Ambulatory Blood Pressure and Number of Subclinical Target Organ Injury Markers in Youth: The SHIP AHOY Study.

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.

Evaluation of the use of visual storytelling as an educational intervention in the cardiac ICU: reaching parents before they are in crisis.

OBJECTIVE: To evaluate the acceptability and safety of educational videos utilising visual storytelling to provide information about the cardiac ICU and post-operative care to parents. Videos were designed to educate, further encourage parents to engage in their child's cardiac care, and address common sources of distress. STUDY DESIGN: Two educational videos and survey were sent to 29 families of children previously admitted to the cardiac ICU (April 2020-March 2021). Views regarding information quality, quantity, format, and relevance were assessed, as were parents' emotional responses. Quantitative thresholds for safety and acceptability were set a priori. An inductive approach to content analysis was applied to identify themes in qualitative data. RESULTS: Sixteen parents participated (response rate: 55%). All acceptability and safety thresholds were met; 92% of parents rated the videos as helpful and 85% were "very" or "extremely likely" to recommend them to other families of children with CHD. No participants reported significant distress after viewing the videos. Expressions of parental engagement with their child's care team were common (92%). In qualitative responses, parents perceived the videos as potentially helpful in reducing distress if viewed prior to cardiac ICU admission. CONCLUSION: Visual storytelling to orient parents to the cardiac ICU and address common stressors was found to be safe and acceptable when tested with parents of children previously admitted to the cardiac ICU. Further prospective studies are needed to test intervention effects when videos are viewed before or during cardiac ICU admission, especially for mitigating anxiety and traumatic stress associated with admission.

Telehealth Use in a National Pediatric Weight Management Sample During the COVID-19 Pandemic.

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.

Functional status predicts pediatric heart transplant outcomes: A united network for organ sharing (UNOS) database study.

BACKGROUND: Functional status predicts waitlist survival in adult heart transplantation and is an independent 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 listing. Standard statistical methods were used to assess relationships between LPPS and outcomes (waitlist 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 listing 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 ≥50 (92% vs 95%-96%, p = 0.0011). Functional status was an independent predictor of post-transplant outcomes in patients with cardiomyopathy. A functional improvement of ≥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.

Absolute Versus Relative Near-Infrared Spectroscopy in Pediatric Cardiac Patients.

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 rs = 0.40; p = 0.001) for the FORESIGHT ELITE and poor ( rs = 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 ( rs = 0.57; p < 0.001) for the FORESIGHT ELITE and in 61% of occasions with moderate correlation ( rs = 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.

Association Between Seasonal Fontan Timing, Viral Infection Burden, and Post-Operative Length of Stay.

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.

Predictors and Outcomes of Arrhythmia on Stage I Palliation of Single Ventricle Patients.

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 = 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.

Association of Blood Pressure-Related Increase in Vascular Stiffness on Other Measures of Target Organ Damage in Youth.

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.

Continuous Arrhythmia Monitoring in Pediatric and Adult Patients With Left Ventricular Noncompaction.

Patients with left ventricular noncompaction (LVNC) are at risk of clinically significant arrhythmias and sudden death. We evaluated whether implantable loop recorders could detect significant arrhythmias that might be missed in these patients during annual Holter monitoring. Selected pediatric and adult patients with LVNC who consented to implantable loop recorder placement were monitored for 3 years (study duration, 10 April 2014-9 December 2019). Fourteen subjects were included (age range, 6.5-36.4 yr; 8 males). Of 13 patients who remained after one device extrusion, one underwent implantable cardioverter-defibrillator placement. Four patients (31%) had significant arrhythmias: atrial tachycardia (n=2), nonsustained ventricular tachycardia (n=1), and atrial fibrillation (n=1). All 4 events were clinically asymptomatic and not associated with left ventricular ejection fraction. In addition, a high frequency of benign arrhythmic patterns was detected. Implantable loop recorders enable continuous, long-term detection of important subclinical arrhythmias in selected patients who have LVNC. These devices may prove to be most valuable in patients who have LVNC and moderate or greater ventricular dysfunction.

Visceral fat and arterial stiffness in youth with healthy weight, obesity, and type 2 diabetes.

