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Homozygous familial hypercholesterolemia (HoFH) is a rare, autosomal semi-dominant lipid metabolism disorder characterized by extremely high low-density lipoprotein cholesterol (LDL-C) levels and premature cardiovascular disease. The objective of this study was to investigate sex-differences in the treatment and outcomes of patients with HoFH. We examined clinical characteristics, lipid-lowering therapy (LLT), and cardiovascular events using descriptive statistics of patients in the Canadian HoFH registry. Major adverse cardiovascular events (MACE) were defined as the composite of cardiovascular death, non-fatal myocardial infarction, and stroke. Sex differences between continuous and categorical variables were analyzed using Mann-Whitney U test and Fisher's Exact test, respectively. This study included 48 patients (27 (56%) female). The median age at diagnosis in females was 14.0 (interquartile range (IQR) 9.0–30.0) and in males was 8.0 (IQR 2.0–23.0) (p = 0.07). Baseline clinical characteristics were comparable between both sexes. The median baseline LDL-C was 12.7 mmol/L (10.0–18.3) in females and 15.3 (10.5–20.0) in males (p = 0.51). Follow up LDL-C levels were 7.6 mmol/L (IQR 4.8–11.0) in females and 6.3 (IQR 4.6–7.5) in males (p = 0.1). Most patients were taking 3 or more LLTs, with comparable proportions in both sexes (p = 0.26). Apheresis was similar in both sexes, 14 (51.8%) vs. 10 (47.6%) (p = 0.2). Over a mean of 10 years of follow-up, MACE occurred in 3 females (11.1%) and 4 males (19.1%) (p = 0.2). Lipid levels and treatment were similar between sexes. MACE occurred in similar proportions between sexes, indicating that HoFH offsets the inherently lower cardiovascular risk in pre-menopausal females. Further investigation into sex-differences in HoFH in larger sample sizes is warranted. [Display omitted] [ABSTRACT FROM AUTHOR]

Pulmonary artery banding (PAB) in isolation or combined with a congenital cardiac surgical procedure is common and has important mortality. We aimed to determine patient characteristics, clinical outcomes, variation in clinical outcomes by diagnoses, and center variation in PAB use. Using The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD), this study evaluated outcomes of patients undergoing PAB across diagnoses, participating centers, and additional procedures. Patients were identified by procedure and diagnosis codes from 2016 to 2019. We separated patients into groups of main and bilateral PAB and described their outcomes, focusing on patients with main PAB. This study identified 3367 PAB procedures from 2016 to 2019 (3% of all STS CHSD cardiovascular cases during this period): 2677 main PAB, 690 bilateral PAB. Operative mortality was 8% after main PAB and 26% after bilateral PAB. There was significant variation in use of main PAB by center, with 115 centers performing at least 1 main PAB procedure (range, 1-134; Q1-Q3, 8-33). For patients with main PAB, there were substantial differences in mortality, depending on timing of main PAB relative to other procedures. The highest operative mortality (25%; P <.0001) was in patients who underwent main PAB after another separate procedure during their admission, with extracorporeal membrane oxygenation being the most frequent preceding procedure. PAB is a frequently used congenital cardiac procedure with high mortality and variation in use across centers. Outcomes vary widely by banding type and patient diagnosis. Main PAB after cardiac surgical procedures, especially extracorporeal membrane oxygenation, is associated with very high operative mortality. [ABSTRACT FROM AUTHOR]

Aims: Clustering of cardiometabolic risk factors (CMRFs) indicates cardiometabolic risk (CMR), a key driver of cardiovascular disease. Early detection and treatment of CMR are important to decrease this risk. To facilitate the identification of individuals at risk, CMRFs are commonly combined into a CMR Score. This scoping review aims to identify CMRFs and methods used to calculate adolescent CMR Scores.Data Synthesis: Systematic searches were executed in Child Development and Adolescent Studies, Ovid MEDLINE, Ovid EMBASE, Ovid PsycINFO, EBSCO CINAHL, Scopus Elsevier, Cochrane CENTRAL, and Nursing and Allied Health. No limits were placed on publication date or geographic location. Studies were included if participants were 10-19 years and the study reported CMRFs in a composite score. Key extracted information included participant characteristics, CMRFs comprising the scores, and methods of score calculation. CMRFs were categorized and data were reported as frequencies. This study identified 170 studies representing 189 CMR Scores. The most common CMRF categories were related to lipids, blood pressure, and adiposity. The most frequent CMRFs were triglyceride z-score, systolic blood pressure z-score, and inverse high-density lipoproteins z-score. Scores were mostly calculated by summing CMRF z-scores without weighting.Conclusions: The range of CMRFs and Scores identified in adolescent CMR literature limits their use and interpretation. Published CMR Scores commonly contain two main limitations: (a) use of an internal cohort as the z-score reference population, and (b) Scores relying on adiposity measures. We highlight the need for a standard set of CMRFs and a consensus for a CMR Score for adolescents. [ABSTRACT FROM AUTHOR]

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2010.07.009 Byline: Maria E. Lagopoulos, Cedric Manlhiot, Brian W. McCrindle, Edgar T. Jaeggi, Mark K. Friedberg, Lynne E. Nield Abstract: We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy. Article History: Received 26 March 2010; Accepted 2 July 2010

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2009.02.014 Byline: Patricia E. Longmuir, Brian W. McCrindle Abstract: Physical activity is important for the health of children after the Fontan procedure. Parents uncertain about physical activity have children who are more sedentary. To understand parent uncertainty, we examined agreement regarding activity restrictions from parents, cardiologists, and medical charts. Author Affiliation: Hospital for Sick Children and Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada Article History: Received 5 December 2008; Accepted 19 February 2009 Article Note: (footnote) The authors are solely responsible for the design and conduct of this study, all analyses, and preparation of the manuscript. Data collection and analyses were supported by the Heart and Stroke Foundation of Ontario, Toronto, Ontario, Canada, grant no. NA 5950, and Canadian Institutes of Health Research, Ottawa, Ontario, Canada (P Longmuir, Doctoral Research Award).

