Guidelines for Performing a Comprehensive Pediatric Transthoracic Echocardiogram: Recommendations From the American Society of Echocardiography.

Echocardiography is a fundamental component of pediatric cardiology, and appropriate indications have been established for its use in the setting of suspected, congenital, or acquired heart disease in children. Since the publication of guidelines for pediatric transthoracic echocardiography in 2006 and 2010, advances in knowledge and technology have expanded the scope of practice beyond the use of traditional modalities such as two-dimensional, M-mode, and Doppler echocardiography to evaluate the cardiac segmental structures and their function. Adjunct modalities such as contrast, three-dimensional, and speckle-tracking echocardiography are now used routinely at many pediatric centers. Guidelines and recommendations for the use of traditional and newer adjunct modalities in children are described in detail in this document. In addition, suggested protocols related to standard operations, infection control, sedation, and quality assurance and improvement are included to provide an organizational structure for centers performing pediatric transthoracic echocardiograms.

Relationship Between Obesity and Global Longitudinal Strain in the Pediatric Single Ventricle Fontan Population Across Ventricular Morphologies.

BACKGROUND: Obesity is associated with diminished myocardial function as measured by strain echocardiography in children and young adults with normal cardiac anatomy. Data are lacking about the effect of obesity on myocardial strain in patients with a single ventricle. In this study, the relationship between body mass index (BMI) and single ventricle myocardial strain in the Fontan population was assessed. METHODS AND RESULTS: Thirty-eight abnormal BMI Fontan cases (21 overweight and 17 obese) and 30 normal BMI Fontan controls matched based on single ventricular morphology, age, and sex were included in the study. Ventricular morphology was categorized as single right ventricle, single left ventricle, or biventricular. Single ventricle global longitudinal peak systolic strain (GLS) and other echocardiographic measurements were performed and compared between groups, with a P≤0.05 defined as significant. The abnormal BMI group demonstrated diminished GLS (-15.7±3.6% versus -17.2±3.2%, [P=0.03]) and elevated systolic blood pressure (P=0.04) compared with the normal BMI group. On subgroup analysis of those with single right ventricle morphology, the abnormal BMI group demonstrated diminished GLS compared with controls. There was no significant difference in GLS between the abnormal BMI and control groups in the single left ventricle and biventricular subgroups. Analyzed by ventricular morphology, no other variables were statistically different in the abnormal BMI group including systolic blood pressure. Inter-reader reproducibility for GLS and strain rate were excellent for both measures. CONCLUSIONS: Obesity has an adverse relationship with myocardial strain in the young Fontan population, with the most maladaptive response seen in the single right ventricle.

Echocardiographic findings in non-hospitalised children and adolescents following acute COVID-19.

BACKGROUND: Although COVID-19 is known to have cardiac effects in children, seen primarily in severe disease, more information is needed about the cardiac effects following COVID-19 in non-hospitalised children and adolescents during recovery. This study aims to compare echocardiographic markers of cardiac size and function of children following acute COVID-19 with those of healthy controls. METHODS: This single-centre retrospective case-control study compared 71 cases seen in cardiology clinic following acute COVID-19 with 33 healthy controls. Apical left ventricle, apical right ventricle, and parasternal short axis at the level of the papillary muscles were analysed to measure ventricular size and systolic function. Strain was analysed on vendor-independent software. Statistical analysis was performed using t-test, chi-square, Wilcoxon rank sum, and regression modelling as appropriate (p < 0.05 significant). RESULTS: Compared to controls, COVID-19 cases had slightly higher left ventricular volumes and lower left ventricular ejection fraction and right ventricular fractional area change that remained within normal range. There were no differences in right or left ventricular longitudinal strain between the two groups. Neither initial severity nor persistence of symptoms after diagnosis predicted these differences. CONCLUSIONS: Echocardiographic findings in children and adolescents 6 weeks to 3 months following acute COVID-19 not requiring hospitalisation were overall reassuring. Compared to healthy controls, the COVID-19 group demonstrated mildly larger left ventricular size and lower conventional measures of biventricular systolic function that remained within the normal range, with no differences in biventricular longitudinal strain. Future studies focusing on longitudinal echocardiographic assessment of patients following acute COVID-19 are needed to better understand these subtle differences in ventricular size and function.

