients with suspected or confirmed COVID-19 and cardiac arrest should receive chest compressions and defibrillation, when indicated, as soon as possible. Because of the importance of ventilation during pediatric and neonatal resuscitation, oxygenation and ventilation should be prioritized. All CPR events should therefore be considered aerosol-generating procedures. Thus, personal protective equipment (PPE) appropriate for aerosol-generating procedures (including N95 respirators or an equivalent) should be donned before resuscitation, and high-efficiency particulate air filters should be used. Any personnel without appropriate PPE should be immediately excused by providers wearing appropriate PPE. Neonatal resuscitation guidance is unchanged from standard algorithms, except for specific attention to infection prevention and control. In summary, health care personnel should continue to reduce the risk of severe acute respiratory syndrome coronavirus 2 transmission through vaccination and use of appropriate PPE during pediatric resuscitations. Health care organizations should ensure the availability and appropriate use of PPE. Because delays or withheld CPR increases the risk to patients for poor clinical outcomes, children and neonates with suspected or confirmed COVID-19 should receive prompt, high-quality CPR in accordance with evidence-based guidelines., (Copyright © 2022 by the American Academy of Pediatrics.)

Background: Coaching in graduate medical education provides a facilitative approach to feedback as well as opportunities for residents and fellows to engage with feedback and develop individualized improvement goals., Objective: To explore the roles and actions of successful coaches in longitudinal coaching relationships and how they enable feedback processes., Methods: Using interpretive description methodology, we performed semi-structured interviews with pediatrics fellows (n=11), faculty coaches (n=9), and program directors (n=2) from 2 pediatric subspecialty fellowship training programs at Ann and Robert H. Lurie Children's Hospital of Chicago. Both training programs had previously implemented longitudinal clinical coaching programs. Interview questions aimed to explore the roles and impacts of coaches within a longitudinal coaching program. Interviews took place in 2019 and 2020., Results: We identified 4 major actions to the coaching role in longitudinal coaching relationships: (1) establish the coach-fellow relationship; (2) prepare for the coaching conversation; (3) facilitate feedback dialogue; and (4) serve as the go-to person to raise uncomfortable issues. Additionally, nearly all participants expressed support for a longitudinal coaching program to support fellows' growth and development of personalized learning goals., Conclusions: By fulfilling these 4 key aspects to the coaching role, coaches in longitudinal relationships with coachees enable feedback processes., Competing Interests: Conflict of interest: The authors declare they have no competing interests.

Objectives: Define a set of entrustable professional activities for pediatric cardiac critical care that are recognized as the core activities of the subspecialty by a diverse group of pediatric cardiac critical care physicians and that can be broadly and consistently applied irrespective of training pathway., Design: Mixed methods study with sequential integration of qualitative and quantitative data., Setting: Structured telephone interviews of pediatric cardiac critical care medical directors at Pediatric Cardiac Critical Care Consortium centers followed by an electronic survey of pediatric cardiac critical care physician members of the Pediatric Cardiac Intensive Care Society from across the United States and internationally., Subjects: Pediatric cardiac intensive care physicians., Interventions: None., Measurements and Main Results: Twenty-four of 26 eligible Pediatric Cardiac Critical Care Consortium medical directors participated in the interviews. Based on qualitative analyses of interview data, we identified an initial set of nine entrustable professional activities. Fifty-eight of 185 eligible physicians completed a subsequent survey asking them to rate their agreement with the entrustable professional activities. It showed consensus (> 80% agreement) with the entire initial set of entrustable professional activities, with greater than 96% agreement in most cases. The feedback from free-text survey responses was incorporated to generate a final set of entrustable professional activities., Conclusions: We generated a set of nine entrustable professional activities, which we believe can be broadly applied to any physician training in pediatric cardiac critical care, irrespective of individual training pathway. Next steps include incorporation of these entrustable professional activities into curriculum design and trainee assessment tools., Competing Interests: Dr. Werho received funding from the University of Michigan Congenital Heart Center, the University of California, San Diego, the American Academy of Pediatrics, and Cleveland Clinic. Dr. McBride received funding from the American Heart Association. The remaining authors have disclosed that they do not have any potential conflicts of interest., (Copyright © 2021 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.)