Patients receiving a right ventricle to pulmonary artery conduit (PC) in infancy will require successive procedures or replacements, each with variable longevity. We sought to identify factors associated with time-related risk of a subsequent surgical replacement (PC3) or transcatheter pulmonary valve insertion (TPVI) after a second surgically placed PC (PC2). From 2002 to 2016, 630 patients from 29 Congenital Heart Surgeons' Society member institutions survived to discharge after initial valved PC insertion (PC1) at age ≤ 2 years. Of those, 355 underwent surgical replacement (PC2) of that initial conduit. Competing risk methodology and multiphase parametric hazard analyses were used to identify factors associated with time-related risk of PC3 or TPVI. Of 355 PC2 patients (median follow-up, 5.3 years), 65 underwent PC3 and 41 TPVI. Factors at PC2 associated with increased time-related risk of PC3 were smaller PC2 Z score (hazard ratio [HR] 1.6, P <.001), concomitant aortic valve intervention (HR 7.6, P =.009), aortic allograft (HR 2.2, P =.008), younger age (HR 1.4, P <.001), and larger Z score of PC1 (HR 1.2, P =.04). Factors at PC2 associated with increased time-related risk of TPVI were aortic allograft (HR: 3.3, P =.006), porcine unstented conduit (HR 4.7, P <.001), and older age (HR 2.3, P =.01). Aortic allograft as PC2 was associated with increased time-related risk of both PC3 and TPVI. Surgeons may reduce risk of these subsequent procedures by not selecting an aortic homograft at PC2, and by oversizing the conduit when anatomically feasible. [ABSTRACT FROM AUTHOR]

Background: The Long-terM OUtcomes after the Multisystem Inflammatory Syndrome In Children (MUSIC) study aims to characterize the frequency and time course of acute and long-term cardiac and non-cardiac sequelae in multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C), which are currently poorly understood.Methods: This multicenter observational cohort study will enroll at least 600 patients <21 years old who meet the Centers for Disease Control and Prevention case definition of MIS-C across multiple North American centers over 2 years. The study will collect detailed hospital and follow-up data for up to 5 years, and optional genetic testing. Cardiac imaging at specific time points includes standardized echocardiographic assessment (all participants) and cardiac magnetic resonance imaging (CMR) in those with left ventricular ejection fraction (LVEF) <45% during the acute illness. The primary outcomes are the worst LVEF and the highest coronary artery z-score of the left anterior descending or right coronary artery. Other outcomes include occurrence and course of non-cardiac organ dysfunction, inflammation, and major medical events. Independent adjudication of cases will classify participants as definite, possible, or not MIS-C. Analysis of the outcomes will include descriptive statistics and regression analysis with stratification by definite or possible MIS-C. The MUSIC study will provide phenotypic data to support basic and translational research studies.Conclusion: The MUSIC study, with the largest cohort of MIS-C patients and the longest follow-up period to date, will make an important contribution to our understanding of the acute cardiac and non-cardiac manifestations of MIS-C and the long-term effects of this public health emergency. [ABSTRACT FROM AUTHOR]

Kawasaki disease (KD) with coronary artery aneurysms (complex KD) presents relentless challenges for families. Psychosocial experiences and needs were explored. A descriptive, exploratory study of adolescents and parents using a needs survey and psychosocial instruments (anxiety, depression, and functional impairment). Fifty-one parents and 38 adolescents participated. Predominant interests were for information sheets, newsletters, Web sites, and phone applications. Gaps in disease-specific knowledge and awareness of coronary artery aneurysms between parents and adolescents were identified. Psychosocial concerns for adolescents included symptoms of anxiety (22%), depression (13%), and functional impairment (22%). Multivariable analyses indicated higher depression scores associated with longer travel distance from specialists (p =.04). Parent-reported social concerns for their adolescent were associated with higher anxiety (p =.005) and functional impairment (p =.005). Written commentary complemented the findings. Care protocols require psychosocial assessment/referral and the use of virtual platforms. The groundwork was laid for developing patient and family-centered strategies. [ABSTRACT FROM AUTHOR]

To understand the effectiveness of a nurse-led transition intervention by analyzing qualitative data generated in the context of a clinical trial. Qualitative study of a two-session transition intervention conducted by registered nurses at two sites. Adolescents aged 16–17 years with moderate or complex congenital heart disease (CHD) had been randomized to a two-session transition intervention or usual care. Session 1 emphasized patient education including creation of a health passport and goal setting. Session 2, two months later, emphasized self-management. Qualitative data extracted from intervention logs, field notes and audio recordings of the sessions were analyzed for content and themes. Data from 111 transition intervention sessions with 57 adolescents were analyzed. Creating a health passport, goal setting, and role-plays were the elements of the intervention most valued by participants. A typology of transition readiness was identified: 1) the independent adolescent (5%), already managing their own care; 2) the ready adolescent who was prepared for transition after completing the intervention (46%); 3) the follow-up needed adolescent who was still in need of extra coaching (26%), and 4) the at-risk adolescent who warranted immediate follow-up (14%). Baseline knowledge and transition surveys scores validated the typology. A two-session nursing intervention met the transition needs of approximately half of adolescents with CHD. However, additional transition-focused care was needed by 40% of participants (groups 3 and 4). These findings will guide pediatric nurses and other healthcare professionals to optimize an individualized approach for ensuring transition readiness for adolescents with CHD. • Two-session nurse-led transition intervention for 16–17-year-olds • Mixed methods unobtrusive data analysis to understand intervention effectiveness • Four types of transition readiness from independent to at-risk to guide practice • Condition knowledge and transition readiness scores consistent with typology • MyHealth Passport, goal setting and role play key transition care elements. [ABSTRACT FROM AUTHOR]

