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Truncus arteriosus is a congenital heart defect with high resource use, cost, and mortality. Value assessment (outcome relative to cost) can improve quality of care and decrease cost. This study hypothesized that truncus arteriosus repair at a high-volume center would result in better outcomes at lower cost (higher value) compared with a low-volume center.This study retrospectively analyzed a multicenter cohort of neonates undergoing truncus arteriosus repair (2004 to 2015) by using the Pediatric Health Information Systems database. Multivariate quantile, logistic, and negative binomial regression models were used to evaluate total hospital cost, in-hospital mortality, ventilation days, intensive care unit length of stay (LOS), hospital LOS, and days of inotropic agent use by center volume (high-volume3/year) and age at repair while adjusting for sex, ethnicity, race, genetic abnormality, prematurity, low birth weight, concurrent interrupted arch repair, and truncal valve repair.Of 1024 neonates with truncus arteriosus, 495 (48%) were treated at high-volume centers. Costs at the 75th percentile were lower at high-volume vs low-volume centers by $28,456 (P = .02) at all ages at repair. Patients at high-volume centers had lower median postoperative ventilation days (5 days vs 6 days; P.001), intensive care unit LOS (13 days vs 19 days; P.001), hospital LOS (23 days vs 28 days; P = .02), and inotropic agent use (3 days vs 4 days; P = .004). In-hospital mortality did not differ by center volume.In neonates undergoing truncus arteriosus repair, costs are lower and outcomes are better at high-volume centers, thus resulting in higher value at all ages of repair. Value-based interventions should be considered to improve outcomes and decrease cost in truncus arteriosus care.

Background: Surgery is the mainstay of treatment for gastric volvulus. Despite its rarity, early experience from recent publications suggests that laparoscopy is a safe and effective approach for t...

OBJECTIVES We leveraged decomposition analysis, commonly used in labor economics, to understand determinants of cost differences related to location of admission in children undergoing neonatal congenital heart surgery. DESIGN A retrospective cohort study. SETTING Pediatric Health Information Systems database. PATIENTS Neonates (

Objective:To assess current demographics and duties of physicians as well as the structure of paediatric cardiac critical care in the United States.Design:REDCap surveys were sent by email from May till August 2019 to medical directors (“directors”) of critical care units at the 120 United States centres submitting data to the Society of Thoracic Surgeons Congenital Heart Surgery Database and to associated faculty from centres that provided email lists. Faculty and directors were asked about personal attributes and clinical duties. Directors were additionally asked about unit structure.Measurements and main results:Responses were received from 66% (79/120) of directors and 62% (294/477) of contacted faculty. Seventy-six percent of directors and 54% of faculty were male, however, faculty Conclusions:Racial and ethnic diversity is markedly deficient in the paediatric cardiac critical care workforce. Although the majority of faculty are male, females make up the majority of the workforce younger than 40 years old. Work hours across all age groups and unit types are high both on- and off-service, with most units providing attending in-house night coverage.

Non-small-cell lung cancer (NSCLC) makes up 80–85% of lung cancer diagnoses. Lung cancer patients undergo surgical procedures, chemotherapy, and/or radiation. Chemotherapy and radiation can induce deleterious systemic side effects, particularly within skeletal muscle. To determine whether metformin reduces NSCLC tumor burden while maintaining skeletal muscle health, C57BL/6J mice were injected with Lewis lung cancer (LL/2), containing a bioluminescent reporter for in vivo tracking, into the left lung. Control and metformin (250 mg/kg) groups received treatments twice weekly. Skeletal muscle was analyzed for changes in genes and proteins related to inflammation, muscle mass, and metabolism. The LL/2 model effectively mimics lung cancer growth and tumor burden. The in vivo data indicate that metformin as administered was not associated with significant improvement in tumor burden in this immunocompetent NSCLC model. Additionally, metformin was not associated with significant changes in key tumor cell division and inflammation markers, or improved skeletal muscle health. Metformin treatment, while exhibiting anti-neoplastic characteristics in many cancers, appears not to be an appropriate monotherapy for NSCLC tumor growth in vivo. Future studies should pursue co-treatment modalities, with metformin as a potentially supportive drug rather than a monotherapy to mitigate cancer progression.

The autoregulation of cerebral blood flow protects against brain injury from transient fluctuations in arterial blood pressure. Impaired autoregulation may contribute to hypoperfusion injury in neonates and infants. Monitoring cerebral autoregulation in neonatal cardiac surgery as a guide for arterial blood pressure management may reduce neurodevelopmental morbidity. Cerebral autoregulation monitoring has been validated in animal models and in an adult trial autoregulation monitoring during bypass improved postoperative delirium scores. The nuances of pediatric cardiac disease and congenital heart surgery make simply applying adult trial findings to this unique population inappropriate. Therefore, dedicated pediatric clinical trials of cerebral autoregulation monitoring are indicated.

The congenital heart care community faces a myriad of public health issues that act as barriers toward optimum patient outcomes. In this article, we attempt to define advocacy and policy initiatives meant to spotlight and potentially address these challenges. Issues are organized into the following 3 key facets of our community: patient population, health care delivery, and workforce. We discuss the social determinants of health and health care disparities that affect patients in the community that require the attention of policy makers. Furthermore, we highlight the many needs of the growing adults with congenital heart disease and those with comorbidities, highlighting concerns regarding the inequities in access to cardiac care and the need for multidisciplinary care. We also recognize the problems of transparency in outcomes reporting and the promising application of telehealth. Finally, we highlight the training of providers, measures of productivity, diversity in the workforce, and the importance of patient–family centered organizations in advocating for patients. Although all of these issues remain relevant to many subspecialties in medicine, this article attempts to illustrate the unique needs of this population and highlight ways in which to work together to address important opportunities for change in the cardiac care community and beyond. This article provides a framework for policy and advocacy efforts for the next decade.