While outcomes for neonates with congenital heart disease have improved, it is apparent that substantial variability exists among centers with regard to the multidisciplinary approach to care for this medically fragile patient population. We endeavored to understand the landscape of neonatal cardiac care in the United States. A survey was distributed to physicians who provide neonatal cardiac care in the United States regarding (1) collaborative efforts in care of neonates with congenital heart disease (CHD); (2) access to neonatal cardiac training; and (3) barriers to the implementation of protocols for neonatal cardiac care. Responses were collected from 10/2018 to 6/2019. We received responses from 172 of 608 physicians (28% response rate) from 89 centers. When compared to responses received from physicians at low-volume centers (< 300 annual bypass cases), those at high-volume centers reported more involvement from the neurodevelopmental teams (58% vs. 29%; P = 0.012) and a standardized transition to outpatient care (68% vs. 52%; P = 0.038). While a majority of cardiothoracic surgery and anesthesiology respondents reported multidisciplinary involvement, less than half of cardiology and neonatology supported this statement. The most commonly reported obstacles to multidisciplinary engagement were culture (61.6%) and logistics (47.1%). Having a standardized neonatal cardiac curriculum for neonatal fellows was positively associated with the perception that multidisciplinary collaboration was "always" in place (53% vs. 40%; P = 0.09). There is considerable variation among centers in regard to personnel involved in neonatal cardiac care, related education, and perceived multidisciplinary collaboration among team members. The survey findings suggest the need to establish concrete standards for neonatal cardiac surgical programs, with ongoing quality improvement processes.

Purpose of Review: This review highlights the use of simulation as an educational tool in the highly specialized pediatric cardiac intensive care unit (PCICU)., Recent Findings: Healthcare simulation is used in high acuity medical environments to test healthcare systems. Healthcare simulation can improve team training, patient safety, and improve medical decision-making. Complex physiologies in the PCICU demand effective teamwork to consistently deliver high-quality patient care. Simulation-based PCICU learning objectives depend on a structured cognitive load framework to account for individual learner abilities, team constructs, and healthcare resources., Summary: PCICU simulation programs are strengthened by utilizing traditional education theory, with careful consideration of complex physiologies, interprofessional personnel, and center-specific resources. Virtual platforms should continue to evolve to provide additional, more convenient venues for individual learners and teams. Healthcare systems should frequently intersect with simulation educators to create relevant learning objectives that will contribute to patient safety, improve team performance, and patient outcomes., (© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2021.)

Objective: Children with congenital heart disease (CHD) have complex unique post-operative care needs. Limited data assess parents' hospital discharge preparedness and education quality following cardiac surgery. The goals were to identify knowledge gaps in discharge preparedness after congenital heart surgery and to assess the acceptability of an educational mobile application to improve discharge preparedness., Methods: Telephonic interviews with parents of children with two-ventricle physiology who underwent cardiac surgery 5-7 days post-discharge and in-person interviews with clinicians were conducted. We collected parent and clinician demographics, parent health literacy information and patient clinical data. We analysed interview transcripts using summative content analysis., Results: We interviewed 26 parents and 6 clinicians. Twenty-two of the 26 (85%) parents felt ready for discharge; 4 of the 6 (67%) clinicians did not feel most parents were ready for discharge. Fifteen of the 26 parents (58%) reported receiving the majority of discharge teaching on the day of discharge. Eight parents did not feel like all of their questions were answered. Most parents (14/26, 54%) preferred visual educational learning aids and could accurately describe important aspects of care. Most parents (23/26, 88%) and all 6 clinicians felt a mobile application for post-operative care education would be helpful., Conclusions: Most parents received education on the day of discharge and could describe the information they received prior to discharge, although there were some preparedness gaps identified after discharge. Clinicians and parents varied in their perceptions of the readiness for discharge. Most responses suggest that a mobile application for discharge education may be helpful for transition to home.

