BACKGROUND: Hypertension in adolescence is associated with subclinical target organ injury. We aimed to determine whether different blood pressure thresholds were associated with an increasing number of target organ injury markers in healthy adolescents. METHODS: A total of 244 participants (mean age 15.5±1.8 years, 60.1% male) were studied. Participants were divided based on systolic clinic and systolic awake ambulatory blood pressure into low- (<75th percentile), mid- (75th–90th percentile), and high-risk (>90th percentile) groups. The ambulatory blood pressure phenotype was classified as normotensive, white-coat, masked, or sustained hypertension. Target organ injury assessments included left ventricular mass, systolic and diastolic function, and vascular stiffness. A multivariable general linear model was constructed to evaluate the association of different participant characteristics with higher numbers of target organ injury markers. RESULTS: A total of 31.2% of participants had 1, 11.9% 2, 3.7% 3, and 0.8% 4 target organ injury markers. The number of target organ injury markers increased according to the risk groups: the percentage of participants with >1 marker in the low-, mid-, and high-risk groups was 6.7%, 19.1%, and 21.8% ( P =0.02) and 9.6%, 15.8%, and 32.2% ( P <0.001), based on clinic and ambulatory blood pressure, respectively. Participants with white-coat (23%), masked (35%), and sustained hypertension (32%) had significantly higher >1 target organ injury marker than normotensives (8%, P <0.001). The results were unchanged in multivariate analysis. CONCLUSIONS: High clinic and ambulatory blood pressure values, as well as ambulatory blood pressure phenotypes (white-coat, masked, and sustained hypertension), were independently associated with an increasing number of subclinical cardiovascular injury markers in adolescents.

OBJECTIVE:To determine whether a specific blood pressure (BP) phenotype, based on clinic and 24-hour ambulatory BP (ABP), is associated with markers of target organ damage in otherwise healthy youth. STUDY DESIGN:In 373 adolescents enrolled in a cross-sectional study of the relationship of BP to cardiovascular risk (Study of High Blood Pressure in Pediatrics: Adult Hypertension Onset in Youth [SHIP-AHOY], clinic BP was measured by auscultation and categorized using the 2017 American Academy of Pediatrics guideline. ABP was measured for 24 hours by an oscillometric device and analyzed using the adult ABP wake systolic BP cut-point (130 mmHg), creating 4 BP phenotype groups: normal BP (n = 212), white coat hypertensive (n = 55), ambulatory hypertensive (n = 63), and masked hypertensive (n = 43). Echocardiographic measures and carotid-femoral pulse wave velocity were measured as surrogate markers of cardiovascular risk. RESULTS:LV mass was lowest in the normal BP group, whereas carotid-femoral pulse wave velocity and multiple measures of cardiac function were worse in the masked and ambulatory hypertensive groups. General linear models were constructed to examine the associations between BP phenotype and the cardiovascular risk measures. BP phenotype was significantly associated with diastolic function (E/e', e'/a'), systolic function (ejection fraction, peak longitudinal strain), and increased arterial stiffness after adjustment for body mass index percentile (all P ≤ .05). CONCLUSIONS:BP phenotype is independently associated with surrogate markers of increased cardiovascular risk in adolescents, including impaired cardiac function and increased vascular stiffness. ABP monitoring has an important role in cardiovascular risk assessment in youth.

Ambulatory hypertension is associated with elevated left ventricular mass index (LVMI), cardiac dysfunction, and increased arterial stiffness in adolescents. Whether the addition of measures of BP variability improves the prediction of subclinical cardiovascular target organ damage (TOD) over mean BP measures is not known. We assessed clinic and ambulatory BP (ABP), anthropometrics, and TOD in 397 adolescents. ABP means standard deviation (SD), BP, and heart rate (HR) dipping were calculated; coefficients of variability (CV) were calculated (SD/mean) to assess ABP variabilities. Measures of TOD included LVMI, left ventricular hypertrophy (LVH), LV systolic shortening, LV diastolic function (e′/a′), and pulse wave velocity. General linear models were used to determine if increased ABP variability measures were significant determinants of TOD in models containing mean ABP percentiles, age, sex, race/ethnicity, BMI z-score, and HR. Mean participant age was 15.6 ± 1.7 years (63