To outline key elements in providing compassionate end-of-life care for pediatric patients with end-stage heart failure on mechanical circulatory support (MCS) such as extracorporeal membrane oxygenation (ECMO) and ventricular assist devices (VADs). Approximately one in five pediatric patients with end-stage heart failure die on VAD support, with higher mortality associated with patients supported on ECMO. While it is considered ethically and legally permissible to withdraw MCS, pediatric-specific guidelines regarding indications and end-of-life management on MCS are lacking. A multidisciplinary approach with early consultation of the palliative care team is essential for all patients being considered for MCS. Guiding families through end-of-life care on MCS requires frequent and honest communication by engaging in shared decision-making regarding prognosis and potential clinical pathways, advanced care and preparedness planning, and identifying and meeting patients’/families’ goals of care.

Mental disorders are the predominant chronic diseases of youth, with substantial life span morbidity and mortality. A wealth of evidence demonstrates that the neurodevelopmental roots of common mental health problems are present in early childhood. Unfortunately, this has not been translated to systematic strategies for improving population-level mental health at this most malleable neurodevelopmental period. We lay out a translational Mental Health, Earlier road map as a key future direction for prevention of mental disorder. This paradigm shift aims to reduce population attributable risk of mental disorder emanating from early life, by preventing, attenuating, or delaying onset/course of chronic psychopathology via the promotion of self-regulation in early childhood within large-scale health care delivery systems. The Earlier Pillar rests on a "science of when to worry" that (a) optimizes clinical assessment methods for characterizing probabilistic clinical risk beginning in infancy via deliberate incorporation of neurodevelopmental heterogeneity, and (b) universal primary-care-based screening targeting patterns of dysregulated irritability as a robust transdiagnostic marker of vulnerability to life span mental health problems. The core of the Healthier Pillar is provision of low-intensity selective intervention promoting self-regulation for young children with developmentally atypical patterns of irritability within an implementation science framework in pediatric primary care to ensure highest population impact and sustainability. These Mental Health, Earlier strategies hold much promise for transforming clinical outlooks and ensuring young children's mental health and well-being in a manner that reverberates throughout the life span.

Context: Professional football linemen are at risk for sleep-disordered breathing (SDB) compared with other types of athletes. It is currently unknown whether college football linemen display a similar risk profile. Objective: (1) To determine for the first time whether college football linemen show risk for SDB and (2) test the hypothesis that SDB risk is higher in college football linemen compared with an athletic comparison group. Design: Descriptive laboratory study. Setting: The Health Risk Assessment Laboratory. Participants: Male football linemen (n = 21) and track (n = 19) Division I athletes between the ages of 18 and 22 years. Interventions: Participants completed the Multivariable Apnea Prediction Index and Epworth Sleepiness Scale surveys, validated measures of symptoms of sleep apnea and daytime sleepiness, respectively. Neck and waist circumferences, blood pressure, Modified Mallampati Index (MMPI), and tonsil size were determined, followed by body composition assessment using dual-energy X-ray absorptiometry. Main Outcome Measures: Scores from surveys, anthropometric data, MMPI, and body composition. Results: Survey data demonstrated a deficiency in sleep quality and efficiency, coinciding with increased self-reported symptoms of apnea (Multivariable Apnea Prediction Index = 0.78) in college linemen relative to track athletes. Neck circumference (44.36 cm), waist circumference (107.07 cm), body mass index (35.87 kg/m2), and percent body fat (29.20%), all of which exceeded the clinical predictors of risk for obstructive sleep apnea, were significantly greater in linemen compared with track athletes. Multivariable Apnea Prediction variables were significantly correlated with MMPI, neck circumference, percent body fat, body mass index, and systolic blood pressure (r ≥ .31, P Conclusions: Risk factors for SDB recognized in professional football linemen are also present at the college level. Screening may minimize present or future risk for SDB, as well as the downstream risk of SDB-associated metabolic and cardiovascular disease.

Neurodevelopmental and psychosocial impairments negatively impact health-related quality of life for survivors with CHD and complicate the transition to independent adulthood. Risk for neurodevelopmental and psychosocial impairments is influenced by a complex interplay among genetic, foetal, surgical, perioperative, family, and social factors, requiring a multi-pronged approach to neuroprotection and intervention. To ensure future research can ultimately reduce the burden of CHD for individuals, families, and society, the most pressing issues in cardiac neurodevelopment requiring scientific investigation must be identified.Through funding from an R13 Grant from the National Heart, Lung, and Blood Institute of the National Institutes of Health of the United States of America, the Cardiac Neurodevelopmental Outcome Collaborative convened a two-day meeting of international experts in cardiac neurodevelopmental and psychosocial research, clinical care, and health disparities, including patient and family stakeholders, to define the cardiac neurodevelopmental and psychosocial outcomes research agenda for the next decade. Seven multidisciplinary working groups were formed to address key domains crucial to the advancement of cardiac neurodevelopmental and psychosocial outcomes research: 1) Foetal Brain Development and Neuroprotection, 2) Surgical/Perioperative Neuroprotection and Neurodevelopment, 3) Characterization of Neurodevelopmental and Psychological Outcomes, 4) Neurodevelopmental and Psychosocial Intervention, 5) Parent Mental Health and Family Functioning, 6) Neurodevelopmental Education, Outreach and Advocacy, and 7) Health Disparities and Neurodevelopmental Outcomes. Working groups identified significant gaps in knowledge and critical questions that must be answered to further knowledge, policy, care, and outcomes. The development of a research agenda in cardiac neurodevelopmental and psychosocial outcomes is critical for informing collaborative initiatives and allocation of funding for research to scientific inquiries of highest value to key stakeholders.