The association between cardiovascular health and cognition in adults with Down syndrome.

INTRODUCTION: Evidence in the general population suggests that predictors of cardiovascular health such as moderate to vigorous physical activity (MVPA), cardiorespiratory fitness, and systolic blood pressure are associated with cognitive function. Studies supporting these associations in adults with Down syndrome (DS) are limited. The purpose of this study was to examine the associations between systolic blood pressure, cardiorespiratory fitness, and MVPA on cognition in adults with DS. METHODS: This is a cross-sectional analysis using baseline data from a trial in adults with DS. Participants attended a laboratory visit where resting blood pressure, cardiorespiratory fitness (VO2 Peak), and cognitive function (CANTAB® DS Battery) were obtained. The cognitive battery included tests measuring multitasking, episodic memory, and reaction time. Physical activity (accelerometer) was collected over the week following the laboratory visit. Pearson correlations and linear regressions were used to measure the impact of systolic blood pressure, cardiorespiratory fitness, and MVPA on cognitive outcomes. RESULTS: Complete data was available for 72 adults with DS (26.8 ± 9.3 years of age, 57% female). At baseline, VO2 Peak (21.1 ± 4.2 ml/kg/min) and MVPA were low (14.4 ± 14.4 min/day), and systolic blood pressure was 118.3 ± 13.3 mmHg. VO2 Peak was correlated with simple movement time (rho = - 0.28, p = 0.03) but was not significant using a linear regression controlling for age and sex. Systolic blood pressure was significantly associated with episodic memory (first attempt memory score: ß = - 0.11, p = 0.002; total errors: ß = 0.58, p = 0.001) and reaction time (five-choice movement time: ß = 4.11, p = 0.03; simple movement time: ß = 6.14, p = 0.005) using age- and sex-adjusted linear regressions. No associations were observed between MVPA and multitasking, episodic memory, or reaction time. CONCLUSION: Predictors of cardiovascular health, including cardiorespiratory fitness and systolic blood pressure, were associated with some aspects of cognition in adults with DS. While future research should examine the role of improved cardiovascular health on delaying decreases in cognitive function and dementia in adults with DS, we recommend that health care providers convey the importance of exercise and cardiovascular health to their patients with DS. TRIAL REGISTRATION: NCT04048759, registered on August 7, 2019.

Peak Oxygen Consumption (V̇O 2peak ) Recovery Delay in a Pediatric Fontan Population.

PURPOSE: The purpose of this study is to identify predictors and correlates of VO2RD in youth with Fontan. METHODS: Cardiopulmonary exercise test data was used from a single center, cross-sectional study of children and adolescents (age, 8-21 yr) with Fontan physiology. The VO2RD was determined using time (s) to <90% of V̇O 2peak and categorized as "low" (≤10 s) or "high" (≥10 s). t Tests and χ 2 analysis were used to compare continuous and categorical variables, respectively. RESULTS: The analysis sample included 30 adolescents with Fontan physiology (age, 14.2 ± 2.4 yr; 67% male) with either right ventricular (RV) dominant (40%) or co/left ventricular (Co/LV) dominant (60%) systemic ventricular morphology. There were no differences in V̇O 2peak between the high and low VO2RD groups (high = 1.3 ± 0.4 L·min -1 ; low = 1.3 ± 0.3 L·min -1 ; P = 0.97). VO2RD in participants with RV dominance was significantly greater than in patients with Co/LV dominance (RV = 23.8 ± 15.8 s; Co/LV = 11.8 ± 16.1 s; P = 0.03). CONCLUSIONS: V̇O 2peak was not correlated with VO2RD when analyzed as high/low VO2RD groups. However, morphology of the systemic single ventricle (RV vs Co/LV) may be related to rate of recovery in V̇O 2 after a peak cardiopulmonary exercise test.

The Evolution of Longitudinal Strain Across Various Univentricular Morphologies Prior to Superior Cavopulmonary Anastomosis.