Objectives: To compare the clinical features and resuscitative measures of children with Kawasaki disease shock syndrome vs septic shock.Study Design: In this retrospective case-control study, children with Kawasaki disease shock syndrome admitted to the intensive care unit from 2007 to 2017 were identified and compared with age-matched controls with septic shock. We studied 9 children with Kawasaki disease shock syndrome and 18 children with septic shock. Clinical characteristics were abstracted and between-group differences were compared.Results: Compared with septic shock controls, children with Kawasaki disease shock syndrome were less likely to have an underlying comorbid illness (1/9 [11%] vs 11/18 [61%]; P = .02) and were more likely to have at least 1 of the 5 classic diagnostic signs of Kawasaki disease at presentation (9/9 [100%] vs 0/18 [0%]; P < .001), a longer duration of illness before admission (9 days [IQR, 7-14 days] vs 3 days [IQR, 1-5 days]; P = .004), and a lower platelet count at presentation (140 [IQR 73, 167]) vs 258 [IQR, 137-334]; P = .02). Among patients who underwent echocardiography, abnormalities such as ventricular dysfunction, valvulitis, and coronary artery dilation were more common in the Kawasaki disease shock syndrome cohort (5/9 [56%] vs 0/7 [0%]; P = .03). There were no differences in volume of fluid resuscitation, vasoactive-inotropic scores, duration of inotropic therapy, or biochemical markers of illness severity (other than platelet count) between the matched groups.Conclusions: A longer duration of illness before admission, lack of any significant underlying medical comorbidities, a lower platelet count, echocardiographic abnormalities, and the presence of classic diagnostic signs of Kawasaki disease at presentation may be useful early features to differentiate Kawasaki disease shock syndrome from septic shock. [ABSTRACT FROM AUTHOR]

Background: Growth abnormalities in single-ventricle survivors may reduce quality of life (QoL) and exercise capacity.Methods: This multicenter, longitudinal analysis evaluated changes in height and body mass index (BMI) compared to population norms and their relationship to mortality, ventricular morphology, QoL, and exercise capacity in the Pediatric Heart Network Fontan studies.Results: Fontan 1 (F1) included 546 participants (12 ± 3.4 years); Fontan 2 (F2), 427 (19 ± 3.4 years); and Fontan 3 (F3), 362 (21 ± 3.5 years), with ~60% male at each time point. Height z-score was -0.67 ± -1.27, -0.60 ± 1.34, and- 0.43 ± 1.14 at F1-F3, lower compared to norms at all time points (P ≤ .001). BMI z-score was similar to population norms. Compared to survivors, participants who died had lower height z-score (P ≤ .001). Participants with dominant right ventricle (n = 112) had lower height z-score (P ≤ .004) compared to dominant left (n = 186) or mixed (n = 64) ventricular morphologies. Higher height z-score was associated with higher Pediatric Quality of Life Inventory for the total score (slope = 2.82 ± 0.52; P ≤ .001). Increase in height z-score (F1 to F3) was associated with increased oxygen consumption (slope = 2.61 ± 1.08; P = .02), whereas, for participants >20 years old, an increase in BMI (F1 to F3) was associated with a decrease in oxygen consumption (slope = -1.25 ± 0.33; P ≤ .001).Conclusions: Fontan survivors, especially those with right ventricular morphology, are shorter when compared to the normal population but have similar BMI. Shorter stature was associated with worse survival. An increase in height z-score over the course of the study was associated with better QoL and exercise capacity; an increase in BMI was associated with worse exercise capacity. [ABSTRACT FROM AUTHOR]

Improved survival for patients with dextro-transposition of the great arteries (d-TGA) has led to an increased focus on functional health status (FHS). We assessed late survival and patient-perceived FHS for repaired TGA patients. From 1985-1990, 830 neonates admitted to 24 Congenital Heart Surgeons' Society (CHSS) institutions with d-TGA underwent repair, including 516 arterial switch, 110 Mustard, 175 Senning, and 29 Rastelli operations. Median follow-up was 24.0 years (range, 0-32.7 years). We performed multiphase parametric hazard analysis for death after repair. Patients completed Pediatric Quality of Life Inventory (PedsQL) Core Scales and Cardiac Module Adult Forms. Patient and operative factors and CHSS General Questionnaire responses were analyzed for association with FHS using multiple linear regression. Survival at 30 years after repair was arterial switch, 80% ± 2%; Mustard, 81% ± 5%; Senning, 70% ± 4%; and Rastelli, 86% ± 8%. The arterial switch had the lowest hazard for late death. TGA patients reported FHS similar to a healthy population in all domains except physical health (lower scores). Symptoms, including chest pain and fainting, and having a pacemaker were associated with lower, and being employed with higher, self-reported physical health. Arterial switch patients reported higher FHS than the atrial switch patients in all domains. Arterial switch patients have a lower risk of premature death and better FHS than those with an atrial switch. Increased surveillance in atrial switch patients is warranted because of their increased risk of late death. Presence of symptoms, pacemaker, and lack of employment are associated with reduced FHS. [ABSTRACT FROM AUTHOR]