BACKGROUND AND OBJECTIVES: Visceral fat is associated with increased cardiovascular risk in adults, but studies in youth are limited. We assessed associations between visceral fat and arterial stiffness in youth with healthy weight, obesity and type 2 diabetes and determined whether relationships were independent of clinical estimates of body fatness. METHODS: This cross-sectional sample included youth ages 10-23 years (67% female, 56% non-black) with healthy weight (body mass index [BMI] = 5th-85th percentile, n = 236), obesity (BMI ≥ 95th percentile, n = 224) and type 2 diabetes (BMI ≥ 95th percentile, n = 145). Visceral fat was assessed via dual-energy X-ray absorptiometry. Carotid-femoral pulse wave velocity (PWV) was assessed via applanation tonometry. Obesity and type 2 diabetes groups were combined for final analyses. Analyses accounted for age, sex, ancestry and mean arterial pressure. RESULTS: Visceral fat and PWV were greater in youth with obesity versus healthy weight (p < 0.001). In youth with obesity, but not healthy weight, visceral fat was positively associated with PWV (p < 0.001) and was predictive of PWV beyond BMI and waist circumference. CONCLUSIONS: Visceral fat likely contributes to subclinical cardiovascular complications in youth. Since cardiovascular health tracks from adolescence to adulthood, longitudinal studies in youth with obesity are required to define the role of visceral fat in lifelong cardiovascular disease risk.

Dietary sodium intake and sodium load is associated with arterial stiffness in children and young adults.

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.

Increased Arterial Stiffness Is Associated With Reduced Diastolic Function in Youth With Obesity and Type 2 Diabetes.

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 (R 2 = 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.

Direct and indirect effects of obesity on progression of carotid arterial injury in youth.

OBJECTIVE: Increased carotid intima-media thickness (cIMT) is associated with cardiovascular (CV) events in adults and with elevated CV risk factors in youth. The aim of this study was to determine whether obesity has an direct or indirect association with cIMT. METHODS: Structural equation modeling was used to elucidate pathways for obesity to change cIMT. Complete data were collected twice on 294 participants (mean age 17.5 years, 16% with type 2 diabetes). Latent baseline and follow-up cIMT, BMI, and CV risk factors were analyzed with SAS 9.4. Model fit was assessed. RESULTS: There were increases in BMI, mean arterial pressure (MAP), low-density lipoprotein cholesterol, triglycerides, high-density lipoprotein (HDL) cholesterol, C-reactive protein, and glucose and a decrease in insulin and glycosylated hemoglobin over 5 years (all p < 0.05). Only bulb (0.031 mm) and internal (0.027 mm) cIMT increased (all p < 0.001). Significant direct effects on cIMT change were baseline MAP (ß 0.23), BMI z score (ß 0.16), change in glucose (ß 0.37), and age (ß 0.37; all p < 0.05). Change in MAP showed a trend (ß 0.14, p = 0.10). BMI also had a significant indirect effect (ß 0.17), whereas non-HDL demonstrated no significant effect. CONCLUSIONS: Baseline adiposity drives increasing blood pressure and glucose in high-risk youth leading to accelerated accumulation of carotid arterial injury. Prevention of acquisition of obesity in youth is critical in slowing development of CV disease.

The Effect of Adiposity on Cardiovascular Function and Myocardial Fibrosis in Patients With Duchenne Muscular Dystrophy.

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.

Diet Quality and Bone Density in Youth with Healthy Weight, Obesity, and Type 2 Diabetes.

PURPOSE: To assess relationships between diet quality and areal bone mineral density (aBMD) in youth with healthy weight, obesity, and type 2 diabetes (T2D). METHODS: We performed a secondary analysis of cross-sectional data from youth (55% African American, 70% female) ages 10-23 years with T2D (n = 90), obesity (BMI > 95th; n = 128), or healthy weight (BMI < 85th; n = 197). Whole body (less head) areal bone mineral density (aBMD) was assessed by dual-energy X-ray absorptiometry (DXA). aBMD was expressed as age-, sex-, and ancestry-specific standard deviation scores (Z-scores). Whole body aBMD Z-scores were adjusted for height-for-age Z-score. Diet was assessed via three-day diaries, and the Healthy Eating Index (HEI) was computed. Total HEI score and HEI subcomponent scores were compared across groups, and associations with aBMD Z-scores were assessed via linear regression adjusted for group, age, sex, and ancestry. RESULTS: Mean HEI was similar between the healthy weight, obesity, and T2D groups. Several HEI sub-components differed between groups, including meats and beans, total vegetables, milk, saturated fat, sodium, oils, and empty calories. The obesity and T2D group had significantly greater aBMD Z-scores compared to the healthy weight group. Multiple linear regression analyses revealed a significant positive association between HEI and aBMD Z-score (p < 0.05). The HEI sub-components for whole grains (p = 0.052) and empty calories (p < 0.05) were positively associated with aBMD Z-score. CONCLUSIONS: Individuals that followed a dietary pattern more closely aligned with the Dietary Guidelines for Americans had greater bone density. Since few studies have investigated the role of diet on bone in youth with obesity-related conditions, additional research is required among these populations.