BACKGROUND: Infants with single-ventricle physiology have increased morbidity, mortality, and ventricular dysfunction in the pre-superior cavopulmonary anastomosis (pre-SCPA) period. Echocardiography-derived longitudinal strain (LS) is emerging as a reliable marker of single-ventricle performance. We aim to assess evolution of LS during the pre-SCPA period across univentricular morphologies and explore associations of LS with modifiable and nonmodifiable factors. METHODS: Ninety-four term infants (36 females) with univentricular physiology who were discharged home prior to stage 2 palliation were serially analyzed for LS (single apical view) and other echo measures at initial hospital discharge and the last pre-SCPA encounter. Ventricular myocardium was tracked for strain along the septum and respective lateral wall for single right ventricular (RV) and left ventricular (LV) groups, and along both right and left lateral walls in functionally univentricular hearts with biventricular (BiV) morphology. Clinical data were obtained from the medical record. RESULTS: Longitudinal strain improved in the total cohort during the pre-SCPA period (16.48% ± 3.31% to 17.57% ± 3.81%, P = .003). Longitudinal strain improved between encounters in the single LV (P = .04) and BiV groups (P = .02). However, LS failed to improve in the RV group (P = .7) with lower LS at both visits compared with the other groups. The RV group, mostly composed of hypoplastic left heart syndrome patients-87% of cases-had a higher incidence of arrhythmias (57%) and unplanned reinterventions (60%), most of which were arch reinterventions. A subanalysis based on arch reintervention showed that LS improves between encounters in the single LV group needing arch reintervention (P = .05) compared with the single RV group needing arch reintervention (P = .89). Lower LS was independently associated with unplanned reinterventions at both encounters (P = .008 and .02). CONCLUSIONS: Single-ventricle LS evolves differently across ventricular morphologies during the pre-SCPA period and is related to the need for unplanned cardiac reinterventions. Lower LS is noted in the single RV group, who mostly have hypoplastic left heart syndrome.

Moderators of peak respiratory exchange ratio during exercise testing in children and adolescents with Fontan physiology.

OBJECTIVES: Many patients with Fontan physiology are unable to achieve the minimum criteria for peak effort during cardiopulmonary exercise testing. The purpose of this study is to determine the influence of physical activity and other clinical predictors related to achieving peak exercise criteria, signified by respiratory exchange ratio ≥ 1.1 in youth with Fontan physiology. METHODS: Secondary analysis of a cross-sectional study of 8-18-year-olds with single ventricle post-Fontan palliation who underwent cardiopulmonary exercise testing (James cycle protocol) and completed a past-year physical activity survey. Bivariate associations were assessed by Wilcoxon rank-sum test and simple regression. Conditional inference forest algorithm was used to classify participants achieving respiratory exchange ratio > 1.1 and to predict peak respiratory exchange ratio. RESULTS: Of the n = 43 participants, 65% were male, mean age was 14.0 ± 2.4 years, and 67.4% (n = 29) achieved respiratory exchange ratio ≥ 1.1. Despite some cardiopulmonary exercise stress test variables achieving statistical significance in bivariate associations with participants achieving respiratory exchange ratio > 1.1, the classification accuracy had area under the precision recall curve of 0.55. All variables together explained 21.4% of the variance in respiratory exchange ratio, with peak oxygen pulse being the most informative. CONCLUSION: Demographic, physical activity, and cardiopulmonary exercise test measures could not classify meeting peak exercise criteria (respiratory exchange ratio ≥ 1.1) at a satisfactory accuracy. Correlations between respiratory exchange ratio and oxygen pulse suggest the augmentation of stroke volume with exercise may affect the Fontan patient's ability to sustain high-intensity exercise.

Examination of Adverse Reactions After COVID-19 Vaccination Among Patients With a History of Multisystem Inflammatory Syndrome in Children.