Diagnosis of CHD substantially affects parent mental health and family functioning, thereby influencing child neurodevelopmental and psychosocial outcomes. Recognition of the need to proactively support parent mental health and family functioning following cardiac diagnosis to promote psychosocial adaptation has increased substantially over recent years. However, significant gaps in knowledge remain and families continue to report critical unmet psychosocial needs. TheParent Mental Health and Family Functioning Working Groupof the Cardiac Neurodevelopmental Outcome Collaborative was formed in 2018 through support from an R13 grant from the National Heart, Lung, and Blood Institute to identify significant knowledge gaps related to parent mental health and family functioning, as well as critical questions that must be answered to further knowledge, policy, care, and outcomes. Conceptually driven investigations are needed to identify parent mental health and family functioning factors with the strongest influence on child outcomes, to obtain a deeper understanding of the biomarkers associated with these factors, and to better understand how parent mental health and family functioning influence child outcomes over time. Investigations are also needed to develop, test, and implement sustainable models of mental health screening and assessment, as well as effective interventions to optimise parent mental health and family functioning to promote psychosocial adaptation. The critical questions and investigations outlined in this paper provide a roadmap for future research to close gaps in knowledge, improve care, and promote positive outcomes for families of children with CHD.

Acute kidney injury (AKI) is a common complication following single ventricle congenital heart surgery. Data regarding AKI following Fontan conversion (FC) surgery are limited. This study evaluated the incidence, predictors of, and prognostic value of AKI following FC. Single-center retrospective cohort study, including consecutive FC patients from December 1994 to December 2016. Medical records were reviewed. AKI was classified into AKI-1/AKI-2/AKI-3 using Kidney Disease: Improving Global Outcomes criteria. Multivariable logistic regression identified risk factors for AKI≥2. Chi-square and 2-sample t-tests assessed associations between AKI≥2 and postoperative outcomes. Mid-term heart-transplant-free survival among AKI0-1 vs AKI2-3 groups was compared using Kaplan-Meier curves and log-rank test. We included 139 FC patients: age at FC 24 (25th-75th, 19-31) years; 81% initial atrio-pulmonary Fontan; follow-up 8.3 ± 5.3 years following FC. Post-FC, 63 patients (45%) developed AKI (AKI-1 = 37 [27%]; AKI-2 = 10 [7%]; AKI-3 = 16 [11%]). AKI recovered by hospital discharge in 86%, 80%, and 19% of patients with AKI-1/AKI-2/AKI-3, respectively. Independent risk factors for AKI≥2 included older age (OR 1.07, 95%CI 1.01-1.15; P = 0.027); ≥3 prior sternotomies (OR = 6.11; 95%CI = 1.59-23.47; P = 0.009); greater preoperative right atrial pressure (OR 1.19; 1.02-1.38; P = 0.024), and prior catheter ablation procedure (OR 3.45; 1.17-10.18; P = 0.036). AKI≥2 was associated with: longer chest tube duration (9 [5-57] vs 7 [3-28] days; P = 0.01); longer mechanical ventilation time (2 [1-117] vs 1 [1-6] days; P = 0.01); greater need for dialysis (31% v s0%; P0.001); and longer postoperative length of stay (18 [8-135] vs 10 [6-58] days; P0.001). AKI 2-3 patients had worse mid-term heart-transplant-free survival. Half of the patients undergoing FC develop AKI. AKI 2-3 is associated with worse early postoperative outcomes and reduced mid-term transplant-free survival following FC. Knowledge of AKI predictors may allow for improved FC risk stratification, patient selection, and perioperative management in this high-risk population.

Introduction: Comparisons of care among centers is currently limited to major endpoints, such as mortality, length of stay, or complication rates. Hypothesis: We hypothesized that comparing individual elements of intensive care over time using classical statistics and “care curves” may highlight important modifiable differences among centers. Methods: We retrospectively reviewed data of key elements of postoperative care after stage-1-palliation (S1P) among 5 US centers during 2009-2018. Minimum mandatory ventilation rate (MMVR), opioid total daily dose (TDD) and fluid balance (FB) were computed from postoperative day (POD) 0 to 28. Cox regression, mixed random effect models and growth chart-like care curves were used to analyze data variability and trajectory over time. Results: During the study period, 502 patients underwent S1P (median age 5 [IQR 3-6] days). Patients were weaned from MMV by POD 8 (IQR 5-16), with significant differences among centers (P Conclusions: Both “care curves” and classical statistics highlighted important modifiable differences in the elements of care among centers. MMVR, opioid TDD, and FB following S1P significantly differ even after adjusting for patient and operative factors. Care curves may be used to guide collaborative quality improvement initiatives in the intensive care unit.

Introduction: Poor physician wellness is associated with detrimental effects on patient outcomes. There are no data on physician wellness in pediatric cardiac critical care. We aimed to define the prevalence of and risk factors for impaired wellness using a targeted survey of the North American pediatric cardiac critical care workforce. Methods: We developed a survey to examine physician wellness and potential risk factors, incorporating the Expanded Physician Well Being Index. Unit directors at 120 congenital heart surgery centers were contacted to provide faculty emails. The survey was distributed to each faculty with targeted reminders from June - September 2019. Univariate and multivariable logistic regression analyses were performed comparing the at-risk respondents to those not at risk for poor wellness. Results: Of the 477 faculty, 294 completed the survey (62%). Faculty reported working a median of 83 hours on service weeks and 50 hours on non-service weeks. Based on Well-Being scores, 34% were in the “at risk” category; 56% reported feeling burned out in the past month and 46% reported feeling overwhelmed. However, 96% felt their work was meaningful. Faculty reported a high incidence of developing unhealthy habits including poor nutrition (50%), infrequent exercise (56%), and lack of self-care (46%). Factors associated with well-being risk are shown in Table 1. Poor interpersonal experiences, higher work hours, and being primarily trained in critical care with an additional cardiac year were associated with risk of poor wellness. Working >100 hours per service week had a significant increase in risk (odds ratio 2.3, 95% confidence interval 1.31-4.40). Availability of wellness services was associated with reduced risk. Conclusion: Burnout and risk of poor wellness are common in pediatric cardiac critical care faculty. Modifiable factors, including wellness service availability and reduced hours of clinical service may improve wellness in the workforce.