The Fontan procedure is the final step of the 3-stage palliative procedure commonly performed in children with single ventricle physiology. Thrombosis remains an important complication in children after this procedure. To date, guideline recommendations for the type and duration of thromboprophylaxis after Fontan surgery are mainly based on extrapolation of knowledge gained from adults at risk for thrombosis in other clinical settings. Warfarin is being used off-label, and because of its multiple interactions with other drugs and food, a new alternative is highly desirable. Rivaroxaban, a direct Factor Xa inhibitor with a predictable pharmacokinetic profile, is a candidate to address this medical need. The UNIVERSE study is a prospective, open-label, active-controlled, multicenter study in children 2 to 8 years of age who have single ventricle physiology and had the Fontan procedure within the 4 months preceding enrollment. This study consists of 2 parts. In Part A, rivaroxaban pharmacokinetics, pharmacodynamics, safety, and tolerability are assessed to validate the pediatric dosing selected. In Part B, safety and efficacy of rivaroxaban versus acetylsalicylic acid are evaluated for thromboprophylaxis in children post–Fontan procedure. Children in each part will receive study drug for 12 months. Part A has been completed with 12 children enrolled. Enrollment into Part B is currently ongoing. The UNIVERSE study aims to provide dosing, pharmacokinetics/pharmacodynamics, safety, and efficacy information on the use of rivaroxaban, an oral anticoagulant, versus acetylsalicylic acid, an antiplatelet agent, in children with single ventricle physiology after the Fontan procedure. [ABSTRACT FROM AUTHOR]

Background: Body mass index measures excess weight for size, and does not differentiate between fat mass (FM) and fat-free mass (FFM). Bioelectrical impedance analysis (BIA) is most commonly used to assess FM and FFM as it is simple and inexpensive. Variables from BIA measurements are used in predictive equations to estimate FM and FFM. To date, these equations have not been validated for use in adolescents with severe obesity.Objectives: In a cohort of adolescents with severe obesity (SO), a BMI ≥ 120% of the 95th percentile, this study aimed to 1) derive a BIA predictive equation data from air displacement plethysmography (ADP) measurements; 2) reassess the equation in a second validation cohort; and 3) compare the accuracy of existing body composition equations.Methods: Adolescents with SO were assessed using ADP and BIA. FM values derived from ADP measurements from the first cohort (n = 27) were used to develop a BIA predictive equation (i.e., Hamilton). A second cohort (n = 65) was used to cross-validate the new and 9 existing BIA predictive equations.Results: Ninety-two adolescents (15.8 ± 1.9 y; BMI: 46.1 ± 9.9 kg/m2) participated. Compared with measured FFM using ADP: 1) the Lazzer, Hamilton, Gray, and Kyle equations were without significant bias; 2) the Hamilton and Gray equations had the smallest absolute and relative differences; 3) the Kyle and Gray equations showed the strongest correlation; 4) the Hamilton equation most accurately predicted FFM within ± 5% of measured FFM; and 5) 8 out of 9 equations had similar root mean squared prediction error values (6.03-6.64 kg).Conclusion: The Hamilton BIA equation developed in this study best predicted body composition values for groups of adolescents with severe obesity in a validation cohort. [ABSTRACT FROM AUTHOR]

Objective: To assess the validity of existing clinical scales assessing the presence of physical and functional abnormalities for diagnosing post-thrombotic syndrome (PTS) in children, including specific evaluation of use in children with congenital heart disease (CHD).Study Design: One hundred children aged >2 years (average age, 6 years), including 33 with CHD and previously proven extremity deep vein thrombosis (DVT), 37 with CHD and no previous DVT, and 30 healthy siblings, were blindly assessed for PTS using the modified Villalta Scale (MVS). All patients aged <6 years underwent neurodevelopmental testing and an age-appropriate quality of life assessment.Results: The MVS identified mild PTS in 20 children and moderate PTS in 1 child (including 14 of 33 [42%] in the CHD/DVT group, 5 of 37 [14%] in the CHD/no DVT group, and 2 of 30 controls [7%]). The diagnosis of PTS was confirmed clinically in 14 patients, all of whom had previous thrombosis and 1 of whom was MVS-negative. MVS had an accuracy of 91% and performed reasonably well as a screening tool but poorly as a diagnostic tool. MVS reliability was acceptable. Children with PTS had similar quality of life as those without PTS but had higher rates of neurodevelopmental delays in gross motor skills (70% vs 24%; P = .02) and problem-solving indicators (60% vs 15%; P = .008).Conclusions: Using the MVS scale for PTS screening in children with CHD is feasible and reliable, and the scale has good correlation with a clinical diagnosis of PTS despite a high prevalence of false-positive findings. Further research is needed to determine the clinical relevance of PTS in this population. [ABSTRACT FROM AUTHOR]

The Fontan operation creates a circulation characterized by elevated central venous pressure and low cardiac output. Over time, these characteristics result in a predictable and persistent decline in exercise performance that is associated with an increase in morbidity and mortality. A medical therapy that targets the abnormalities of the Fontan circulation might, therefore, be associated with improved outcomes. Udenafil, a phosphodiesterase type 5 inhibitor, has undergone phase I/II testing in adolescents who have had the Fontan operation and has been shown to be safe and well tolerated in the short term. However, there are no data regarding the long-term efficacy of udenafil in this population. The Fontan Udenafil Exercise Longitudinal (FUEL) Trial is a randomized, double-blind, placebo-controlled phase III clinical trial being conducted by the Pediatric Heart Network in collaboration with Mezzion Pharma Co, Ltd. This trial is designed to test the hypothesis that treatment with udenafil will lead to an improvement in exercise capacity in adolescents who have undergone the Fontan operation. A safety extension trial, the FUEL Open-Label Extension Trial (FUEL OLE), offers the opportunity for all FUEL subjects to obtain open-label udenafil for an additional 12 months following completion of FUEL, and evaluates the long-term safety and tolerability of this medication. This manuscript describes the rationale and study design for FUEL and FUEL OLE. Together, these trials provide an opportunity to better understand the role of medical management in the care of those who have undergone the Fontan operation. [ABSTRACT FROM AUTHOR]