Importance: Data are limited regarding adverse reactions after COVID-19 vaccination in patients with a history of multisystem inflammatory syndrome in children (MIS-C). The lack of vaccine safety data in this unique population may cause hesitancy and concern for many families and health care professionals. Objective: To describe adverse reactions following COVID-19 vaccination in patients with a history of MIS-C. Design, Setting, and Participants: In this multicenter cross-sectional study including 22 North American centers participating in a National Heart, Lung, and Blood Institute, National Institutes of Health-sponsored study, Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children (MUSIC), patients with a prior diagnosis of MIS-C who were eligible for COVID-19 vaccination (age ≥5 years; ≥90 days after MIS-C diagnosis) were surveyed between December 13, 2021, and February 18, 2022, regarding COVID-19 vaccination status and adverse reactions. Exposures: COVID-19 vaccination after MIS-C diagnosis. Main Outcomes and Measures: The main outcome was adverse reactions following COVID-19 vaccination. Comparisons were made using the Wilcoxon rank sum test for continuous variables and the χ2 or Fisher exact test for categorical variables. Results: Of 385 vaccine-eligible patients who were surveyed, 185 (48.1%) received at least 1 vaccine dose; 136 of the vaccinated patients (73.5%) were male, and the median age was 12.2 years (IQR, 9.5-14.7 years). Among vaccinated patients, 1 (0.5%) identified as American Indian/Alaska Native, non-Hispanic; 9 (4.9%) as Asian, non-Hispanic; 45 (24.3%) as Black, non-Hispanic; 59 (31.9%) as Hispanic or Latino; 53 (28.6%) as White, non-Hispanic; 2 (1.1%) as multiracial, non-Hispanic; and 2 (1.1%) as other, non-Hispanic; 14 (7.6%) had unknown or undeclared race and ethnicity. The median time from MIS-C diagnosis to first vaccine dose was 9.0 months (IQR, 5.1-11.9 months); 31 patients (16.8%) received 1 dose, 142 (76.8%) received 2 doses, and 12 (6.5%) received 3 doses. Almost all patients received the BNT162b2 vaccine (347 of 351 vaccine doses [98.9%]). Minor adverse reactions were observed in 90 patients (48.6%) and were most often arm soreness (62 patients [33.5%]) and/or fatigue (32 [17.3%]). In 32 patients (17.3%), adverse reactions were treated with medications, most commonly acetaminophen (21 patients [11.4%]) or ibuprofen (11 [5.9%]). Four patients (2.2%) sought medical evaluation, but none required testing or hospitalization. There were no patients with any serious adverse events, including myocarditis or recurrence of MIS-C. Conclusions and Relevance: In this cross-sectional study of patients with a history of MIS-C, no serious adverse events were reported after COVID-19 vaccination. These findings suggest that the safety profile of COVID-19 vaccination administered at least 90 days following MIS-C diagnosis appears to be similar to that in the general population.

Single ventricular strain measures correlate with peak oxygen consumption in children and adolescents with Fontan circulation.

INTRODUCTION: Children with a single ventricle post-Fontan palliation are at increased risk of poor outcomes with peak oxygen consumption acting as a surrogate outcome marker. The purpose of this study is to evaluate the relationship between peak oxygen consumption and echocardiographic measures of ventricular function and deformation, including ventricular global longitudinal strain and dyssynchrony, in children and adolescents following Fontan palliation. METHODS: Patients (age 8-21 years) with single ventricle post-Fontan palliation were prospectively recruited and participated in an echocardiogram, including views optimised for two-dimensional speckle tracking, and a cardiopulmonary exercise test on a cycle ergometer to maximal volitional fatigue. RESULTS: Thirty-eight patients (mean age 13.7 ± 2.3 years) post-Fontan palliation had either a single left ventricular (n = 20), single right ventricular (n = 14), or biventricular (n = 4) morphology. Peak oxygen consumption (24.9 ± 5.6 ml/kg/minute) was correlated with global longitudinal strain (r = -0.435, p = 0.007), a strain discoordination time to peak index (r = -0.48, p = 0.003), and the presence of an electro-mechanical dyssynchrony strain pattern (p = 0.008). On multivariate regression modelling, these three variables were associated with peak oxygen consumption independently of age and sex. The single right ventricular group had evidence of possible diastolic dysfunction by E/e' compared to the single left ventricular and biventricular groups (p = 0.001). CONCLUSIONS: Strain analysis measures are correlated with peak oxygen consumption in this cohort of children, adolescents, and young adults following Fontan palliation, suggesting that ventricular mechanics may influence the efficiency of the Fontan circulation.