Introduction: The safety of vigorous exercise for individuals with appropriately-treated LQTS remains unproven, and physical activity practices in this population have not been described. Methods: LIVE-LQTS prospectively enrolled individuals age 8-60 years with overt LQTS or gene mutation carriers. Participants (or parents, for children) answered surveys describing activity patterns. Vigorous exercise was defined as > 6 METS for > 60 hours per year. Clinical and demographic data were derived from medical record review. Results: Among 1554 participants, 804 (52%) reported exercising vigorously, 473 of these competitively, 577 (37%) exercising at moderate, and 173 (11%) at low levels. Seven percent of those working describe jobs more active than walking, including 10 in protective services and 19 in sports/coaching. Over 90% are genotype positive, 57% are phenotype positive at rest and an additional 16% have exercise-induced QT prolongation. Forty four percent have had documented ventricular arrhythmias or syncope. Eighty-four percent are appropriately treated with either beta-blockers, ICD, and/or sympathectomy, although among beta-blocked patients, only 60% are on the most effective agents. Among adults, BMI was significantly lower among the vigorous exercisers. Conclusion: While the high percentage of vigorous exercisers seen in this study may not represent all patients with LQTS due to self-selection for study participation, many patients with LQTS are engaged in vigorous exercise or competitive athletics. Prospective follow up of this cohort is ongoing and will determine safety of vigorous exercise through comparison of arrhythmic outcomes in vigorous exercisers vs moderate/low level exercisers.

Among children with congenital heart disease (CHD), there is often neurodevelopmental and behavioral impairment with unclear implications regarding adult socioeconomic achievements. We aimed to compare economic self-sufficiency and educational attainment in CHD adults with sibling and general population controls. Using Danish population-based nationwide registries, this cohort study aimed to include all CHD subjects greater than 13 years born between 1963 and 1993. Comparison cohorts included: (1) sibling cohort and (2) general population cohort matched 10:1 on birth year and gender. We computed cumulative incidences of time to first full year of economic self-sufficiency, as well as educational attainment. We assessed the relative probability of self-sufficiency in all cohorts before 30 years of age, defined by Statistics Denmark federal standard. In total, we identified 7,019 CHD subjects, 6,257 full siblings, and 68,805 general population controls. The cumulative incidence of self-sufficiency by age 20 and 35 years for CHD subjects (49% and 84%, respectively) was lower than sibling (68% and 96%) and general population cohorts (67% and 95%). The relative probability of self-sufficiency for CHD subjects compared with siblings was 0.44 (95% confidence interval 0.39 to 0.49). By age 30, adults with CHD were less likely than their siblings to attain all levels of education. Among those achieving higher educational milestones, differences in self-sufficiency between cohorts were absent by age 35. In conclusion, CHD is associated with reduced adult economic self-sufficiency, and the relation between educational level attained and self-sufficiency may suggest that targeted interventions have the potential to improve adult self-sufficiency.

Over the last two decades, heart centres have developed strategies to meet the neurodevelopmental needs of children with congenital heart disease. Since the publication of guidelines in 2012, cardiac neurodevelopmental follow-up programmes have become more widespread. Local neurodevelopmental programmes, however, have been developed independently in widely varying environments. We sought to characterise variation in structure and personnel in cardiac neurodevelopmental programmes. A 31-item survey was sent to all member institutions of the Cardiac Neurodevelopmental Outcome Collaborative. Multidisciplinary teams at each centre completed the survey. Responses were compiled in a descriptive fashion. Of the 29 invited centres, 23 responded to the survey (79%). Centres reported more anticipated neurodevelopment visits between birth and 5 years of age (median 5, range 2–8) than 5–18 years (median 2, range 0–10) with 53% of centres lacking any standard for routine neurodevelopment evaluations after 5 years of age. Estimated annual neurodevelopment clinic volume ranged from 85 to 428 visits with a median of 16% of visits involving children >5 years of age. Among responding centres, the Bayley Scales of Infant and Toddler Development and Wechsler Preschool and Primary Scale of Intelligence were the most routinely used tests. Neonatal clinical assessment was more common (64%) than routine neonatal brain imaging (23%) during hospitalisation. In response to clinical need and published guidelines, centres have established formal cardiac neurodevelopment follow-up programmes. Centres vary considerably in their approaches to routine screening and objective testing, with many centres currently focussing their resources on evaluating younger patients.

OBJECTIVES: To test the effect of a 4-month telehealth home monitoring program (REACH), layered on usual care, on postdischarge outcomes in parents of infants recovering from cardiac surgery and their infants. METHODS: Randomized trial of infants discharged from the hospital after cardiac surgery for congenital heart disease. Consecutive infants with complex congenital heart disease undergoing cardiac surgery within 21 days of life were enrolled at 3 university-affiliated pediatric cardiac centers. RESULTS: From 2012 to 2016, 219 parent-infant dyads were enrolled; 109 were randomly assigned to the intervention group and 110 to the control group. At 4 months postdischarge, parenting stress was not significantly different between groups (total Parenting Stress Index in the intervention group was 220 and in the control group was 215; P = .61). The percentages of parents who met posttraumatic stress disorder (PTSD) criteria and parent quality of life inventory scores were also not significantly different between the 2 groups (PTSD in the intervention group was 18% and was 18% in the control group; P =.56; the mean Ulm Quality of Life Inventory for Parents in the intervention group was 71 andwas 70 in the control group; P = .88). Infant growth in both groups was suboptimal (the mean weight-for-age z scores were −1.1 in the intervention group and −1.2 in the control group; P = .56), and more infants in the intervention group were readmitted to the hospital (66% in the intervention group versus 57% in the control group; P < .001). CONCLUSIONS: When added to usual care, the REACH intervention was not associated with an improvement in parent or infant outcomes. Four months after neonatal heart surgery, ∼20% of parents demonstrate PTSD symptoms. Suboptimal infant growth and hospital readmissions were common.

This study aimed at assessing the long-term oncological outcomes of intracorporeal ileocolic anastomosis (ICA) for laparoscopic right hemicolectomy for colon cancer compared with extracorporeal anastomosis (ECA).We performed a retrospective analysis of 149 consecutive patients who underwent laparoscopic right hemicolectomy for colon cancer between January 2006 and December 2012.Eighty and 69 patients underwent intracorporeal and ECA, respectively. The two groups were demographically comparable. ICA exhibited a significantly shorter operative time (Intracorporeal ICA can reduce operative time and is associated with similar postoperative and long-term oncological outcomes compared to the ECA technique.