Background:Hypertension in adolescence is associated with subclinical target organ injury (TOI). We aimed to determine whether different blood pressure (BP) thresholds were associated with increasing number of TOI markers in healthy adolescents. Methods:244 participants (mean age 15.5±1.8 years, 60.1% male) were studied. Participants were divided based on both systolic clinic and ambulatory BP (ABP), into low- (<75 th percentile), mid- (75 th -90 th percentile) and high-risk (>90 th percentile) groups. TOI assessments included left ventricular mass, systolic and diastolic function, and vascular stiffness. The number of TOI markers for each participant was calculated. A multivariable general linear model was constructed to evaluate the association of different participant characteristics with higher numbers of TOI markers. Results:47.5% of participants had at least one TOI marker: 31.2% had one, 11.9% two, 3.7% three, and 0.8% four. The number of TOI markers increased according to the BP risk groups: the percentage of participants with more than one TOI in the low-, mid-, and high groups based on clinic BP was 6.7%, 19.1%, and 21.8% (p=0.02), and based on ABP was 9.6%, 15.8%, and 32.2% (p<0.001). In a multivariable regression analysis, both clinic BP percentile and ambulatory SBP index were independently associated with the number of TOI markers. When both clinic and ABP were included in the model, only the ambulatory SBP index was significantly associated with the number of markers. Conclusion:High SBP, especially when assessed by ABPM, was associated with an increasing number of subclinical cardiovascular injury markers in adolescents.

Background: This study aimed to assess the implementation and access to telehealth-delivered pediatric weight management (PWM) during the initial phase of the COVID-19 pandemic at six US PWM programs (PWMP) using the RE-AIM (Reach, Effectiveness, Adoption, Implementation, Maintenance) framework. Methods: The COVID-19 period (COVID) was defined in this retrospective, multisite study as the time when each site closed in-person care during 2020. The Pre-COVID period (Pre-COVID) was an equivalent time frame in 2019. Patients were stratified by visit completion status. Patient characteristics for COVID and Pre-COVID were compared to examine potential changes/disparities in access to care. Results: There were 3297 unique patients included across the six sites. On average, telehealth was initiated 4 days after in-person clinic closure. Compared with Pre-COVID, COVID (mean duration: 9 weeks) yielded fewer total completed visits (1300 vs. 2157) and decreased revenue (mean proportion of nonreimbursed visits 33.30% vs. 16.67%). Among the completed visits, COVID included a lower proportion of new visits and fewer patients who were male, non-English speaking, Hispanic, or Asian and more patients who were Black or lived ≥20 miles from the program site (p < 0.05 for all). Among no-show/canceled visits, COVID included more patients who had private insurance, older age, or a longer time since the last follow-up. Conclusion: Rapid implementation of telehealth during COVID facilitated continuity of PWM care. Clinic volume and reimbursement were lower during COVID and differences in the patient population reached by telehealth emerged. Further characterization of barriers to telehealth for PWM is needed.

Therapeutic Area: ASCVD/CVD Risk Factors Background: Obesity-related cardiovascular (CV) risk factors are associated with increased left ventricular mass, a risk factor for adult CV disease. Whether there are race differences in this relationship is not well described. Our aim was to utilize an existing longitudinal data set of black and white females to evaluate racial differences in the impact of CV risk factors on adiposity measured by different modalities and to determine the contribution of adiposity to increased LVM. Methods: Data from the National Growth and Health Study (NGHS), a study of racial differences in the development of obesity, were analyzed including demographics, medical history, anthropometrics, dual energy X-ray absorptiometry (DEXA), MRI (for abdominal subcutaneous and visceral fat), echocardiogram and laboratory data. NGHS enrolled 871 girls at age 9 or 10, 50% black and 50% white. In these analyses, data collected at the time of the patient's first DEXA scan (at age 20-21 years) and LVM index (LVMI) collected from the patient's last echocardiogram (at ages 24-27) were analyzed. Means and standard deviations were calculated for the total population and then stratified by race. T-tests were performed to assess for significant difference between races. Results: At baseline, black subjects had significantly higher weight, BMI, waist circumference, total fat mass by DEXA, subcutaneous fat mass by MRI, blood pressure, insulin, and glucose values. White subjects were found to have higher fasting triglyceride and total cholesterol values. LVMI was significantly greater in black subjects. Conclusions: Significant racial differences in CV risk factors and LVMI were found in young adulthood. Future analyses will address the contribution of central and peripheral adiposity measured by different modalities to these differences.