Objectives: To assess the distribution, service delivery, and staffing of pediatric cardiac intensive care in the United States., Design: Based on a 2016 national PICU survey, and verified through online searching and clinician networking, medical centers were identified with a separate cardiac ICU or mixed ICU. These centers were sent a structured web-based survey up to four times, with follow-up by mail and phone for nonresponders., Setting: Cardiac ICUs were defined as specialized units, specifically for the treatment of children with life-threatening primary cardiac conditions. Mixed ICUs were defined as separate units, specifically for the treatment of children with life-threatening conditions, including primary cardiac disease., Participants: Cardiac ICU or mixed ICU physician medical directors or designees., Measurements and Main Results: One-hundred twenty ICUs were identified: 61 (51%) were mixed ICUs and 59 (49%) were cardiac ICUs. Seventy five percent of institutions at least sometimes used a neonatal ICU prior to surgery. The most common temporary cardiac support beyond extracorporeal membrane oxygenation was a centrifugal pump such as Centrimag. Durable cardiac support devices were far more common in separate cardiac ICUs (84% vs 20%; p < 0.0001). Significantly less availability of electrophysiology, heart failure, and cardiac anesthesia consultation was available in mixed ICUs (p = 0.0003, p < 0.0001, p = 0.042 respectively). ICU attending physicians were in-house day and night 98% of the time in mixed ICUs and 87% of the time in cardiac ICUs. Nurse practitioners were consistent front-line providers in the ICUs caring for children with primary cardiac disease staffing 88% of cardiac ICUs and 56% of mixed ICUs. Mixed ICUs were more commonly staffed with pediatric residents, and critical care fellows were found in more cardiac ICUs (83% vs 77%; p < 0.0001)., Conclusions: Mixed ICUs and cardiac ICUs have statistically different staffing models and available services. More evaluation is needed to understand how this may impact patient outcomes and training programs of physicians and nurses.

At the 14th Annual International Meeting for the Pediatric Cardiac Intensive Care Society, the authors presented a simulation workshop for junior multidisciplinary providers focused on cardiopulmonary interactions. We provide an overview of educational theories of particular relevance to curricular design for simulation-based or enhanced activities. We then demonstrate how these theories are applied to curriculum development for individuals to teams and for novice to experts. We review the role of simulation in cardiac intensive care education and the education theories that support its use. Finally, we demonstrate how a conceptual framework, SIMZones, can be applied to design effective simulation-based teaching.

Pulmonary hypertension with transposition of the great arteries is associated with significant morbidity and mortality. At the worst end of the spectrum are patients who undergo extracorporeal support perioperatively. We describe our experience with three patients who received preoperative extracorporeal support and separated from cardiopulmonary bypass successfully on conventional postoperative care, with no significant deficits on follow-up.

This review article will discuss the indications for and outcomes of neonates with congenital heart disease who receive extracorporeal membrane oxygenation (ECMO) support. Most commonly, ECMO is used as a perioperative bridge to recovery or temporary support for those after cardiac arrest or near arrest in patients with congenital or acquired heart disease. What had historically been considered a contraindication to ECMO, is evolving and more of the sickest and most complicated babies are cared for on ECMO. Given that, it is imperative for aggressive survellience for long-term morbidity in survivors, particularly neurodevelopmental outcomes., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Introduction: A theoretical framework was recently proposed that encapsulates learner responses to simulated death due to action or inaction in the pediatric context. This framework, however, was developed at an institution that allows simulated death and thus does not address the experience of those centers at which this technique is not used. To address this, we performed a parallel qualitative study with the intent of augmenting the initial framework., Methods: We conducted focus groups, using a constructivist grounded theory approach, using physicians and nurses who have experienced a simulated cardiac arrest. The participants were recruited via e-mail. Transcripts were analyzed by coders blinded to the original framework to generate a list of provisional themes that were iteratively refined. These themes were then compared with the themes from the original article and used to derive a consensus model that incorporated the most relevant features of each., Results: Focus group data yielded 7 themes. Six were similar to those developed in the original framework. One important exception was noted; however, those learners not exposed to patient death due to action or inaction often felt that the mannequin's survival was artificial. This additional theme was incorporated into a revised framework., Discussion: The original framework addresses most aspects of learner reactions to simulated death. Our work suggests that adding the theme pertaining to the lack of realism that can be perceived when the mannequin is unexpectedly saved results in a more robust theoretical framework transferable to centers that do not allow mannequin death.