Background Optimal surgical approach for repair of coarctation of the aorta (CoA) remains controversial. This study aimed to evaluate reintervention rates and its predictors by using a strategy of resection with extended end-to-end anastomosis (REEEA) through left thoracotomy. Methods A retrospective analysis was performed for all patients who underwent isolated CoA repair or simultaneous repair of CoA and ventricular septal defect repair by REEEA between January 2000 and December 2015 at Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, Illinois. Patients with complex congenital heart disease were excluded. Transverse arch hypoplasia was defined as echocardiographic z-score lower than −2 or by documentation in medical or operative reports. Reintervention was defined as the need for balloon angioplasty or reoperation. Hypertension was defined as antihypertensive medication use or blood pressure greater than or equal to the 95th percentile. Results A total of 251 patients with median age at repair of 14.6 days met inclusion criteria. Repair was by left thoracotomy in 226 (90%). Follow-up data were available for 186 of 251 patients, with median follow-up time of 5.4 years (range, 0.2 to 15.3 years); 169 (91%) of these patients underwent thoracotomy. There were no early deaths or early reoperations. A proximal transverse arch z-score lower than −4.1 or a distal transverse arch z-score of less than −2.8 was predictive of repair through sternotomy. Only 4 (2%) patients required reintervention (2 patients had balloon angioplasties, 2 had reoperations). Transverse arch hypoplasia was a risk factor for reintervention (p = 0.048), but surgical approach was not (p = 0.35). Late hypertension was identified in only 33 of 186 (18%) patients. Conclusions Repair of CoA, even with associated transverse arch hypoplasia, by REEEA through left thoracotomy has a low mortality, low reintervention rate, and low incidence of late hypertension.

Background:This is a cross-sectional study aiming to understand the early characteristics and background of bone health impairment in clinically well children with Fontan circulation.Methods:We enrolled 10 clinically well children with Fontan palliation (operated >5 years before study entrance, Tanner stage ≤3, age 12.1 ± 1.77 years, 7 males) and 11 healthy controls (age 12.0 ± 1.45 years, 9 males) at two children’s hospitals. All patients underwent peripheral quantitative CT. For the Fontan group, we obtained clinical characteristics, NYHA class, cardiac index by MRI, dual x-ray absorptiometry, and biochemical studies. Linear regression was used to compare radius and tibia peripheral quantitative CT measures between Fontan patients and controls.Results:All Fontan patients were clinically well (NYHA class 1 or 2, cardiac index 4.85 ± 1.51 L/min/m2) and without significant comorbidities. Adjusted trabecular bone mineral density, cortical thickness, and bone strength index at the radius were significantly decreased in Fontan patients compared to controls with mean differences −30.13 mg/cm3 (p = 0.041), −0.31 mm (p = 0.043), and −6.65 mg2/mm4 (p = 0.036), respectively. No differences were found for tibial measures. In Fontan patients, the mean height-adjusted lumbar bone mineral density and total body less head z scores were −0.46 ± 1.1 and −0.63 ± 1.1, respectively, which are below the average, but within normal range for age and sex.Conclusions:In a clinically well Fontan cohort, we found significant bone deficits by peripheral quantitative CT in the radius but not the tibia, suggesting non-weight-bearing bones may be more vulnerable to the unique haemodynamics of the Fontan circulation.

Due to improvements in care for patients with congenital heart disease (CHD) over the last 3 decades, early operative mortality for paediatric cardiac surgery is now very low: the raw 30-day mortality rate from registry data is 2–4%. This article provides ten things you need to know about long-term outcomes after paediatric cardiac surgery.

Approximately 32,000 infants are born with CHDs each year in the United States of America. Of every 1000 live births, 2.3 require surgical or transcatheter intervention in the first year of life. There are few more stressful times for parents than when their neonate receives a diagnosis of complex CHD requiring surgery. The stress of caring for these infants is often unrelenting and may last for weeks, months, and often years, placing parents at risk for developing post-traumatic stress disorder, as well as a drastic decrease in quality of life. Anxiety often peaks in the days and weeks after discharge from the hospital as families no longer have immediate access to nursing and medical staff. The purpose of this paper is to describe the methods of a randomised controlled trial that was designed to determine whether REACH would favourably affect parental and infant outcomes by decreasing parental stress, improve parental quality of life, increase infant stability, and decrease resource utilisation in infants with complex CHD.

Background Chronic stress plays a critical role in many of today's diseases and causes of death. Tobacco use reliably increases the likelihood of chronic disease development and premature death. In addition, habitual tobacco use elevates risk of chronic inflammatory diseases, and glucocorticoid therapy is often less effective in smokers compared with nonsmokers. Taken together, smokers may develop glucocorticoid insensitivity, thereby removing the body's greatest anti-inflammatory mechanism. Purpose The purpose of this study was to examine glucocorticoid sensitivity among 24 smokers and 24 age-, sex-, and body mass index-matched never smokers who were clinically healthy individuals (i.e., no diagnosis or medication use for chronic diseases and normotensive). Method Participants visited the lab after a 12 hr fast, provided a blood sample, and completed a series of psychosocial questionnaires. Smokers continued smoking ad libitum before the lab visit. Group differences in glucocorticoid sensitivity were examined using ANCOVA and repeated with linear mixed model to account for possible dependence among immune outcomes that matching participants on age, sex, and body mass index may have introduced. Results Prior to clinical disease onset, smokers' peripheral blood mononuclear cells (PBMCs) exhibited reduced glucocorticoid sensitivity as well as a diminished inflammatory response to lipopolysaccharide compared with never smokers' PBMCs; results were identical regardless of statistical modeling used. Conclusions Cigarette smoking, a self-initiated pharmacological chronic stressor, may provide a unique opportunity to examine early wear and tear on physiological functioning that may lead to chronic disease development. Additional research into PBMCs' intracellular changes must be examined as well as repeating this study in a larger, more heterogeneous population.

Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associate...