BACKGROUND: Functional status predicts waitlist survival in adult heart transplantation and is an inde-pendent predictor of outcomes in pediatric liver transplantation. This has not been studied in pediatric heart transplantation. Study aims were to determine the association of: (1) functional status at listing with waitlist and post-transplant outcomes, and (2) functional status at transplant with post-transplant outcomes in pediatric heart transplantation.METHODS: Retrospective United Network of Organ Sharing database study of pediatric patients listed for heart transplant between 2005 and 2019 with Lansky Play Performance Scale (LPPS) scores at list -ing. Standard statistical methods were used to assess relationships between LPPS and outcomes (wait-list and post-transplant). Negative waitlist outcome was defined as death or removal from waitlist due to clinical deterioration.RESULTS: There were 4,169 patients identified, including 1,080 with LPPS 80-100 (normal activity), 1,603 with LPPS 50-70 (mild limitations), and 1,486 with LPPS 10-40 (severe limitations). LPPS 10-40 correlated with negative waitlist outcomes (HR 1.69, CI 1.59-1.80, p < 0.0001). While LLPS at list -ing had no association with post-transplant survival, those with LPPS 10-40 at transplant had inferior 1-year post-transplant survival compared to those with LPPS & GE;50 (92% vs 95%-96%, p = 0.0011). Functional status was an independent predictor of post-transplant outcomes in patients with cardiomy-opathy. A functional improvement of & GE;20 points between listing and transplant (N = 770, 24%) was associated with higher 1-year post-transplant survival (HR 1.63, 95% CI: 1.10-2.41, p = 0.018).CONCLUSIONS: Functional status is associated with waitlist and post-transplant outcomes. Interventions targeting functional impairment may improve pediatric heart transplantation outcomes. J Heart Lung Transplant 2023;42:964-973 & COPY; 2023 International Society for Heart and Lung Transplantation. All rights reserved.

The growth hormone and insulin-like growth factor (IGF) system is integral to human growth. Genome-wide association studies (GWAS) have identified variants associated with height and located near the genes in this pathway. However, mechanisms underlying these genetic associations are not understood. To investigate the regulation of the genes in this pathway and mechanisms by which regulation could affect growth, we performed GWAS of measured serum protein levels of IGF-I, IGF binding protein-3 (IGFBP-3), pregnancy-associated plasma protein A (PAPP-A2), IGF-II and IGFBP-5 in 838 children (3-18 years) from the Cincinnati Genomic Control Cohort. We identified variants associated with protein levels near IGFBP3 and IGFBP5 genes, which contain multiple signals of association with height and other skeletal growth phenotypes. Surprisingly, variants that associate with protein levels at these two loci do not colocalize with height associations, confirmed through conditional analysis. Rather, the IGFBP3 signal (associated with total IGFBP-3 and IGF-II levels) colocalizes with an association with sitting height ratio (SHR); the IGFBP5 signal (associated with IGFBP-5 levels) colocalizes with birth weight. Indeed, height-associated single nucleotide polymorphisms near genes encoding other proteins in this pathway are not associated with serum levels, possibly excluding PAPP-A2. Mendelian randomization supports a stronger causal relationship of measured serum levels with SHR (for IGFBP-3) and birth weight (for IGFBP-5) than with height. In conclusion, we begin to characterize the genetic regulation of serum levels of IGF-related proteins in childhood. Furthermore, our data strongly suggest the existence of growth-regulating mechanisms acting through IGF-related genes in ways that are not reflected in measured serum levels of the corresponding proteins.

The Fontan procedure (FP) is typically a semi-elective surgery performed between 2 and 5 years of age to complete staged single ventricle palliation. Optimal timing for the FP, particularly in relation to seasonal infectious burden, remains unclear. We queried the Pediatric Health Information System (PHIS) database for all admissions for viral respiratory infections (VRI) from January 2006 to September 2015 and separately for all admissions with a primary procedure code of FP. The PHIS query generated 2,767,142 admissions for VRI and 6349 admissions for the FP from 45 children’s hospitals. Of all FP, 2124 (33.5%) were performed from October through March. The median length of stay after Fontan procedure was 9 days (IQR 7–15). Median length of stay after FP was correlated with VRI burden (correlation coefficient = 0.3, p = 0.03). April through August (weeks 18 through 35) had the lowest VRI admission burden and FP length of stay was significantly shorter during this time (13.6 ± 14.8 days vs 14.9 ± 20.3 days, p = 0.03). The FP is frequently performed during the viral respiratory season. This timing is associated with an increased post-operative length of stay after the FP. For elective FP, ideal timing that avoids the viral respiratory season and minimizes post-operative LOS is April through August.