Objectives: To examine patterns of non-high-density lipoprotein (HDL) cholesterol in early childhood and identify factors associated with persistent high non-HDL cholesterol in healthy urban children.Study Design: We identified all children enrolled in a primary care practice-based research network called TARGet Kids! (The Applied Research Group for Kids) with ≥3 laboratory measurements of non-HDL cholesterol. Latent class growth model analysis was performed to identify distinct trajectory groups for non-HDL cholesterol. Trajectory groups were then categorized into "normal" vs "persistent-high" non-HDL cholesterol based on guideline cut-off values and logistic regression was completed to examine the association between trajectory group and the presence of anthropometric and cardiometabolic risk factors.Results: A total of 608 children met inclusion criteria for the trajectory analysis (median age at enrolment = 18.3, IQR = 27.9 months). Four trajectory groups were identified with 2 groups (n = 451) categorized as normal non-HDL cholesterol and 2 groups (n = 157) as persistent high non-HDL cholesterol. Family history of high cholesterol (OR 2.04, 95% CI 1.27-3.28) was associated significantly with persistent high non-HDL cholesterol, whereas East/Southeast Asian vs European ethnicity (OR 0.33, 95% CI 0.14-0.78), longer breastfeeding duration (OR 0.96, 95% CI 0.93-1.00), and greater birth weight (OR 0.69, 95% CI 0.48-1.00) were associated with lower odds of persistent high non-HDL cholesterol.Conclusions: Patterns of non-HDL cholesterol are identified during early childhood, and family history of high cholesterol was associated most strongly with persistent high non-HDL cholesterol. Future research should inform the development of a clinical prediction tool for lipids in early childhood to identify children who may benefit from interventions to promote cardiovascular health. [ABSTRACT FROM AUTHOR]

Traditional dietary assessment tools, such as food records and dietary recalls, are time and resource intensive to determine achievement of cholesterol-lowering dietary targets defined in the Cardiovascular Health Integrated Lifestyle Diet (CHILD-2). We developed a simplified 10-point assessment tool called the Healthy Eating Assessment Tool (HEAT) to more efficiently assess a patient's overall dietary quality and behavior during a time-limited dietitian encounter. We sought to determine the utility of the HEAT in relation to meeting dietary fat/cholesterol restriction cutpoints of the CHILD-2, and its association with markers of adiposity and lipid variables. This 2-year single center, prospective cross-sectional study recruited 2–18-year-old patients assessed in a pediatric dyslipidemia clinic. Patients who had incomplete food records or diagnoses/diets that significantly impacted dietary intake were excluded. Nutritional analysis results of 7-day food records, BMI z-score, waist:height ratio and lipid variables were assessed for association with individual HEAT scores (Pearson correlation), including HEAT score categories (Poor 0-4.5, Fair 5-6.5, Good 7-8.5, Excellent 9-10; ANOVA). 70 participants were enrolled with a mean age of 12.6 ± 3.8 years (29 females; 41%). HEAT score category was Poor for 8 (11%), Fair for 28 (40%), Good for 23 (33%) and Excellent for 11 participants (16%). Higher HEAT score was significantly correlated with lower total fat percentage of total daily calories (r=-0.27, p=0.02), and higher intake of fiber (r=0.46, p=<0.01), fruits (r=0.31, p=<0.01), and vegetables (r=0.32, p=<0.01). Only patients with the highest HEAT scores (Good 43%, Excellent 64%) met the CHILD-2 cutpoint of <25% total fat calories (p=0.03), with a non-significant trend for saturated fat to <8% of total daily calories (Excellent 64%), and no association with cholesterol intake. Lower HEAT score was correlated with higher BMI z-score (r=-0.31, p=<0.01) and waist:height ratio (r=-0.31, p=<0.01). There was no association between HEAT score and any lipid variable after adjusting for age, sex, amount of moderate to vigorous physical activity, hours of screen time, lipid-lowering medications, BMI z-score and waist:height ratio. HEAT score associations with meeting CHILD-2 fat targets were modest, with more consistent associations with markers of adiposity, and no independent association with lipid levels. While fat-restricted diets are safe they are not particularly effective for treatment of dyslipidemia or for weight management alone. The HEAT may be a more useful and simplified way of assessing and tracking broader dietary goals in clinical practice. No [ABSTRACT FROM AUTHOR]