Objectives The postsurgical care of children with congenital heart disease may be complicated by the need for cardiorespiratory support, including tracheostomy. The variation of the use of tracheostomy across multiple pediatric cardiac surgical centers has not been defined. We describe multicenter variation in the use of tracheostomy in children undergoing congenital heart surgery. Design We retrospectively analyzed a multicenter cohort. Setting Pediatric Health Information Systems database retrospective cohort. Patients Children less than 18 years who underwent both tracheostomy and cardiac surgery (1/04-6/14). Interventions Univariate and multivariate statistics were performed, stratifying by high (≥ 75th percentile) and low (≤ 25th percentile) tracheostomy volume and adjusting for patient characteristics in multivariate models. Measurements and main results Out of 123,510 hospitalizations involving cardiac surgery, 1,292 tracheostomies (1.2%) were performed (46 hospitals). The rate of tracheostomy placement ranged from 0.3% to 2.5% with no difference in the rate of tracheostomy placement between high and low tracheostomy use centers (p = 0.8). The median time to tracheostomy was 63 days (interquartile range, 36-100), and there was no difference between high- and low-tracheostomy centers. High-tracheostomy centers had $420,000 lower hospital charges than low-volume centers (p = 0.03). Tracheostomy day greater than the median (63 d), Risk Adjustment for Congenital Heart Surgery-1 score 6, and extracorporeal membrane oxygenation were significantly associated with adjusted increased odds of mortality. Later hospital day of tracheostomy was associated with a $13,000/d increase in total hospital charges (p Conclusions Variation in the usage of tracheostomy in infants and children undergoing congenital heart surgery exists across the country. High-tracheostomy centers had lower hospital charges. Late tracheostomy placement, higher congenital heart disease surgical risk, and extracorporeal membrane oxygenation use are independent predictors of in-hospital mortality in this population.

ObjectiveNorwood palliation for patients with single ventricle heart disease is associated with a significant risk for acute kidney injury, which portends a worse prognosis. We sought to investigate the impact of hybrid stage I palliation (Hybrid) on acute kidney injury risk.DesignThis study is a single-centre prospective case–control study of seven consecutive neonates with single ventricle undergoing Hybrid palliation. Levels of serum creatinine and four novel urinary biomarkers, namely neutrophil gelatinase-associated lipocalin, interleukin-18, liver fatty acid-binding protein, and kidney injury molecule-1, were obtained before and after palliation. Acute kidney injury was defined as a ⩾50% increase in serum creatinine within 48 hours after the procedure. Data were compared with a contemporary cohort of 12 neonates with single ventricle who underwent Norwood palliation.ResultsPatients who underwent Hybrid were more likely to be high-risk candidates (86 versus 25%, p=0.01) compared with those who underwent Norwood. Despite similar preoperative serum creatinine levels, there was a trend towards higher levels of postoperative peak serum creatinine (0.7 [0.63, 0.94] versus 0.56 [0.47, 0.74], p=0.06) and rate of acute kidney injury (67 versus 29%, p=0.17) in the Norwood cohort. Preoperative neutrophil gelatinase-associated lipocalin (58.4 [11, 86.3] versus 6.3 [5, 16.2], p=0.07) and interleukin-18 (30.6 [9.6, 167.2] versus 6.3 [6.3, 16.4], p=0.03) levels were higher in the Hybrid cohort. Nevertheless, longitudinal mixed-effect models demonstrated Hybrid palliation to be a protective factor against increased postoperative levels of neutrophil gelatinase-associated lipocalin (estimate −1.8 [−3.0, −9.0], pConclusionsIn this single-centre case–control study, postoperative acute kidney injury risk did not differ significantly by single ventricle stage I treatment strategy; however, postoperative elevation in novel urinary biomarkers, consistent with subclinical kidney injury, was encountered in the Norwood cohort but not in the Hybrid cohort.

Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The overarching aim is to provide insight into the integrative pathophysiology that governs the Fontan circulation and stimulate thoughtful approaches to advance research.

Objective Exercise intolerance afflicts Fontan patients with total cavopulmonary connections (TCPCs) causing a reduction in quality of life. Optimising TCPC design is hypothesised to have a beneficial effect on exercise capacity. This study investigates relationships between TCPC geometries and exercise haemodynamics and performance. Methods This study included 47 patients who completed metabolic exercise stress test with cardiac magnetic resonance (CMR). Phase-contrast CMR images were acquired immediately following supine lower limb exercise. Both anatomies and exercise vessel flow rates at ventilatory anaerobic threshold (VAT) were extracted. The vascular modelling toolkits were used to analyse TCPC geometries. Computational simulations were performed to quantify TCPC indexed power loss (iPL) at VAT. Results A highly significant inverse correlation was found between the TCPC diameter index, which factors in the narrowing of TCPC vessels, with iPL at VAT (r=−0.723, p 2 ) at VAT (r=0.373, p=0.01), VO 2 at peak exercise (r=0.485, p=0.001) and work at VAT/weight (r=0.368, p=0.01). iPL at VAT was negatively correlated with VO 2 at VAT (r=−0.337, p=0.02), VO 2 at peak exercise (r=−0.394, p=0.007) and work at VAT/weight (r=−0.208, p=0.17). Conclusions Eliminating vessel narrowing in TCPCs and reducing elevated iPL at VAT could enhance exercise tolerance for patients with TCPCs. These findings could help plan surgical or catheter-based strategies to improve patients’ exercise capacity.

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.