This study examined Morbidity and Mortality (M&M) review practices and perspectives of physicians and parents regarding parent participation in M&M review. Surveys were distributed to parents of children with a prior hospitalization for congenital heart disease (CHD) and physicians caring for pediatric CHD patients. Response distributions and Fisher's exact tests were performed to compare parent and physician responses. Qualitative survey data were thematically analyzed. Ninety-two parent and 36 physician surveys were analyzed. Physicians reported parent input or participation was rarely sought in M&M review. Parents with direct experience of adverse events or death of their child reported providers discussed events with them in a timely manner and answered their questions; however, nearly half wished their healthcare team had done something differently during the disclosure. There was no statistical difference between groups regarding transparency (P = .37, .79); however, there was a significant difference in perspectives regarding parental involvement in the M&M review (P < .00 1). Common themes important to parents which emerged from the qualitative analysis were being adequately informed, feeling their perspectives were acknowledged and respected, having attentive and empathetic providers, and receiving consistent messaging. Although rarely included in current practice, parent participation in M&M could offer unique insight and increase accountability to proposed change elucidated by M&M review.

Background In Fontan circulation, diastolic dysfunction portends a worse clinical outcome but may be concealed during routine assessment. Invasive evaluation with rapid volume expansion (RVE) can identify patients with occult diastolic dysfunction (ODD). We sought to evaluate the association between ODD and adverse clinical outcomes at medium‐term follow‐up. Methods and Results We conducted a single‐center observational study of patients with Fontan circulation who underwent clinical catheterization with RVE from 2012 to 2017. ODD was defined as post‐RVE end‐diastolic pressure ≥15 mm Hg. A composite adverse clinical outcome included mortality, cardiac transplant, ventricular assist device, plastic bronchitis, protein‐losing enteropathy, arrhythmia, stroke/thrombus, or cardiac‐related hospital admission. Proportional hazards regression was used to compare the ODD‐positive and ODD‐negative groups for risk of the composite adverse clinical outcome. Eighty‐nine patients with Fontan circulation (47% female patients) were included at a median age of 14 years. ODD was identified in 31%. Fontan duration was longer in the ODD group ( P =0.001). The composite adverse clinical outcome occurred more frequently in the ODD group (52 versus 26%, P =0.03) during a median follow‐up duration of 2.9 years after catheterization. ODD (hazard ratio [HR], 2.68 [95% CI, 1.28–5.66]; P =0.02) and Fontan duration (HR, 1.07 [95% CI, 1.02–1.12]; P =0.003) were associated with the composite adverse clinical outcome. When stratified by Fontan duration, ODD remained significantly associated with the hazard of adverse clinical outcomes in patients with a Fontan duration ≥10 years (HR, 2.57 [95% CI, 1.03–6.57]; P =0.04). Conclusions Cardiac catheterization with rapid volume expansion reveals a significant incidence of ODD, which relates to Fontan duration. ODD is associated with an increased hazard of adverse clinical outcomes during medium‐term follow‐up, especially in patients with longer Fontan duration. ODD may portend a worse prognosis in Fontan circulation.

Objective To evaluate the impact of the 2017 American Academy of Pediatrics hypertension Clinical Practice Guideline (CPG), compared with the previous guideline ("Fourth Report"), on the frequency of hypertensive blood pressure (BP) measurements in childhood and associations with hypertension in adulthood using data from the International Childhood Cardiovascular Cohort Consortium. Study design Childhood BPs were categorized in normal, prehypertensive/elevated, and hypertensive (stage 1 and 2) ranges using the Fourth Report and the CPG. Participants were contacted in adulthood to assess self-reported hypertension. The associations between childhood hypertensive range BPs and self-reported adult hypertension were evaluated. Results Data were available for 34 014 youth (10.4 +/- 3.1 years, 50.6% female) with 92 751 BP assessments. Compared with the Fourth Report, the CPG increased hypertensive readings from 7.6% to 13.5% and from 1.3% to 2.5% for stage 1 and 2 hypertensive range, respectively (P <.0001). Of 12 761 adults (48.8 +/- 7.9 years, 43% male), 3839 (30.1%) had self-reported hypertension. The sensitivity for predicting adult hypertension among those with hypertensive range BPs at any point in childhood, as defined by the Fourth Report and the CPG, respectively, was 13.4% and 22.4% (specificity 92.3% and 85.9%, P <.001), with no significant impact on positive and negative predictive values. Associations with self-reported adult hypertension were similar and weak (c-statistic range 0.61-0.68) for hypertensive range BPs as defined by the Fourth Report and CPG. Conclusions The CPG significantly increased the prevalence of childhood BPs in hypertensive ranges and improved the sensitivity, without an overall strengthened association, of predicting self-reported adult hypertension.