Cardiac Structure and Function in Adults with Down Syndrome.

Various factors may alter the risk for cardiovascular disease in adults with Down syndrome (Ds), yet few studies have examined differences in cardiac physiology in this population. Previous research suggested lower systolic and diastolic function, but inconsistent methodologies and younger samples warrant research in adults with Ds. Our aim is to compare the cardiac structure and function of adults with Ds to age- and sex-matched adults without Ds. Echocardiography was used to assess systolic function, diastolic function, and cardiac structure in n = 19 adults (Ds n = 9, control n = 10). Regarding cardiac structure, adults with Ds had increased left ventricular posterior wall thickness at end-systole compared to adults without Ds (p = 0.007). Regarding systolic and diastolic function, adults with Ds were found to have lower septal peak systolic annular velocity (S') (p = 0.026), lower lateral and septal mitral annular early diastolic velocity (E') (p = 0.007 and p = 0.025, respectively), lower lateral peak mitral annular late diastolic velocity (A') (p = 0.027), and higher lateral and septal mitral annular early systolic velocity to diastolic velocity ratios (E/e') (p = 0.001 and p = 0.001, respectively). Differences in both cardiac structure and function were found when comparing adults with Ds to matched adults without Ds. Most of the differences were indicative of worse diastolic function.

Electrocardiographic changes in non-hospitalised children with COVID-19.

OBJECTIVES: Many children diagnosed with COVID-19 infections did not require hospitalisation. Our objective was to analyse electrocardiographic changes in children with asymptomatic, mild or moderate COVID-19 who did not require hospitalisation. METHODS: All children are seen in a paediatric cardiology clinic who had asymptomatic, mild or moderate COVID-19 that did not require hospitalisation and had at least one electrocardiogram after their diagnosis were included in this retrospective analysis. Records were reviewed to determine COVID-19 disease severity and presence of Long COVID. Rhythm assessment, atrial enlargement, ventricular hypertrophy, PR/QRS/QT interval duration and ST-T wave abnormalities were analysed by a paediatric electrophysiologist. Clinically ordered echocardiograms were reviewed for signs of myopericarditis (left ventricular ejection fraction and pericardial effusion) on any subject with an electrocardiographic abnormality. RESULTS: Of the 82 children meeting inclusion criteria (14.4 years, range 1-18 years, 57% male), 17 patients (21%) demonstrated electrocardiographic changes. Ten patients (12%) had electrocardiogram of borderline significance, which included isolated mild PR prolongation or mild repolarisation abnormalities. The other seven patients (9%) had concerning electrocardiographic findings consisting of more significant repolarisation abnormalities. None of the patients with an abnormal electrocardiogram revealed any echocardiographic abnormality. All abnormal electrocardiograms normalised over time except in two cases. Across the entire cohort, greater COVID-19 disease severity and long COVID were not associated with electrocardiographic abnormalities. CONCLUSIONS: Electrocardiographic abnormalities are present in a minority of children with an asymptomatic, mild or moderate COVID-19 infection. Many of these changes resolved over time and no evidence of myopericarditis was present on echocardiography.

Clinically Suspected Myocarditis Temporally Related to COVID-19 Vaccination in Adolescents and Young Adults: Suspected Myocarditis After COVID-19 Vaccination.