Summary Background & aims Severe obesity in children and adolescents is now a serious global health concern. Accurate measurements of resting energy expenditure (REE) is a key foundation for successful obesity treatment. Clinical dietitians rely heavily on measured or calculated REE to tailor dietary interventions. Indirect calorimetry (IC) is the gold standard for measuring REE. However, predictive resting energy expenditure (PREE) equations are commonly used when IC is unavailable due to cost or practicality. PREE equations differ based on variables such as age, gender, weight, and height and selecting the most accurate PREE for an individual is crucial to avoid over or underestimation of energy requirements. Published studies investigating the accuracy of PREE equations in obese children and adolescents have reported inconsistent findings, which likely result from heterogeneity in the patient populations studied. Accordingly, this study aimed to (a) assess the accuracy of the published PREE equations in a group of severely obese (SO) adolescents using IC measurement, and (b) determine if there is a BMI threshold at which the PREE equations become less accurate. Methods SO adolescents were studied using IC. REE was calculated using nine commonly used PREE equations. Generalized linear regression equations were used to compare absolute and relative differences between calculated and measured REE (MREE) for each PREE equation. Accuracy was calculated as the percentage of subjects with PREE values within 10 percent of MREE. Results 226 SO adolescents (mean ± SD age: 15.9 ± 1.9 years; weight: 126.9 ± 24.5 kg; BMI: 44.9 ± 8.1 kg/m 2 ) participated. Mean MREE was 2163 ± 443 kcal/d. PREE calculated by the Mifflin equation was the only equation without a statistically significant bias compared to MREE (mean bias of −23 ± 307 kcal/d; p = 0.26). Mifflin was also the most accurate with 61% of individuals within ±10% of MREE. PREE equations accuracy was not associated with degree of BMI elevation (31–69 kg/m 2 ). Conclusions In adolescents with severe obesity, the Mifflin equation best predicts REE. This should be the equation applied when using PREE to optimize nutritional care in this population. [ABSTRACT FROM AUTHOR]

Introduction For those living with Kawasaki disease and coronary artery aneurysms, little is known about the psychosocial burden faced by parents and their children. Methods Exploratory, descriptive, mixed-methods design examining survey and interview data about health-related uncertainty, intrusiveness, and self-efficacy. Results Parents' uncertainty was associated with missed diagnosis, higher income, and maternal education. Higher uncertainty scores among children were associated with absence of chest pain and lower number of echocardiograms. High intrusiveness scores among parents were associated with previous cardiac catheterization, use of anticoagulants, lower parent education and income, and missed diagnosis. High intrusiveness scores among children were associated with high paternal education. Children's total self-efficacy scores increased with chest pain and larger aneurysm size. Qualitative analysis showed two central themes: Psychosocial Struggle and Cautious Optimism . Discussion Negative illness impact is associated with a more intense medical experience and psychosocial limitations. Timely assessment and support are warranted to meet parents' and children's needs. [ABSTRACT FROM AUTHOR]

Background: It is unknown whether continuous cardiac resynchronization therapy (CRT) can lead to sustained improvement in hemodynamics after surgery for congenital heart disease (CHD).Objective: We investigated whether CRT improves cardiac index (CI) and blood pressure in infants after biventricular repair of CHD.Methods: We randomized infants younger than 4 months after biventricular CHD surgery to standard care or standard care plus CRT for 48 hours or until extubation if sooner. Change in the primary outcome of CI and blood pressure over time was compared between groups. For subgroup analysis, QRS duration was considered prolonged if greater than the 98th percentile.Results: Forty-two patients were randomized: 21 controls and 21 patients receiving CRT (median weight 4 kg). There were no identified adverse events from pacing. The change in CI over time was not different between patients receiving CRT and controls, but trended toward improvement in patients with wide QRS who received CRT (n = 9) vs controls with wide QRS (n = 8) (+1.65 (0.86) L/(min·m2); P = .06). Controls with wide QRS experienced the smallest increase in CI (0.33 L/(min·m2)). Blood pressure was significantly higher in infants with wide QRS who received CRT than in controls (+7.14 (3.08) mm Hg; P = .02). Serum lactate level, catecholamine use, ventilation time, and length of intensive care unit stay were similar between the 2 groups.Conclusion: CRT improved blood pressure and a trend toward higher CI in infants after repair of biventricular CHD with prolonged QRS duration. These findings warrant further study of CRT to improve postoperative recovery in infants with electrical dyssynchrony. [ABSTRACT FROM AUTHOR]

Background: Unfavorable left ventricular (LV) remodelling may be associated with adverse outcomes after Tetralogy of Fallot (TOF) repair. We sought to assess T1 cardiovascular magnetic resonance (CMR) markers of diffuse LV myocardial fibrosis in children after TOF repair, and associated factors. Methods: In this prospective, cross-sectional study, native (=non-contrast) T1 times and extracellular volume fraction (ECV) were quantified in the LV myocardium using CMR. Results were related to ventricular volumes and function, degree of pulmonary regurgitation, as well as surgical characteristics, and exercise capacity. Results: There was no difference in native T1 times or ECV between 31 TOF patients (age at CMR 13.9 ± 2.4 years, 19 male) and 15 controls (age at CMR 13.4 ± 2.6 years, 7 male). Female TOF patients had higher ECVs than males (25.2 ± 2.9 % versus 22.7 ± 3.3 %, p < 0.05). In the patient group, higher native T1 and ECV correlated with higher Z-Scores of right and left ventricular end-diastolic volumes, but not with reduced left and right ventricular ejection fraction or higher pulmonary regurgitation fraction. Longer cardiopulmonary bypass and aortic cross clamp times at surgery correlated with increased native T1 times and ECVs (r =0.48, p < 0.05 and r = 0.65, p < 0.01, respectively). Maximum workload (percent of predicted for normal) correlated inversely with ECV (r = -0.62, p < 0.05). Higher native T1 times correlated with worse LV longitudinal (r = 0.50, p < 0.05) and mid short axis circumferential strain (r = 0.38, p <0.05). Conclusions: As compared to controls, TOF patients did not express higher markers of diffuse fibrosis. Longer cardiopulmonary bypass and aortic cross clamp times at surgery as well as biventricular enlargement and reduced exercise tolerance are associated with markers of diffuse myocardial fibrosis after TOF repair. Female patients have higher markers of diffuse myocardial fibrosis than males. [ABSTRACT FROM AUTHOR]