Pediatric cardiac critical care is a new and emerging field. There is no standardization to the current education provided, and high-quality patient outcomes require such standardization. For physicians, this includes fellowship training, specific competencies, and a certification process. For advanced practice providers, a standardized curriculum as well as a certification process is needed. There is evidence that supports a finding that critical care nursing experience may have a positive impact on outcomes from pediatric cardiac surgery. A rigorous orientation and meaningful continuing education may augment that. For all disciplines and levels of expertise, simulation is a useful modality in the education in pediatric cardiac critical care.

Background: We describe the implementation of and outcomes associated with an acuity adaptable care model for pediatric patients undergoing cardiac surgery., Methods: Consecutive patients undergoing an index cardiac operation between July 2007 and June 2015 were included. From July 2007 through June 2010, a conventional model existed in which patients moved among units and care teams based on age, severity of illness, and operative status (conventional group). A transitional period existed between July 2010 and June 8, 2012 (transitional group). From June 9, 2012, through June 2015, an acuity adaptable model was used in which patients remained in the cardiac care unit and received care from the same clinical team throughout their hospitalization (acuity adaptable group)., Results: Included were 2,363 patients: 925 in the conventional group, 520 in the transitional group, and 918 in the acuity adaptable group. In relation to the conventional group, the adjusted odds of operative mortality in the acuity adaptable group was 0.55 (95% confidence interval: 0.26-1.18; P = .12). The failure to rescue rate (ie, number of deaths in patients with any complication divided by the number of total patients with any complication) decreased (conventional group, 8.7%; acuity adaptable group, 4.2%; P = .04). In relation to the conventional group, postoperative hospital length of stay tended to be shorter in the acuity adaptable group ( P = .07)., Conclusions: The implementation of an acuity adaptable care model was feasible in our pediatric cardiac program. The favorable associations identified between the new model and outcomes are promising but warrant confirmation in a larger, multicenter study.