BACKGROUND: Understanding the clinical course and short-term outcomes of suspected myocarditis after the coronavirus disease 2019 (COVID-19) vaccination has important public health implications in the decision to vaccinate youth. METHODS: We retrospectively collected data on patients <21 years old presenting before July 4, 2021, with suspected myocarditis within 30 days of COVID-19 vaccination. Lake Louise criteria were used for cardiac MRI findings. Myocarditis cases were classified as confirmed or probable on the basis of the Centers for Disease Control and Prevention definitions. RESULTS: We report on 139 adolescents and young adults with 140 episodes of suspected myocarditis (49 confirmed, 91 probable) at 26 centers. Most patients were male (n=126, 90.6%) and White (n=92, 66.2%); 29 (20.9%) were Hispanic; and the median age was 15.8 years (range, 12.1-20.3; interquartile range [IQR], 14.5-17.0). Suspected myocarditis occurred in 136 patients (97.8%) after the mRNA vaccine, with 131 (94.2%) after the Pfizer-BioNTech vaccine; 128 (91.4%) occurred after the second dose. Symptoms started at a median of 2 days (range, 0-22; IQR, 1-3) after vaccination. The most common symptom was chest pain (99.3%). Patients were treated with nonsteroidal anti-inflammatory drugs (81.3%), intravenous immunoglobulin (21.6%), glucocorticoids (21.6%), colchicine (7.9%), or no anti-inflammatory therapies (8.6%). Twenty-six patients (18.7%) were in the intensive care unit, 2 were treated with inotropic/vasoactive support, and none required extracorporeal membrane oxygenation or died. Median hospital stay was 2 days (range, 0-10; IQR, 2-3). All patients had elevated troponin I (n=111, 8.12 ng/mL; IQR, 3.50-15.90) or T (n=28, 0.61 ng/mL; IQR, 0.25-1.30); 69.8% had abnormal ECGs and arrhythmias (7 with nonsustained ventricular tachycardia); and 18.7% had left ventricular ejection fraction <55% on echocardiogram. Of 97 patients who underwent cardiac MRI at a median 5 days (range, 0-88; IQR, 3-17) from symptom onset, 75 (77.3%) had abnormal findings: 74 (76.3%) had late gadolinium enhancement, 54 (55.7%) had myocardial edema, and 49 (50.5%) met Lake Louise criteria. Among 26 patients with left ventricular ejection fraction <55% on echocardiogram, all with follow-up had normalized function (n=25). CONCLUSIONS: Most cases of suspected COVID-19 vaccine myocarditis occurring in persons <21 years have a mild clinical course with rapid resolution of symptoms. Abnormal findings on cardiac MRI were frequent. Future studies should evaluate risk factors, mechanisms, and long-term outcomes.

Intervendor Agreement for Right Ventricular Global Longitudinal Strain in Children.

BACKGROUND: Right ventricular global longitudinal strain (RVGLS) has emerged as an important technique for clinical evaluation of (RV) function. The routine application of RVGLS in pediatrics remains limited by a lack of data on agreement between vendors. The aim of this study was to investigate intervendor agreement for RVGLS between the two commonly used analysis vendors in pediatrics, hypothesizing that RVGLS has good intervendor agreement, although it is likely lower than intravendor agreement (inter- and intraobserver reproducibility). METHODS: Seventy infants and children with normal cardiac anatomy and varying ventricular function were included after prospectively obtaining RV-focused four-chamber apical images on the GE Vivid E95. Images were analyzed for RVGLS at acquired frame rates in EchoPAC (GE) and TomTec (TT) and in the compressed Digital Imaging and Communications in Medicine format in TT. Intraclass correlation coefficients and Bland-Altman plots were used to test intervendor agreement and intravendor reproducibility. RESULTS: RVGLS measurements were equally feasible using TT and EchoPAC analysis (92%). There was good to excellent agreement for RVGLS between TT and EchoPAC analysis, with a relatively higher intraclass correlation coefficient between GE and TT at the acquired frame rate (0.85) than between GE and TT at the compressed frame rate (0.75) and significantly higher agreement in patients with abnormal RV function (0.7-0.9) than those with normal function (0.4-0.6). Intra- andinterobserver reproducibility for RVGLS was excellent (intraclass correlation coefficient = 0.74-0.96). Heart rate ≥ 100 beats/min and acquisition frame rate/heart rate ≤ 0.7 were associated with diminished agreement, especially when compressed data were involved. CONCLUSIONS: RVGLS analyzed using EchoPAC and TT show good agreement, especially when analyzed at acquisition frame rates and in the setting of abnormal RV function. Otherwise, RVGLS should ideally be analyzed using the same vendor, and intervendor comparisons should be undertaken with caution, particularly if data are in a compressed format.