Introduction: Excess visceral fat is implicated in obesity-related cardiometabolic disease, but normal age, sex, and ancestry-related trends in visceral fat among youth with varying body size and metabolic health status are unknown. Objective: The objective of this study was to characterize age, sex, and ancestry-related trends in abdominal visceral fat among youth with healthy weight, obesity, and type 2 diabetes. Methods: This cross-sectional sample included youth ages 10 to 23 years (67% female, 56% non-black) with healthy weight (BMI=5 th to 85 th percentile, n=236), obesity (BMI > 95 th percentile, n=224), and type 2 diabetes (BMI > 95 th percentile, n=145). Visceral fat (cm 2 ) was assessed via dual-energy X-ray absorptiometry. Multiple linear regression analyses were performed to examine interaction and main effects of age, sex, and ancestry in relation to visceral fat (log) in the three groups separately. Results: Age was positively associated with visceral fat in the healthy weight (P<0.001), obese (P<0.001), and type 2 diabetes groups (P=0.051), such that visceral fat was greater in later adolescents and young adults compared to younger children. Non-blacks had significantly greater visceral fat compared to blacks in the healthy weight (P=0.003), obesity (P<0.001), and type 2 diabetes groups (P<0.001). Sex differences in visceral fat differed by group. In the healthy weight group, males had significantly greater visceral fat than females, but in the obesity group, females had greater visceral fat than males (both P<0.001). In the type 2 diabetes group, females tended to have greater visceral fat, but this difference did not reach statistical significance (P=0.060). When accounting for BMI, significant sex and ancestry differences were maintained, but age associations were nullified. Conclusions: This study reports novel findings with respect to demographic determinants of visceral fat assessed via DXA in children, adolescents, and young adults. Our findings that sex differences in visceral fat in youth with obesity and type 2 diabetes persisted following control for BMI are novel and of clinical interest. Specifically, that females may accrue greater visceral adiposity with weight gain when compared to their male counterparts might help to explain differing risk for obesity-related chronic disease. Prospective studies of youth during critical periods of growth are needed to define the consequences of excess visceral fat accrual with respect to future cardiometabolic risk.

OBJECTIVES:Near-infrared spectroscopy (NIRS) has been increasingly accepted as a noninvasive marker of regional tissue oxygenation despite concerns of imprecision and wide limits of agreement (LOA) with invasive oximetry. New generation absolute monitors may have improved accuracy compared with trend monitors. We sought to compare the concordance with invasive venous oximetry of a new generation absolute NIRS-oximeter (FORESIGHT ELITE; CASMED, Branford, CT) with a modern widely used trend monitor (INVOS 5100C; Medtronic, Minneapolis, MN). DESIGN:Prospective single-center study. SETTING:Tertiary pediatric heart center. PATIENTS:Children undergoing elective cardiac catheterization under general anesthesia. Time-paired venous oximetry samples (jugular and renal) were compared with NIRS-derived oximetry by two monitors using regression and Bland-Altman analysis. INTERVENTIONS:None. MEASUREMENTS AND MAIN RESULTS:We enrolled 36 children (19 female, 10 cyanotic) with median age 4.1 years (25-75%, 2.5-7.8 yr) and weight 16.7 kg (12.3-29.1 kg). The absolute difference between NIRS-derived and invasive jugular oximetry was less than 10% in 67% of occasions for both monitors. Correlation was fair (Spearman r(s) = 0.40; p = 0.001) for the FORESIGHT ELITE and poor (r(s) = 0.06; p = 0.71) for the INVOS 5100C. Bias and LOA were +6.7% (+22%, -9%) versus +1.3% (LOA = +24%, -21%), respectively. The absolute difference between NIRS-derived and invasive renal oximetry was less than 10% in 80% of occasions with moderate correlation (r(s) = 0.57; p < 0.001) for the FORESIGHT ELITE and in 61% of occasions with moderate correlation (r(s) = 0.58; p < 0.001) for the INVOS 5100C; bias and LOA were +3.6% (+19%, -12%) and -1.4 % (+27%, -30%), respectively. NIRS correlation with renal venous oximetry was worse for cyanotic versus noncyanotic patients (p = 0.02). CONCLUSIONS:Concordance and LOA of NIRS-derived oximetry with invasive venous oximetry in the cerebral and renal vascular beds was suboptimal for clinical decision-making. Cyanosis adversely affected NIRS performance in the renal site.