Purpose Isometric handgrip (IHG) training lowers systolic and diastolic blood pressure (SBP and DBP, respectively), but the efficacy of IHG training in cardiopulmonary rehabilitation patients is unknown. The purpose of this study was to determine if IHG decreases blood pressure in cardiopulmonary rehabilitation patients. Methods Cardiopulmonary rehabilitation program participants (n = 11; 50-80 yr old) were randomized to IHG (n = 6) or control (CON; no treatment; n = 5) groups. IHG participants completed an IHG training program at 30% maximal voluntary contraction, 3 d/wk for 6 wk. Resting SBP, DBP, and heart rate were assessed weekly. Results Mean regression for SBP following IHG was negative (-1.04 ± 0.80). Mean regression in the CON group was positive (0.50 ± 0.88), but there was no significant difference between groups. Separate analysis of weeks 1 to 7 yielded a negative mean regression (-1.12 ± 0.54) in the IHG group, but positive (1.2 ± 0.60) in the CON group. A Wilcoxon test of these differences yielded significance for SBP (P = .009). In 3 of 6 IHG participants, SBP was lower (mean ± SD: -16 ± 11 mm Hg; P = .12), and in 2 IHG participants, DBP was lower (-9 ± 1 mm Hg; P = .06) compared with baseline. In 2 of 5 CON participants, SBP was not significantly lower (-11 ± 7 mm Hg) and, in 3 of 5 CON participants, DBP was lower (-7 ± 4 mm Hg; P = .04). Conclusions Our data suggest that standard IHG training may be inadequate for blood pressure management immediately following a major cardiac or pulmonary event. Future work with a larger cohort and more developed training protocol to determine the efficacy of IHG training in patients with cardiopulmonary disease is warranted.

As survivable outcomes among patients with complex congenital heart disease (cCHD) have continued to improve over the last several decades, more attention is being dedicated to interventions that impact not just survival but quality of life among patients with cCHD. In particular, patients with cCHD are at risk for impaired neurodevelopmental outcomes. In this review summarizing select presentations given at the 14th Annual Pediatric Cardiac Intensive Care Society’s Annual Meeting in 2019, we discuss the neurodevelopmental phenotype of patients with cCHD, patients at greatest risk of impaired development, and three specific modifiable risk factors impacting development.

There are scarce data about the prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease. The purpose of this study is to provide a multi-institutional description and comparison of the overall prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease.Retrospective multi-institutional study.The Pediatric Health Information System database.Neonates with congenital heart disease between 2004 and 2014.None.The primary study measure is the prevalence of necrotizing enterocolitis. Secondary measures include in-hospital mortality, hospital charges, ICU length of stay, hospital length of stay, and 30-day readmission. The prevalence of necrotizing enterocolitis was 3.7% (1,448/38,770) and varied significantly among different congenital heart disease diagnoses. The lowest prevalence of necrotizing enterocolitis was in transposition of the great arteries (n = 104, 2.1%). Compared with transposition of the great arteries, necrotizing enterocolitis occurred more frequently in neonates with hypoplastic left heart syndrome (odds ratio, 2.7; 95% CI, 2.1-3.3), truncus arteriosus (odds ratio, 2.6; 95% CI, 1.9-3.5), common ventricle (odds ratio, 2.1; 95% CI, 1.5-2.8), and aortic arch obstruction (odds ratio, 1.4; 95% CI, 1.1-1.7). Prematurity is a significant risk factor for necrotizing enterocolitis and for mortality in neonates with necrotizing enterocolitis, conferring varying risk by cardiac diagnosis. Unadjusted mortality associated with necrotizing enterocolitis was 24.4% (vs 11.8% in neonates without necrotizing enterocolitis; p0.001), and necrotizing enterocolitis increased the adjusted mortality in neonates with transposition of the great arteries (odds ratio, 2.5; 95% CI, 1.5-4.4), aortic arch obstruction (odds ratio, 1.8; 95% CI, 1.3-2.6), and tetralogy of Fallot (odds ratio, 1.6; 95% CI, 1.1-2.4). Necrotizing enterocolitis was associated with increased hospital charges (p0.0001), ICU length of stay (p = 0.001), and length of stay (p = 0.001).The prevalence of necrotizing enterocolitis among neonates with congenital heart disease is 3.7% and is associated with increased in-hospital mortality, length of stay, and hospital charges. The prevalence and associated mortality of necrotizing enterocolitis in congenital heart disease vary among different heart defects.

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Up to 70 000 patients may be alive worldwide today with Fontan circulation, and this population is expected to double in the next 20 years. In the absence of a subpulmonary ventricle, Fontan circulation is characterized by chronically elevated systemic venous pressures and decreased cardiac output. The addition of this acquired abnormal circulation to innate abnormalities associated with single-ventricle congenital heart disease exposes these patients to a variety of complications. Circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein-losing enteropathy, and plastic bronchitis are potential complications of the Fontan circulation. Abnormalities in body composition, bone structure, and growth have been detected. Liver fibrosis and renal dysfunction are common and may progress over time. Cognitive, neuropsychological, and behavioral deficits are highly prevalent. As a testimony to the success of the current strategy of care, the proportion of adults with Fontan circulation is increasing. Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined. There is a clear need for consensus on how best to follow up patients with Fontan circulation and to treat their complications. This American Heart Association statement summarizes the current state of knowledge on the Fontan circulation and its consequences. A proposed surveillance testing toolkit provides recommendations for a range of acceptable approaches to follow-up care for the patient with Fontan circulation. Gaps in knowledge and areas for future focus of investigation are highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for these unique individuals. ispartof: CIRCULATION vol:140 issue:6 pages:E234-E284 ispartof: location:United States status: published

BACKGROUND: The clinical spectrum of pediatric acute myocarditis ranges from minimal symptoms with intact hemodynamics to rapid cardiovascular collapse and death. We sought to identify factors on initial presentation associated with subsequent hemodynamic compromise. METHODS: We performed a retrospective cohort study of patients with acute myocarditis at a freestanding pediatric hospital from 2007 to 2016. We defined 2 cohorts: high-acuity patients with hemodynamic compromise defined as requiring inotropic or vasoactive medications, cardiopulmonary resuscitation, extracorporeal membrane oxygenation, ventricular assist devices, or transplant or who died and low-acuity patients without these interventions. We collected the first recorded set of vital signs, symptoms, laboratory values, and chest radiograph, electrocardiogram, and echocardiography results. Univariate analysis was performed, and 2 multivariable logistic regression models were created to discriminate between cohorts. RESULTS: A total of 74 patients were included: 33 high acuity and 41 low acuity. There were significant differences in demographics, symptoms, and physical examination, laboratory, electrocardiogram, and echocardiography findings between high- and low-acuity cohorts. Multivariable logistic regression models were highly discriminate in predicting those in the high-acuity cohort. The first model included presence of tachycardia, tachypnea, creatinine, and cardiomegaly on chest radiograph (area under the curve = 0.913). The second model added the presence of pericardial effusion to the above variables (area under the curve = 0.964). CONCLUSIONS: Models based on factors available at initial presentation with acute myocarditis are predictive of subsequent hemodynamic compromise. If our results can be validated in a multicenter study, these models may help disposition patients with suspected acute myocarditis (with those who meet model criteria being admitted to centers capable of rapidly providing extracorporeal membrane oxygenation, ventricular assist devices, and heart transplant evaluation).