Objective: To create scenarios of simulated decompensating pediatric patients to train pediatric rapid response teams (RRTs) and to determine whether the scenario scores provide a valid assessment of RRT performance with the hypothesis that RRTs led by intensivists-in-training would be better prepared to manage the scenarios than teams led by nurse practitioners., Study Design: A set of 10 simulated scenarios was designed for the training and assessment of pediatric RRTs. Pediatric RRTs, comprising a pediatric intensive care unit (PICU) registered nurse and respiratory therapist, led by a PICU intensivist-in-training or a pediatric nurse practitioner, managed 7 simulated acutely decompensating patients. Two raters evaluated the scenario performances and psychometric analyses of the scenarios were performed., Results: The teams readily managed scenarios such as supraventricular tachycardia and opioid overdose but had difficulty with more complicated scenarios such as aortic coarctation or head injury. The management of any particular scenario was reasonably predictive of overall team performance. The teams led by the PICU intensivists-in-training outperformed the teams led by the pediatric nurse practitioners., Conclusions: Simulation provides a method for RRTs to develop decision-making skills in managing decompensating pediatric patients. The multiple scenario assessment provided a moderately reliable team score. The greater scores achieved by PICU intensivist-in-training-led teams provides some evidence to support the validity of the assessment., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Training guidelines state that pediatricians should be able to diagnose, manage, and triage patients with heart disease. Acutely ill cardiac patients present infrequently and with high acuity, yet residents receive less exposure to acute cardiac conditions than previous generations. Trainees must learn to manage these situations despite this gap. Simulation has been used successfully to train learners to provide acute care. We hypothesized that a simulation-based cardiac curriculum would improve residents' ability to manage cardiac patients., Methods: Pediatric residents completed 4 simulation cases followed by debriefing and a computer presentation reviewing the learning objectives. Subjects returned at 1 month for postintervention cases and again at 4-6 months to measure knowledge retention. Cases were scored by 2 raters using a dichotomous checklist. We used repeated measure ANOVA and effect size to compare groups and intra-class correlation (ICC) to assess inter-rater reliability., Results: Twenty-five participants were enrolled. Scores were low on pretesting but showed significant improvement (P < .05) in all 4 cases. No decay was noted on late testing. Pre-post effect sizes ranged from 1.1 to 2.1, demonstrating meaningful improvement. Inter-rater reliability (ICC) ranged from 0.61 to 0.93 across cases., Conclusions: This novel simulation-based curriculum targets a gap in pediatric training and offers an effective way to train pediatricians. We plan to expand this curriculum to new populations of participants and have integrated it into our resident cardiology rotation., (© 2017 Wiley Periodicals, Inc.)

Objective: We performed a systematic review as part of the International Liaison Committee on Resuscitation process to create a consensus on science statement regarding amiodarone or lidocaine during pediatric cardiac arrest for the 2015 International Liaison Committee on Resuscitation's Consensus on Science and Treatment Recommendations., Data Sources: Studies were identified from comprehensive searches in PubMed, Embase, and the Cochrane Library., Study Selection: Studies eligible for inclusion were randomized controlled and observational studies on the relative clinical effect of amiodarone or lidocaine in cardiac arrest., Data Extraction: Studies addressing the clinical effect of amiodarone versus lidocaine were extracted and reviewed for inclusion and exclusion criteria by the reviewers. Studies were rigorously analyzed thereafter., Data Synthesis: We identified three articles addressing lidocaine versus amiodarone in cardiac arrest: 1) a prospective study assessing lidocaine versus amiodarone for refractory ventricular fibrillation in out-of-hospital adults; 2) an observational retrospective cohort study of inpatient pediatric patients with ventricular fibrillation or pulseless ventricular tachycardia who received lidocaine, amiodarone, neither or both; and 3) a prospective study of ventricular tachycardia with a pulse in adults. The first study showed a statistically significant improvement in survival to hospital admission with amiodarone (22.8% vs 12.0%; p = 0.009) and a lack of statistical difference for survival at discharge (p = 0.34). The second article demonstrated 44% return of spontaneous circulation for amiodarone and 64% for lidocaine (odds ratio, 2.02; 1.36-3.03) with no statistical difference for survival at hospital discharge. The third article demonstrated 48.3% arrhythmia termination for amiodarone versus 10.3% for lidocaine (p < 0.05). All were classified as lower quality studies without preference for one agent., Conclusions: The confidence in effect estimates is so low that International Liaison Committee on Resuscitation felt that a recommendation to use of amiodarone over lidocaine is too speculative; we suggest that amiodarone or lidocaine can be used in the setting of pulseless ventricular tachycardia/ventricular fibrillation in infants and children.