Background: Hypertension-related increased arterial stiffness predicts development of target organ damage (TOD) and cardiovascular disease. We hypothesized that blood pressure (BP)–related increased arterial stiffness is present in youth with elevated BP and is associated with TOD. Methods: Participants were stratified by systolic BP into low- (systolic BP <75th percentile, n=155), mid- (systolic BP ≥80th and <90th percentile, n=88), and high-risk BP categories (≥90th percentile, n=139), based on age-, sex- and height-specific pediatric BP cut points. Clinic BP, 24-hour ambulatory BP monitoring, anthropometrics, and laboratory data were obtained. Arterial stiffness measures included carotid-femoral pulse wave velocity and aortic stiffness. Left ventricular mass index, left ventricular systolic and diastolic function, and urine albumin/creatinine were collected. ANOVA with Bonferroni correction was used to evaluate differences in cardiovascular risk factors, pulse wave velocity, and cardiac function across groups. General linear models were used to examine factors associated with arterial stiffness and to determine whether arterial stiffness is associated with TOD after accounting for BP. Results: Pulse wave velocity increased across groups. Aortic distensibility, distensibility coefficient, and compliance were greater in low than in the mid or high group. Significant determinants of arterial stiffness were sex, age, adiposity, BP, and LDL (low-density lipoprotein) cholesterol. Pulse wave velocity and aortic compliance were significantly associated with TOD (systolic and diastolic cardiac function and urine albumin/creatinine ratio) after controlling for BP. Conclusions: Higher arterial stiffness is associated with elevated BP and TOD in youth emphasizing the need for primary prevention of cardiovascular disease.

Objective: The aim of this study was to examine the association of sodium intake (g/day) and sodium load (Na-L; mg/kcal/day) on arterial stiffness in youth. Methods: A cross-sectional analysis of 723 youth enrolled in a study evaluating the cardiovascular effects of obesity and type 2 diabetes mellitus (T2DM). Three measures of arterial stiffness were evaluated: brachial distensibility (BrachD), carotid-femoral pulse wave velocity (PWVcf) and augmentation index (AIx). Three-day diet histories yielded mean daily sodium and calorie intake. Youth were divided into Na-L tertiles: Low (<= 1.67 mg/kcal per day); Medium (1.68--1.98 mg/kcal per day) and High (>1.98 mg/kcal per day). General linear models adjusting for demographics, % body fat, T2DM and SBP z-score evaluated the independent association of Na-L with arterial stiffness. Results: Mean age was 17.9 years (10-24 years), 35% male, 59% nonwhite and 31% T2DM. Mean (+/- standard deviation) dietary intake was calories = 2074 (+/- 797) kcal/day; Na = 3.793 (+/- 1567) g/day; Na- = 1.86 (+/- 0.753) mg/kcal per day. With higher levels of dietary Na intake and Na-L, measures of arterial stiffness worsened: BrachD decreased (Na intake: beta = -0.09, P = 0.003; Na-L: beta = -0.28, P < 0.0001), PWVcf increased (Na intake: beta = 0.07, P = 0.007; Na-L: beta = 0.21, P < 0.0001) but AIx did not change (Na intake: beta = -0.4, P = 0.2; Na-L: beta = 0.89, P = 0.11). In multivariable analysis, High Na-L was independently associated with BrachD, PWVcf and AIx (P < 0.05 for all), with age modifying the association of High Na-L with PWVcf and AIx. Conclusion: Sodium intake and load are associated with arterial stiffness, a preclinical measure of CVD, among a paediatric population. Paediatricians should stress healthy dietary choices to reduce accelerated vascular ageing.