Background Antithrombin is one of the main natural coagulation system inhibitors. It is potentiated by heparin, and may be a key component of heparin response, particularly in infants aged <1 year. We sought to determine the impact of baseline antithrombin activity on response to heparin and thrombin generation during cardiopulmonary bypass (CPB). Methods Secondary analysis was performed using linear regression analyses, which combined patients from a trial of individualized versus weight-based heparin management for 90 infants aged <1 year undergoing cardiac surgery. Results Mean baseline antithrombin activity was 0.69 ± 0.16 U/mL, and it was lower in neonates than in older infants (0.57 ± 0.15 vs 0.77 ± 0.12 U/mL; P < .001). Lower baseline antithrombin activity was associated with lower postheparin anti-Xa activity (EST [SE]: +0.47 (0.19) U/mL per 100 U/kg heparin; P = .01) and higher heparin doses during surgery (EST [SE]: +51 (17) U/kg per hour; P = .003). The administration of fresh frozen plasma attenuated the effect of low baseline antithrombin activity (interaction P value = .009). Patients with lower anti-Xa activity recorded during CPB had higher levels of thrombin-antithrombin complex (EST [SE]: +12.8 (4.7) ng/mL per −1 U/mL anti-Xa; P = .006); prothrombin activation fragment 1.2 (EST [SE]: +0.13 (0.07) log pg/mL per −1 U/mL anti-Xa; P = .06); and D-dimer (EST [SE]: −0.25 (0.09) log ng/mL per −1 U/mL anti-Xa; P = .009) in the postoperative period after adjustment for baseline antithrombin activity, duration of CPB, amount of fresh frozen plasma and heparin used throughout surgery in multivariable models. Conclusions Low circulating antithrombin activity is associated with lower heparin efficacy, which ultimately leads to a lower ability to suppress thrombin generation during CPB. Determination of risk factors for heparin resistance, and potentially, antithrombin replacement therapy, may individualize and improve anticoagulation treatment. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate management and outcomes of thrombosis after pediatric cardiac surgery and stratify thrombi according to risk of short- and long-term complications to better guide therapeutic choices.Study Design: Retrospective review was performed of 513 thrombi (400 occlusive) diagnosed after 213 pediatric cardiac operations. Long-term outcomes over time were assessed with the use of parametric hazard regression models.Results: Serious complications and/or high-intensity treatment occurred with 17%-24% of thrombi depending on location, most commonly in thrombi affecting the cardiac and cerebral circulation. Bleeding complications affected 13% of patients; associated factors included thrombolytics (OR 8.7, P < .001), greater daily dose of unfractionated heparin (OR 1.25 per 5 U/kg/day, P = .03), and extracorporeal support (OR 4.5, P = .007). Radiologic thrombus persistence was identified in 30% ± 3% at 12 months; associated factors included extracorporeal support (hazard ratio [HR] 1.9, P = .003), venous (HR 1.7, P = .003), and occlusive thrombi at presentation (HR 1.8, P = .001); greater oxygen saturation before surgery (HR 1.13/10%, P = .05) and thrombi in femoral veins (HR 1.9, P = .001) were associated with increased hazard of resolution. Freedom from postthrombotic syndrome was 83% ± 4% at 6 years, greater number of persistent vessel segment occlusions (HR 1.8/vessel, P = .001) and greater fibrinogen at diagnosis (HR 1.1 per g/L, P = .02) were associated with increased hazard.Conclusions: Thrombosis outcomes after pediatric cardiac surgery remain suboptimal. Given that more intensive treatment would likely increase the risk of bleeding, the focus should be on both thrombosis-prevention strategies, as well as in tailoring therapy according to a thrombosis outcome risk stratification approach. [ABSTRACT FROM AUTHOR]

Objective Heterozygous familial hypercholesterolemia (HeFH) is an autosomal dominant disorder leading to premature atherosclerosis. Guidelines recommend initiating statins early to reduce low-density lipoprotein cholesterol (LDL-C). Studies have evaluated rosuvastatin in children aged ≥10 years, but its efficacy and safety in younger children is unknown. Methods Children with HeFH and fasting LDL-C >4.92 mmol/L (190 mg/dL) or >4.10 mmol/L (>158 mg/dL) with other cardiovascular risk factors received rosuvastatin 5 mg daily. Based on LDL-C targets (<2.85 mmol/L [<110 mg/dL]), rosuvastatin could be uptitrated to 10 mg (aged 6–9 years) or 20 mg (aged 10–17 years). Treatment lasted 2 years. Changes in lipid values, growth, sexual maturation, and adverse events (AEs) were assessed. Results The intention-to-treat analysis included 197 patients. At 24 months, LDL-C was reduced by 43, 45, and 35% vs baseline in patients aged 6–9, 10–13, and 14–17 years, respectively ( P < .001 for all groups). Most AEs were mild. Intermittent myalgia was reported in 11 (6%) patients and did not lead to discontinuation of rosuvastatin treatment. Serious AEs were reported by 9 (5%) patients, all considered unrelated to treatment by the investigators. No clinically important changes in hepatic biochemistry were reported. Rosuvastatin treatment did not appear to adversely affect height, weight, or sexual maturation. Conclusions In HeFH patients aged 6–17 years, rosuvastatin 5–20 mg over 2 years significantly reduced LDL-C compared with baseline. Treatment was well tolerated, with no adverse effects on growth or sexual maturation. [ABSTRACT FROM AUTHOR]