In children with fulminant myocarditis (FM), we sought to describe presenting characteristics and clinical outcomes, and identify risk factors for cardiac arrest and mechanical circulatory support (MCS). A retrospective review of patients with FM admitted at our institution between January 1, 2004, and June 31, 2015, was performed. We compared characteristics and outcomes of FM patients who received cardiopulmonary resuscitation (CPR) and/or were placed on MCS (CPR/MCS group) to those who did not develop these outcomes (Control group). There were 28 patients who met criteria for FM. Median age was 1.2 years (1 day-17 years). Recovery of myocardial function occurred in 13 patients (46%); 6 (21%) had chronic ventricular dysfunction, 6 (21%) underwent heart transplantation, and 3 (11%) died prior to hospital discharge (including one death following heart transplant). Of the 28 FM patients, 13 (46%) developed cardiac arrest (n = 11) and/or received MCS (n = 8). When compared to controls, patients in the CPR/MCS group had a higher peak b-type natriuretic peptide (BNP) levels (p = 0.03) and peak inotropic scores (p = 0.02). No significant differences were found between groups in demographics; chest radiograph, electrocardiogram, or echocardiogram findings; or initial laboratory values including BNP, troponin, C-reactive protein, lactate, and creatinine (p > 0.05 for all). Children with FM are at high risk of cardiovascular collapse leading to the use of CPR or MCS. Aside from peak BNP levels and inotropic scores, the most presenting characteristics were not helpful for predicting these outcomes. FM patients should ideally receive care in centers that provide emergent MCS.

Background: Mental fatigue is impaired cognitive function induced by engaging in cognitively demanding activities. Pediatric intensive care unit (PICU) rounds are demanding and may be a cause of impaired cognitive functioning. The purpose of this study is to evaluate if PICU rounds induce poorer performance on cognitive tasks after rounds compared to before rounds and assess the feasibility of cognitive testing in the PICU., Methods: This was a prospective study of residents in the PICU. Participants were evaluated before and after rounds on a single day, consisting of two tests of cognitive function that are sensitive to mental fatigue, the cognitive estimation test (CET) and the repeatable episodic memory test (REMT)., Results: Thirty residents participated. The mean length of rounds was 191 min (SD 33.8 min), the mean number of patients rounded on by the team was 14.9 (SD 2.3) and the median patients presented by the participant was two (range 0-6). The average number of words recalled on the REMT was significantly lower after rounds compared to before (29.6 vs. 31.2, p < 0.05). There were significantly more falsely recalled words after rounds (1.3 vs. 0.7, p=0.02). There was a correlation between worsening performance and later time of testing in the 4-week PICU rotation (r=0.42, p < 0.02). There were no differences in performance on the CET., Conclusions: PICU rounds induced impairments on cognitive testing but the effect size is small and not consistent across tests. There is an increased susceptibility to impaired cognition induced by rounds over the course of a rotation, this finding merits further investigation.

Objectives: Focusing on critically ill children with cardiac disease, we will review common causes of fluid perturbations, clinical recognition, and strategies to minimize and treat fluid-related complications., Data Source: MEDLINE and PubMed., Conclusions: Meticulous fluid management is vital in critically ill children with cardiac disease. Fluid therapy is important to maintain adequate blood volume and perfusion pressure in order to support cardiac output, tissue perfusion, and oxygen delivery. However, fluid overload and acute kidney injury are common and are associated with increased morbidity and mortality. Understanding the etiologies for disturbances in volume status and the pathophysiology surrounding those conditions is crucial for providing optimal care.