BACKGROUND Arrhythmias are common in single ventricle patients though their effect on outcomes during stage I palliation (S1P) is unclear. OBJECTIVES The authors sought to study associated risks for arrhythmia in patients undergoing S1P for single ventricle disease and evaluate the outcome of arrhythmias and their treatment strategies on survival. METHODS Retrospective patient, surgical, medication, and arrhythmia data were obtained from the NPC-QIC (National Pediatric Cardiology Quality Improvement Collaborative) database. Bivariate analysis of variables associated with arrhythmias, as well as those associated with survival, was performed at the time of stage II palliation. Appropriate variables were included in multivariate modeling. RESULTS Of the 2,048 patients included in the study, 36% had arrhythmia noted during their S1P hospitalization, with supraventricular tachycardia (12%) and focal atrial tachycardia (11%) the most common. At S1P discharge, 11% of patients were on an antiarrhythmic medication. Arrhythmias were associated with lower survival and increased hospital length of stay. Heterotaxy syndrome, younger age at S1P, male sex, and additional anomalies were associated with increased risk of arrhythmia in multivariable modeling (P <= 0.01). Arrhythmia and female sex were associated with increased mortality, whereas antiarrhythmic medication and digoxin use were associated with decreased mortality (P <= 0.003, model area under the curve 1/4 0.79). The use of antiarrhythmic medications within the subcohort of arrhythmia patients was also associated with decreased risk of mortality (P < 0.0001; odds ratio: 2.0-7.2). CONCLUSIONS Arrhythmias are common during admission for S1P and associated with poor outcomes. The use of antiarrhythmic medications may improve survival, though future studies are needed.

Background: Increased arterial stiffness is associated with diastolic dysfunction in adults. Data in youth are lacking, so we examined the impact of arterial stiffness on diastolic function in youth.Methods: We obtained diastolic function and augmentation index, pulse wave velocity, brachial artery distensibility, and carotid stiffness on 612 youth [10–24 years, 65% female, 38% normal weight, 36% obese, and 26% with type 2 diabetes mellitus (T2DM)]. Participants were classified as compliant (C) vs. stiff (S) arteries based on seven arterial stiffness parameters [Global Stiffness Index (GSI), S = GSI > 4). Mean differences in covariates were evaluated by Student's t-tests. A stepwise regression analysis was performed to determine if GSI was an independent predictor of diastolic function.Results: Lower diastolic function and more adverse cardiovascular disease (CVD) risk factors were present in the S group (n = 67) than the C group (n = 545) (p < 0.001). Covariates that were associated with diastolic dysfunction were higher GSI, male sex, higher body mass index (BMI), and systolic blood pressure (SBP) z-score (R2 = 0.18 to 0.25; p ≤ 0.05).Conclusion: Adverse diastolic function is seen in youth with increased arterial stiffness independent of CVD risk factors. Interventions to improve arterial stiffness prior to clinical onset of diastolic dysfunction are needed to prevent development of heart failure.

Background Patients with Duchenne muscular dystrophy (DMD) develop cardiomyopathy because of a dystrophin deficiency causing fibrofatty replacement of the myocardium. Corticosteroid use and mobility limitations place these patients at risk for increased adiposity. We sought to determine the association of adiposity with cardiovascular dysfunction in patients with DMD. Methods and Results This was a retrospective review of patients with DMD who underwent both cardiac magnetic resonance imaging and dual‐energy x‐ray absorptiometry within 1 year. The cardiac magnetic resonance imaging parameters included left ventricular ejection fraction and the presence of late gadolinium enhancement (LGE positive [LGE+]). The adiposity indices, measured by dual‐energy x‐ray absorptiometry, included percentage of body fat, whole body fat mass indexed to height, and body mass index. A total of 324 patients were identified. Fifty‐two percent had LGE+, and 36% had cardiac dysfunction (left ventricular ejection fraction <55%). Patients with cardiac dysfunction had higher whole body fat mass indexed to height and body mass index on univariate analysis (mean difference between patients with and without cardiac dysfunction: +2.9 kg/m, P=0.001; and +1.5 kg/m2, P=0.03, respectively). whole body fat mass indexed to height remained independently associated with cardiac dysfunction on multivariable analysis after adjusting for age, LGE+, and corticosteroid duration. High whole body fat mass indexed to height and percentage of body fat were associated with LGE+ on univariate analysis (mean difference between patients with and without LGE+: +2.0 kg/m, P=0.02; and +2.4%, P=0.02, respectively). Using multivariable analysis, including age and cardiac dysfunction, high percentage of body fat remained independently associated with LGE+. Conclusions This study demonstrates an independent association of adiposity with cardiac dysfunction and LGE+ in patients with DMD. Preventing adiposity may mitigate the later development of ventricular dysfunction in DMD.