Objectives To evaluate the lipid-altering efficacy and safety of ezetimibe monotherapy in young children with heterozygous familial hypercholesterolemia (HeFH) or nonfamilial hypercholesterolemia (nonFH). Study design One hundred thirty-eight children 6-10 years of age with diagnosed HeFH or clinically important nonFH (low-density lipoprotein cholesterol [LDL-C] ≥160 mg/dL [4.1 mmol/L]) were enrolled into a multicenter, 12-week, randomized, double-blind, placebo-controlled study. Following screening/drug washout and a 5-week single-blind placebo-run-in with diet stabilization, subjects were randomized 2:1 to daily ezetimibe 10 mg (n = 93) or placebo (n = 45) for 12 weeks. Lipid-altering efficacy and safety were assessed in all treated patients. Results Overall, mean age was 8.3 years, 57% were girls, 80% were white, mean baseline LDL-C was 228 mg/dL (5.9 mmol/L), and 91% had HeFH. After 12 weeks, ezetimibe significantly reduced LDL-C by 27% after adjustment for placebo (P < .001) and produced significant reductions in total cholesterol (21%), nonhigh-density lipoprotein cholesterol (26%), and apolipoprotein B (20%) (P < .001 for all). LDL-C lowering response in sex, race, baseline lipids, and HeFH/nonFH subgroups was generally consistent with overall study results. Ezetimibe was well tolerated, with a safety profile similar to studies in older children, adolescents, and adults. Conclusions Ezetimibe monotherapy produced clinically relevant reductions in LDL-C and other key lipid variables in young children with primary HeFH or clinically important nonFH, with a favorable safety/tolerability profile. [ABSTRACT FROM AUTHOR]

Background: Low weight is an established risk factor for mortality after congenital cardiac surgery. Given the advances in the care of neonates and infants after surgery, we sought to examine the effect of low weight on outcomes in the current era. Methods: From 2002 to 2012, 2051 infants aged 90 days or less underwent cardiac surgery including 534 (26.0%) with single-ventricle pathology. Regression models examined the effect of low weight (≤2.5 kg; n = 274, 13.4%) on early and late outcomes. Results: Overall, the incidence of prematurity, associated chromosomal/extracardiac abnormalities was higher in infants who weighed 2.5 kg or less than in those who weighed more than 2.5 kg; the incidence of single-ventricle pathology was comparable between the 2 groups. In addition, infants who weighed 2.5 kg or less underwent more palliation and had a higher proportion of STAT (Society of Thoracic Surgeons—European Association for Cardio-Thoracic Surgery) risk category 4 and 5 procedures. Adjusted regression models showed that low weight (≤2.5 kg) did not increase unplanned reoperation (odds ratio [OR], 0.90; 95% confidence interval [CI], 0.48-1.67; P = .73) or extracorporeal membrane oxygenation requirement (OR, 1.23; 95% CI, 0.68-2.22; P = .49), however it was associated with significant increase in hospital mortality (OR, 2.15; 95% CI, 1.33-3.50; P = .002). In addition, there was a significant association between low weight and increased duration of postoperative mechanical ventilation and intensive care unit and hospital stays. Adjusted hazard analysis showed that weight equal to or less than 2.5 kg was associated with diminished late survival (hazard ratio, 1.89; 95% CI, 1.39-2.55; P < .001) and that was evident in all patients subgroups (P < .001 for all). Conclusions: In a large single-center series, low weight continues to be associated with increased early mortality risk and resource utilization after palliative and corrective cardiac surgery. The hazard of death in low-weight patients continues beyond the perioperative period for at least 1 year before normalizing. Strategies to improve outcomes for this high-risk population must address perioperative care, outpatient surveillance, and management. [ABSTRACT FROM AUTHOR]

Objective: Despite improved long-term survival after pediatric heart transplantation, perioperative mortality has remained high. We sought to understand the factors associated with perioperative graft loss after pediatric heart transplantation. Methods: The factors associated with primary heart transplant mortality and retransplantation before hospital discharge in 226 pediatric heart transplant recipients (1995-2010) at a single-center institution were analyzed using multivariable logistic regression models adjusted for age at surgery and year of surgery. Results: A total of 26 patients died (n = 21) or underwent retransplantion (n = 5) before hospital discharge secondary to primary graft failure (n = 10), multisystem organ failure (n = 5), infection (n = 4), rejection (n = 2), and perioperative complications (n = 5). United Network for Organ Sharing status 1 (vs status 2) at transplantation was associated with an increased odds of death from noncardiac causes (odd ratio [OR], 4.7; 95% confidence level [CI], 1.2-22.3; P = .002). The factors associated with increased odds of perioperative mortality or retransplant were pre- and post-transplant extracorporeal membrane oxygenation (OR, 5.3; 95% CI, 1.5-18.7; P = .01; and OR, 25.9; 95% CI, 7.0-95.9; P < .001), longer ischemic times (OR, 1.4 per 30 minutes; 95% CI, 1.0-2.0; P = .04), reoperation after transplantation (OR, 3.5; 95% CI, 1.2-10.4; P = .02), and transplantation before 2002 (OR, 4.5; 95% CI, 1.4-14.9; P = .01), respectively. Conclusions: The use of extracorporeal membrane oxygenation (both before and after transplantation), a longer ischemic time, and reoperation were key factors associated with perioperative graft loss, with noncardiac mortality closely related to United Network for Organ Sharing status at heart transplantation. Knowledge of the perioperative risk factors and how they affect graft survival will help guide difficult decisions around eligibility, timing of primary listing, and appropriateness for retransplantation, and potentially affect long-term survival. [Copyright &y& Elsevier]