The promotion of physical activity for the prevention of Alzheimer's disease in adults with Down Syndrome: Rationale and design for a 12 Month randomized trial.

Nearly all individuals with Down Syndrome (DS) display pathology associated with Alzheimer's disease (AD) beginning as early as age 30. Previous research in typically developed adults suggests that increased moderate-to-vigorous physical activity (MVPA) may improve cognitive function and protect against age-related structural and functional changes in the brain; however, the potential impact of increased MVPA on the development of AD in adults with DS has not been evaluated. Despite the potential positive impact of MVPA on cognition and AD risk, participation in MVPA among young adults with DS is low. The limited research evaluating strategies for increasing MVPA in adults with DS has been unsuccessful in increasing MVPA. Results from our preliminary investigation where we remotely delivered real-time MVPA, led by a trained health educator, to groups of adults with DS in their homes via video conferencing on a tablet computer demonstrated high attendance, increased MVPA during group sessions, and improvements in cognitive function. However, the sustainability, impact on total daily MVPA, optimal session frequency, and potential impacts on cognitive function and brain health of remotely delivered group MVPA sessions in adults with DS are unknown. Therefore, we will conduct a trial in 80 non-demented adults with DS to determine the feasibility and potential efficacy of remotely delivered group MVPA sessions to increase daily MVPA, relative to a usual care control. Secondarily we will assess the impact of MVPA on cardiovascular fitness, quality of life, cognitive function and brain parameters related to AD. NCT REGISTRATION: NCT04048759.

Association of left ventricular size with regional right ventricular mechanics in Hypoplastic Left Heart Syndrome.

BACKGROUND: In Hypoplastic Left Heart Syndrome (HLHS), RV dysfunction is associated with poor outcomes. However, the effect of varying LV size on regional RV mechanics and outcome has not been studied. METHODS: Twenty newborns (0-7 days) with HLHS had pre-stage 1 and pre-stage 2 echocardiograms prospectively protocoled for strain analysis of the apical 4-chamber view. RV longitudinal strain was analyzed, and LV size was classified as diminutive (no visible LV chamber) or moderate size (visible LV chamber). Clinical outcome was reported as alive vs death or transplant (D-TP) at final clinical follow-up (pre-stage 3). Groups were compared with t-test, Fisher's Exact, and ANOVA tests as appropriate. RESULTS: At pre-stage 1, infants with a diminutive LV (7/20, 35%) vs a moderately hypoplastic LV (13/20, 65%) did not have significantly different global RV strain (-18.4 ±â€¯2.6% vs -18.8 ±â€¯3.2%; p = 0.83). However, basal septal strain was significantly diminished in the moderately hypoplastic LV group vs the diminutive LV group (-4.4 ±â€¯6.0% vs -14.7 ±â€¯3.3%; p < 0.005). There was severely diminished septal strain in nearly all (11/13) of the moderately hypoplastic group. At the pre-stage II echo, global RV strain between groups remained similar (p = 0.76) as did the diminished septal strain in the moderate LV group (p = 0.86). The moderately hypoplastic LV group had worse clinical outcomes (6/13 D-TP vs 0/7 D-TP; p = 0.05). CONCLUSIONS: In this small HLHS cohort, diminished septal strain leading to asymmetric RV mechanics may be associated with poor outcomes in those with larger LV/septal size.

Reproducibility and Intervendor Agreement of Left Ventricular Global Systolic Strain in Children Using a Layer-Specific Analysis.