Author(s): Jenkins, Kathy J; Botto, Lorenzo D; Correa, Adolfo; Foster, Elyse; Kupiec, Jennifer K; Marino, Bradley S; Oster, Matthew E; Stout, Karen K; Honein, Margaret A

Objectives: The Pediatric Advanced Life Support recommendations were developed for otherwise healthy infants and children with normal cardiac anatomy. Patients with acquired and congenital heart disease require specific considerations that may differ from the Pediatric Advanced Life Support recommendations. Our aim is to present prearrest, arrest, and postarrest considerations that are unique to children with congenital and acquired heart disease. Data Source: MEDLINE and PubMed. Conclusion: A clear understanding of the underlying anatomy and physiology of congenital and acquired heart disease is imperative in order to employ the appropriate modifications to the current Pediatric Advanced Life Support recommendations and to optimize outcomes.

BackgroundFontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients.Methods and resultsThis study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman’s Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6–33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (−0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (−0.32, p2versus lower-risk group=2.96 L/minute/m2, (pConclusionsHigher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation.

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.

The number of patients surviving with congenital heart disease (CHD) has soared over the last 3 decades. Adults constitute the fastest-growing segment of the CHD population, now outnumbering children. Research to date on the heart-brain intersection in this population has been focused largely on neurodevelopmental outcomes in childhood and adolescence. Mutations in genes that are highly expressed in heart and brain may cause cerebral dysgenesis. Together with altered cerebral perfusion in utero, these factors are associated with abnormalities of brain structure and brain immaturity in a significant portion of neonates with critical CHD even before they undergo cardiac surgery. In infancy and childhood, the brain may be affected by risk factors related to heart disease itself or to its interventional treatments. As children with CHD become adults, they increasingly develop heart failure, atrial fibrillation, hypertension, diabetes mellitus, and coronary disease. These acquired cardiovascular comorbidities can be expected to have effects similar to those in the general population on cerebral blood flow, brain volumes, and dementia. In both children and adults, cardiovascular disease may have adverse effects on achievement, executive function, memory, language, social interactions, and quality of life. Against the backdrop of shifting demographics, risk factors for brain injury in the CHD population are cumulative and synergistic. As neurodevelopmental sequelae in children with CHD evolve to cognitive decline or dementia during adulthood, a growing population of CHD can be expected to require support services. We highlight evidence gaps and future research directions.

Objective To assess how obesity or diabetes mellitus impacts outcomes in patients undergoing cardiac surgery in pediatric hospitals. Design A multi-institutional, matched case-control study of the Pediatric Health Information System database was performed. Setting Tertiary children's hospitals in the United States. Patients. All cardiac surgical cases in patients with obesity or diabetes mellitus between 2004 and 2012 were included. Cases were matched to controls by age, sex, race, and Risk Adjustment for Congenital Heart Surgery score. Outcome Measures Mortality, surgical complications, and hospital utilization. Differences in outcome measures were assessed by chi-square and Mann–Whitney tests. P value

Glucocorticoid hormones (GCs) are important regulators of lipid metabolism, promoting lipolysis with acute treatment but lipogenesis with chronic exposure. Conventional wisdom posits that these disparate outcomes are mediated by the classical glucocorticoid receptor GRα. There is insufficient knowledge of the GC receptors (GRα and GRβ) in metabolic conditions such as obesity and diabetes. We present acute models of GC exposure that induce lipolysis, such as exercise, as well as chronic-excess models that cause obesity and lipid accumulation in the liver, such as hepatic steatosis. Alternative mechanisms are then proposed for the lipogenic actions of GCs, including induction of GC resistance by the GRβ isoform, and promotion of lipogenesis by GC activation of the mineralocorticoid receptor (MR). Finally, the potential involvement of chaperone proteins in the regulation of adipogenesis is considered. This reevaluation may prove useful to future studies on the steroidal basis of adipogenesis and obesity.

Children with long QT syndrome (LQTS) live with the risk of sudden death, activity restrictions, and the need for daily medications. We sought to evaluate the quality of life (QOL), self-perception, and behavior of patients with LQTS as perceived by both patients and their parents and identify predictors of lower QOL. QOL (Pediatric QOL Inventory [PedsQL] and Pediatric Cardiac Quality of Life Inventory [PCQLI]), self-perception, and behavioral inventories were completed by patients with LQTS and their parents. Comparison of PedsQL scores was made to published data for healthy children using t tests, and PCQLI scores were compared with those of patients with differing complexity of congenital heart disease. Mixed modeling was used for multivariable analysis. Sixty-one patients with LQTS were evaluated (age 13.6 ± 3.0 years; male 49%). Compared with healthy children, the PedsQL Total, Psychosocial, and Physical Health Summary scores were significantly lower for patients with LQTS and parent proxy reports (p ≤0.001). In general, PCQLI scores of patients with LQTS and parents were similar to those of patients with tetralogy of Fallot (p ≥0.2), lower than those of patients with bicuspid aortic valve (p ≤0.02), and higher than those of patients with single ventricle (p ≤0.03). Lower patient and parent PCQLI scores were associated with internalizing problems. For parents, the presence of a cardiac device and medication side effects were additionally associated with lower PCQLI scores. In conclusion, patients with LQTS and their parents report lower QOL than normal children secondary to physical and psychosocial factors. Increasing focus on the psychological well-being of these patients is needed in an effort to improve their QOL.