Background: Palliative procedures performed before the Fontan procedure may require ligation of the subclavian arteries, thereby affecting flow to the vertebral arteries. In adults with functionally univentricular heart disease, the implications of altered brainstem vascular anatomy for perioperative management of failing Fontan circulation are not known., Methods: We identified abnormal posterior circulation anatomy in an adult patient with failing Fontan circulation who experienced a brainstem stroke after Fontan conversion. We then changed our clinical practice to include detailed preoperative neurologic evaluation of adults with univentricular heart disease and failing Fontan circulation. Here, we report the clinical and neuroimaging findings in 5 consecutive patients before and after this change in practice., Results: Five patients ages 28 to 42 years had Fontan procedures performed in childhood, and underwent either Fontan conversion or cardiac transplantation. Patient 1 experienced an episode of decreased cerebral perfusion pressure on postoperative day 3, and experienced an ischemic brainstem stroke causing transient locked-in syndrome. A change in practice was made, and patients 2, 3, and 4 were evaluated preoperatively by the neurocritical care service. These patients then had higher target blood pressures perioperatively and no neurologic injury. Patient 5 was evaluated for symptoms consistent with subclavian steal. Neuroimaging in 3 patients was abnormal, with atrophic vertebral arteries, an occluded vertebral artery, and retrograde perfusion of a vertebral artery., Conclusions: In adults with failing Fontan circulation there is a potential for neurologic complications as a result of venous congestion with elevated central venous pressures, and aberrant posterior circulation. The patient's history and brain imaging may be used to identify at-risk patients and to tailor perioperative management during Fontan conversion or heart transplantation to mitigate the risk for brainstem ischemia., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: The aims of the study were (1) to create multiple scenarios that simulate a range of urgent and emergent extracorporeal membrane oxygenation (ECMO) events and (2) to determine whether these scenarios can provide reliable and valid measures of a specialist's advanced skill in managing ECMO emergencies., Design: Multiscenario simulation-based performance assessment was performed., Setting: The study was conducted in the Saigh Pediatric Simulation Center at St. Louis Children's Hospital., Subjects: ECMO clinical specialists participated in the study., Measurements and Main Results: Twenty-five ECMO specialists completed 8 scenarios presenting acute events in simulated ECMO patients. Participants were evaluated by 2 separate reviewers for completion of key actions and for global performance. The scores were highest for the hemodilution scenario, whereas the air entrainment scenario had the lowest scores. Psychometric analysis demonstrated that ECMO specialists with more than 1 year of experience outperformed the specialists with less than 1 year of experience. Participants endorsed these sessions as important and representative of events that might be encountered in practice., Conclusions: The scenarios could serve as a component of an ECMO education curriculum and be used to assess clinical specialists' readiness to manage ECMO emergencies.

The evolution and development of pediatric cardiac critical care as a distinct subspecialty have occurred rapidly over the past 20 years. As the field has grown, models for education, training, and care delivery have changed as well. This review will highlight the current state of education, training, and parental involvement in care delivery for pediatric cardiac critical care as initially., (© The Author(s) 2016.)

Low cardiac output syndrome frequently complicates the post-operative care of infants and children following cardiac surgery. The onset of low cardiac output follows a predictable course in the hours following cardiopulmonary bypass, as myocardial performance declines in the face of an elevated demand for cardiac output. When demand outstrips supply, shock ensues, and early recognition and intervention can decrease mortality. Multifactorial in etiology, this article will discuss the pathophysiology of low cardiac output syndrome, including myocardial depression following bypass, altered cardiac loading conditions, and inflammation driving a hypermetabolic state. Contributions from altered neurohormonal, thyroid, and adrenal axes will also be discussed. Sources included the clinical experiences of four cardiac intensivists, supported throughout by primary sources and relevant reviews obtained through PubMed searches and from seminal textbooks in the field. This article addresses the second of eight topics comprising the special issue entitled "Pharmacologic strategies with afterload reduction in low cardiac output syndrome after pediatric cardiac surgery".

This review offers a critical-care perspective on the pathophysiology, monitoring, and management of acute heart failure syndromes in children. An in-depth understanding of the cardiovascular physiological disturbances in this population of patients is essential to correctly interpret clinical signs, symptoms and monitoring data, and to implement appropriate therapies. In this regard, the myocardial force-velocity relationship, the Frank-Starling mechanism, and pressure-volume loops are discussed. A variety of monitoring modalities are used to provide insight into the haemodynamic state, clinical trajectory, and response to treatment. Critical-care treatment of acute heart failure is based on the fundamental principles of optimising the delivery of oxygen and minimising metabolic demands. The former may be achieved by optimising systemic arterial oxygen content and the variables that determine cardiac output: heart rate and rhythm, preload, afterload, and contractility. Metabolic demands may be decreased by a number of ways including positive pressure ventilation, temperature control, and sedation. Mechanical circulatory support should be considered for refractory cases. In the near future, monitoring modalities may be improved by the capture and analysis of complex clinical data such as pressure waveforms and heart rate variability. Using predictive modelling and streaming analytics, these data may then be used to develop automated, real-time clinical decision support tools. Given the barriers to conducting multi-centre trials in this population of patients, the thoughtful analysis of data from multi-centre clinical registries and administrative databases will also likely have an impact on clinical practice.