Predictors and outcomes of heart block during surgical stage I palliation of patients with a single ventricle: A report from the NPC-QIC.

BACKGROUND: Mortality in cohorts with a single ventricle remains high with multiple associated factors. The effect of heart block during stage I palliation remains unclear. OBJECTIVE: The purpose of this study was to study patient and surgical risks of heart block and its effect on 12-month transplant-free survival in patients with a single ventricle. METHODS: Patient, surgical, outcome data and heart block status (transient and permanent) were obtained from the National Pediatric Cardiology Quality Improvement Collaborative single ventricle database. Bivariate analysis was performed comparing patients with and without heart block, and multivariate modeling was used to identify variables associated with block. One-year outcomes were analyzed to identify variables associated with lower 12-month transplant-free survival. RESULTS: In total, 1423 patients were identified, of whom 28 (2%) developed heart block (second degree or complete) during their surgical admission. Associated risk factors for block included heterotaxy syndrome (odds ratio [OR] 6.4) and atrial flutter/fibrillation (OR 3.8). Patients with heart block had lower 12-month survival, though only in patients with complete heart block as opposed to second degree block. At 12 months of age, 43% (12/28) of patients with heart block died and were more likely to experience mortality at 12 months than patients without block (OR 4.9; 95% confidence interval 1.4-17.5; P = .01). CONCLUSION: Although rare, complete heart block after stage I palliation represents an additional risk of poor outcomes in this high-risk patient population. Heterotaxy syndrome was the most significant risk factor for the development of heart block after stage I palliation. The role of transient block in outcomes and potential rescue with long-term pacing remains unknown and requires additional study.

Left Atrial Strain in the Repaired Tetralogy of Fallot Population: Comparisons to Biventricular Function, Native T1 Values, Exercise Parameters and Healthy Controls.

BACKGROUND: Non-invasive imaging markers in patients with repaired tetralogy of Fallot (rTOF) are still being investigated to inform clinical decision making. Atrial function is a prognostic indicator in many acquired and congenital heart diseases. We sought to examine the relationship between cardiac MRI (CMR)-derived indices of left atrial (LA) function, native left ventricular (LV) T1 values, biventricular systolic function, and exercise capacity in rTOF. METHODS: Sixty-six patients with rTOF without prior pulmonary valve replacement who underwent CMR (median age 18.5 years) were identified. Twenty-one adult rTOF patients (age range 19-32 years) were compared with 20 age-matched healthy volunteers (age range 19-34 years). LA reservoir, conduit, and pump global longitudinal strain (GLS) and strain rate (SR) were determined by tissue tracking. Native LV T1 values were measured on rTOF patients. Pearson correlations were performed to determine bivariate associations. RESULTS: Adult rTOF patients had higher pump GLS, pump:conduit, and pump:reservoir GLS ratios, and lower conduit:reservoir GLS ratio, LV ejection fraction (EF), and right ventricular EF compared to controls (p < 0.001 for each comparison). LA conduit:reservoir GLS and pump:reservoir GLS had correlations to native LV T1 (ρ = 0.26, p = 0.03 and ρ = - 0.26, p = 0.03, respectively). LA reservoir SR had positive correlation to RV EF (ρ = 0.27, p = 0.03). There were no statistically significant correlations between LA function and exercise capacity. CONCLUSIONS: LA function is altered in adolescent and young adult patients with rTOF indicating worse diastolic function and relates to increasing native LV T1 values. Future studies are indicated to investigate the progression of adverse atrial-ventricular interactions and poor outcomes in this population.

Echocardiographic myocardial strain analysis describes subclinical cardiac dysfunction after craniospinal irradiation in pediatric and young adult patients with central nervous system tumors.