BACKGROUND: Speckle-tracking strain analysis provides additive data to the assessment of pediatric and congenital heart disease; however, the variety of strain analysis software platforms by different vendors and the lack of data on intervendor strain agreement in children have limited its utility. The purpose of this study is to evaluate the intervendor agreement of strain on two commonly used analysis platforms in pediatrics by layer of myocardium and data compression. METHODS: This prospective study analyzed two-dimensional speckle-tracking strain on two software platforms in 53 children with normal cardiac segmental anatomy and varying function. Three standard apical views and one parasternal short-axis view were exported at their acquired frame rates to workstations with GE EchoPAC and TomTec software and then also to TomTec at compressed frame rates. Both software platforms had been updated with European Association of Cardiovascular Imaging/American Society of Echocardiography Task Force recommendations for left ventricular (LV) global strain. Intravendor and intervendor agreement between layer-specific comparisons were assessed using Bland-Altman analysis (limits of agreement and bias) and intraclass correlation coefficients. RESULTS: This study included subjects with normal LV function (n = 38) and cardiomyopathy (n = 15) with an age range of 1 month to 18 years. Intertechnique agreement by default vendor myocardial layer (GE mid-TomTec endocardial layer) was robust for both global longitudinal (GLS) and circumferential strain (GCS; higher for GLS than GCS). Intravendor (inter- and interreader) agreement was slightly higher than intervendor. Only small differences in intraclass correlation coefficients were present between various myocardial layers and acquired versus compressed TomTec data with narrow limits of agreement and small bias except in certain subgroup comparisons. CONCLUSIONS: Comparison of LV GLS and GCS between two commonly used software platforms after European Association of Cardiovascular Imaging/American Society of Echocardiography Industry Task Force recommendations demonstrated good to excellent agreement in pediatrics, regardless of the layer of analysis or the image format, although some degree of variability remains between vendor platforms. Overall, GLS agreement was more robust than GCS, and this difference is exaggerated in specific subanalyses. These data suggest that comparisons of strain values obtained on these two vendors will be reasonable, but caution should be used when the indication is the detection of small differences between serial echocardiograms.

Anomalous aortic origin of the right coronary artery from the non-coronary sinus of Valsalva.

We report a rare case of anomalous aortic origin of the right coronary artery from the posterior/non-coronary sinus of Valsalva in a 9-year-old male diagnosed during the workup of premature ventricular contractions. The finding was initially noted on transthoracic echocardiogram and further confirmed with computed tomography. The anomalous coronary artery shows a wide ostium with no intramural or interarterial course.

Remote Exercise for Adults with Down Syndrome.

PURPOSE: Adults with Down syndrome are less physically active than their typically developed peers. The purpose of this study was to assess the feasibility of delivering moderate-to- vigorous exercise sessions, led by a trained health educator using real-time video conferencing, to groups of young adults with Down syndrome in their homes. METHODS: Participants were randomized to 30-minute group exercise sessions either 1 or 2 times a week delivered on an iPad mini tablet computer using the Zoom video conferencing application, and were asked to attend individual support/education sessions once a week using FaceTime® on the iPad, for 12 weeks. Minutes of MVPA during all group sessions were assessed using a Fitbit Charge HR activity/heart rate monitor. Participants were also asked to complete weekly homework assignments involving MVPA. RESULTS: Twenty-seven participants (n = 14,1 session·wk-1, n =13, 2 sessions·wk-1), mean age 27.9 ± 7.1 yrs., ~ 41% female, enrolled in and completed the 12-wk. intervention. Attendance at group exercise and individual support/education sessions did not differ significantly between those randomized to 1 (exercise sessions =89.9 ± 8.8%, support/education sessions = 81.2 ±18.7%) or 2 sessions·wk-1 (exercise sessions = 88.8 ± 7.7%; p=0.79, support/education sessions= 86.0 ± 20.9%; p=0.87). Participants averaged 27.7 ± 5.7 mins·session-1 of MVPA with no significant difference between the 1 (26.6 ± 3.0 mins·session-1) and 2 session·wk-1 groups (28.8 ± 7.7 mins·session-1, p=0.16). The completion rate for homework assignments did not differ significantly between the 1 (21.4 ± 26.3%) and 2 session·wk-1 groups (37.7 ± 21.7%, p=0.28). CONCLUSION: Exercise delivered by group video conferencing may be a feasible and potentially effective approach for increasing MVPA in adults with Down syndrome.