Background: Management of status epilepticus (SE) in the pediatric population is highly time-sensitive. Failure to follow a standard management algorithm may be due to ineffective provider education, and can lead to unfavorable outcomes., Objective: To design a learning module using high-fidelity simulation technology to teach mastery achievement of a hospital algorithm for managing SE., Methods: Thirty pediatrics interns were enrolled. Using the Angoff method, an expert panel developed the minimal passing score, which defined mastery. Scoring of simulated performance was done by 2 observers. Sessions were digitally recorded. After the pretest, participants were debriefed on the algorithm and required to repeat the simulation. If mastery (minimal passing score) was not achieved, debriefing and the simulation were repeated until mastery was met. Once mastery was met, participants graded their comfort level in managing SE., Results: No participants achieved mastery at pretest. After debriefing and deliberate simulator training, all (n=30) achieved mastery of the algorithm: 30% achieved mastery after 1 posttest, 63% after a second, and 6.7% after a third. The Krippendorff α was 0.94, indicating strong interrater agreement. Participants reported more self-efficacy in managing SE, a preference for simulation-based education for learning practice-based algorithms of critical conditions, and highly rated the educational intervention., Conclusions: A simulation-based mastery learning program using deliberate practice dramatically improves pediatrics residents' execution of a SE management protocol. Participants enjoyed and benefited from simulation education. Future applications include improving adherence to other hospital protocols.

A generalized anxiety disorder diagnosis must include thorough history-taking, the use of age-appropriate screening tools, and physical assessment. Research and development into the use of screening tools and effectiveness of treatment strategies for generalized anxiety disorder is needed to better manage adolescents with the disorder.

Objective: To assess the cost-effectiveness of sensor-augmented insulin pump therapy with "Low Glucose Suspend" (LGS) functionality versus standard pump therapy with self-monitoring of blood glucose in patients with type 1 diabetes who have impaired awareness of hypoglycemia., Methods: A clinical trial-based economic evaluation was performed in which the net costs and effectiveness of the two treatment modalities were calculated and expressed as an incremental cost-effectiveness ratio (ICER). The clinical outcome of interest for the evaluation was the rate of severe hypoglycemia in each arm of the LGS study. Quality-of-life utility scores were calculated using the three-level EuroQol five-dimensional questionnaire. Resource use costs were estimated using public sources., Results: After 6 months, the use of sensor-augmented insulin pump therapy with LGS significantly reduced the incidence of severe hypoglycemia compared with standard pump therapy (incident rate difference 1.85 [0.17-3.53]; P = 0.037). Based on a primary randomized study, the ICER per severe hypoglycemic event avoided was $18,257 for all patients and $14,944 for those aged 12 years and older. Including all major medical resource costs (e.g., hospital admissions), the ICERs were $17,602 and $14,289, respectively. Over the 6-month period, the cost per quality-adjusted life-year gained was $40,803 for patients aged 12 years and older., Conclusions: Based on the Australian experience evaluating new interventions across a broad range of therapeutic areas, sensor-augmented insulin pump therapy with LGS may be considered a cost-effective alternative to standard pump therapy with self-monitoring of blood glucose in hypoglycemia unaware patients with type 1 diabetes., (Copyright © 2014 International Society for Pharmacoeconomics and Outcomes Research (ISPOR). Published by Elsevier Inc. All rights reserved.)