BACKGROUND: Craniospinal irradiation (CSI) is part of the treatment of central nervous system (CNS) tumors and is associated with cardiovascular disease in adults. Global myocardial strain analysis including longitudinal peak systolic strain (GLS), circumferential peak systolic strain (GCS), and radial peak systolic strain (GRS) can reveal subclinical cardiac dysfunction. METHODS: Retrospective, single-center study in patients managed with CSI vs. age-matched controls. Clinical data and echocardiography, including myocardial strain analysis, were collected at early (< 12 months) and late (≥ 12 months) time points after completion of CSI. RESULTS: Echocardiograms were available at 20 early and 34 late time points. Patients at the late time point were older (21.7 ± 10.4 vs. 13.3 ± 9.6 years) and further out from CSI (13.1 ± 8.8 vs. 0.2 ± 0.3 years). Standard echocardiographic parameters were normal for both groups. For early, CSI vs. control: GLS was - 16.8 ± 3.6% vs. -21.3 ± 4.0% (p = 0.0002), GCS was - 22.5 ± 5.2% vs. -21.3 ± 3.4% (p = 0.28), and GRS was 21.8 ± 11.0% vs. 26.9 ± 7.7% (p = 0.07). For late, CSI vs. control: GLS was - 16.2 ± 5.4% vs. -21.6 ± 3.7% (p < 0.0001), GCS was - 20.9 ± 6.8% vs. -21.9 ± 3.5% (p = 0.42), and GRS was 22.5 ± 10.0% vs. 27.3 ± 8.3% (p = 0.03). Radiation type (proton vs. photon), and radiation dose (< 30 Gy vs. ≥ 30 Gy) did not impact any parameter, although numbers were small. CONCLUSIONS: Subclinical cardiac systolic dysfunction by GLS is present both early and late after CSI. These results argue for future studies to determine baseline cardiovascular status and the need for early initiation of longitudinal follow-up post CSI.

Variations in native T1 values in patients with Duchenne muscular dystrophy with and without late gadolinium enhancement.

Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder leading to progressive skeletal and cardiac myopathy. Elevated myocardial T1 values correlate with fibrosis in most disease processes, but DMD skeletal and cardiac histopathology is defined by fibrofatty replacement that may result in a decrease in T1 values, due to the low T1 of fat. The study goal was to assess myocardial T1 values in DMD patients with and without late gadolinium enhancement (LGE). A retrospective analysis was performed on all patients with DMD referred for CMR at our institution from 7/5/2017 to 10/24/2018. T1 measurements were performed using breath-held modified Look Locker inversion recovery (MOLLI) sequences at the basal and mid-ventricular levels. The cohort was separated into patients without the presence of LGE (LGE-) and patients with current or previous LGE (LGE+). A total of 207 CMR studies were analyzed. The LGE- group comprised 88 patients while 119 patients were in the LGE+ group. The LGE+ group was older, had larger indexed LV end-diastolic volume and lower LV ejection fraction (LVEF) compared to the LGE- group. T1 values in the LGE+ group were lower compared to the LGE- group (mid T1 1012 ms vs. 1035 ms; p = 0.002), with 5 CMR studies demonstrating mid T1 values < 900 ms. There was no correlation between mid T1 and LVEF in the LGE- group. In the LGE+ cohort, lower T1 values correlated with worse LVEF (r = 0.34, p = 0.0002). The association between mid T1 values and LVEF remained statistically significant on multivariable analysis when accounting for number of LGE segments, LVEDVi, and age (p = 0.009). This is the largest study assessing native T1 values in patients with DMD. The results demonstrate that patients with LGE had lower T1 values than patients without LGE. In the LGE+ group, lower T1 values correlated with worse LV systolic function. These results are consistent with the evolving recognition of fibrofatty replacement in advanced stages of DMD myopathy. Furthermore, our study supports that there is not a simple linear relationship between increasing T1 values and advancing disease progression reported in most other cardiomyopathies.

Progression of Vasculopathy in Young Individuals with Turner Syndrome.

Vasculopathy has been identified in young individuals with Turner syndrome (TS). No studies in young individuals with TS have investigated whether this vasculopathy progresses over time. The objective of this study is to describe the changes in vasculopathy over time in a cohort of young individuals with TS. Repeat ultrasound and SphygmoCor CPV® (AtCor Medical) measurements of carotid thickness and peripheral arterial stiffness were performed. Vascular measurements were compared at baseline and follow-up. Follow-up measurements were also compared to historical lean (L) and obese (O) age-, race-, and sex-matched non-TS controls. Thirty-five individuals with TS were studied at a mean age of 19.4 years (range, 13.9-27.5). Mean time to follow-up was 7.2 years (range, 7.1-7.8). Carotid intima media thickness increased by 0.03 ± 0.07 mm (p < 0.01) over time, but was less than L and O controls at follow-up. Pulse wave velocity carotid-femoral increased by 0.51 ± 0.86 m/s (p < 0.01) over time, but was similar to L and less than O controls at follow-up. Augmentation index (AIx) remained unchanged (p = 0.09) over time, but was significantly higher at follow-up than both control groups (p < 0.01 for both). There were no identified differences between 45,X and other TS genotypes. We demonstrate evidence of vascular thickening and stiffening over 7 years in a cohort of young individuals with TS, as well as a persistently increased augmentation index compared to L and O non-TS controls. It is unclear whether the increase in vascular structure and function are related to normal aging or if TS is a risk factor. Higher body mass index seems to be a risk factor. Early estrogen replacement and longer exposure to growth hormone therapy need to be further explored as potential protective factors.