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Three-dimensional echocardiography (3DE) evaluation of right ventricular (RV) volumes and ejection fraction (EF) is increasingly used for clinical serial assessments and management in children. This study aims to generate sex-specific reference values and z-score equations for RV volumetric parameters, independent of age and body size indices, derived from multiple populations across North America.We prospectively recruited 455 healthy children (ages 0 to 18 years) from 5 centres. 3DE of the RV were acquired using various vendors with analyses performed offline using vendor-independent software. 3DE datasets with all walls of the RV endocardium visible were included. We reported data on RV EF, and generated z scores for end-systolic volumes (ESV), end-diastolic volumes (EDV) and stroke volume (SV). Differences between the sexes were explored.Of 455 3DE datasets, 312 (68%) met imaging criteria for analysis. Median age was 10.1 years (interquartile ratio [IQR]: 5.6, 14.0) with 17% being younger than 3 years of age. The mean and standard deviation for RV EDV, ESV, and SV for male and female patients were reported. We provided a downloadable z-score calculator with height and weight as independent variables to facilitate clinical utility. Although statistically significant differences between male and female RVEF was present (female 52.9 ± 3.9% vs male 51.6 ± 3.5%, P = 0.006), after adjusting for age, height, and weight, the magnitude of difference was clinically insignificant.Sex-specific reference values for pediatric RV volumes and EF, and z-score equations were derived from children 3DE datasets across 5 centres in North America.

Background: Cardiac manifestations in multisystem inflammatory syndrome in children (MIS-C) occur in ∼80% of patients. Left ventricular (LV) systolic dysfunction is the most frequent cardiac finding. Methods: In this single-centre, retrospective cohort study, we report on detailed assessment of LV function in MIS-C patients using strain and strain rate analysis. We compare those with normal peak systolic strain z-scores (both longitudinal and circumferential strain) to those with abnormal peak systolic strain z-scores (decreased circumferential and/or longitudinal strain). Results: Among 25 patients, 14 (56%) were male, 20 (80%) were Black or Hispanic, 13 (52%) were overweight/obese, and the median age was 11.4 years (interquartile range: 7.5 to 16). Median ejection fraction (EF) was 55.2% (interquartile range: 48.3% to 58%), with the abnormal strain patients having a lower EF (P < 0.01). Demographics were similar between groups. The abnormal strain patients had more organ systems involved and were more likely to require inotropic support. In a comparison of MIS-C patients with normal EF (n = 15) to controls, MIS-C patients had lower peak systolic strain as well as lower early diastolic strain rates. In patients with initially depressed function, EF normalized in 8 of 10 (80%), but 4 of 11 (36%) patients had persistently abnormal systolic strain after discharge. Conclusions: LV systolic dysfunction is common in the acute phase of MIS-C, and detection may be improved with strain imaging. Longitudinal cardiac follow-up is imperative, as some patients may be at risk for persistent LV dysfunction. Résumé: Contexte: Des manifestations cardiaques sont observées chez environ 80 % des patients atteints du syndrome inflammatoire multisystémique de l'enfant (SIM-E). La dysfonction systolique ventriculaire gauche est le problème cardiaque observé le plus fréquemment. Méthodologie: Dans cette étude de cohorte rétrospective et unicentrique, nous rapportons les résultats d'une évaluation détaillée de la fonction ventriculaire gauche chez des patients atteints du SIM-E sous l'angle de l'étude des contraintes et des taux de contrainte. Nous comparons les patients dont les écarts z des pics de contrainte systolique sont normaux (contraintes tant longitudinales que circonférentielles) et ceux dont les écarts z des pics de contrainte systolique sont anormaux (réduction de la contrainte circonférentielle ou longitudinale). Résultats: Sur 25 patients, 14 (56 %) étaient de sexe masculin, 20 (80 %) étaient noirs ou hispaniques, 13 (52 %) étaient en surpoids ou obèses, et l'âge médian était de 11,4 ans (intervalle interquartile : de 7,5 à 16). La fraction d'éjection (FE) médiane était de 55,2 % (intervalle interquartile : de 48,3 % à 58 %), et était moins élevée chez les patients présentant une contrainte anormale (p < 0,01). Les caractéristiques démographiques étaient comparables dans tous les groupes. Les patients chez lesquels la contrainte était anormale présentaient un plus grand nombre d'organes atteints et étaient plus susceptibles de nécessiter un soutien inotrope. Comparativement au groupe témoin, les patients SIM-E ayant une FE normale (n = 15) présentaient un pic de contrainte systolique moins élevé et des taux de contrainte diastolique précoce plus faibles. Chez les patients dont la fonction était déprimée à l'origine, la FE s'est normalisée chez huit patients sur 10 (80 %), mais quatre sur 11 (36 %) présentaient une contrainte systolique persistant après leur sortie de l'hôpital. Conclusions: La dysfonction systolique ventriculaire gauche est fréquente dans la phase aiguë du SIM-E, et son repérage pourrait être amélioré par l'imagerie permettant de visualiser les contraintes. Un suivi cardiaque longitudinal est impératif, car certains patients peuvent être à risque de souffrir d'une dysfonction ventriculaire gauche persistante.

Strain values vary with age in children and are both vendor- and platform-specific. Philips QLab 10.8 and Tomtec AutoStrain are two widely-utilized strain analysis platforms, and both incorporate recent EACVI-ASE-Industry Strain Standardization Task Force guidelines. We sought to establish normal strain values and Z-scores for both platforms using a large dataset of healthy children and to compare values among these two platforms and a previous version-QLab 10.5-which predated the Task Force guidelines.Echocardiograms from 1,032 subjects21 years old with structurally and functionally normal hearts were included. Images were obtained on the Philips EPIQ platform. Left ventricular (LV) and right ventricular (RV) strain were analyzed using QLab 10.8 and AutoStrain and measurement reliability was assessed. Z-score equations were derived as a function of age for QLab 10.8 (LV longitudinal and circumferential strain) and AutoStrain (LV and RV longitudinal strain). A subset (n=309) was analyzed by QLab 10.5. Strain values were compared among the three platforms.For both of the newer platforms, strain varied with age, with magnitude reaching a maximum at 4-5 years. For LV longitudinal strain, the largest differences in value were observed in the youngest patients when using QLab 10.5; the other two platforms were similar. LV circumferential strain measurements (QLab 10.5 vs 10.8) were different for all ages, as were measurements of RV longitudinal strain (QLab 10.8 vs AutoStrain). Reliability was greater for AutoStrain than for QLab 10.8, and greater for LV than for RV strain.We generated normal RV and LV strain values and Z-scores from a large cohort of children for two commonly-utilized platforms in pediatric echocardiography laboratories. Following incorporation of Task Force guidelines, the greatest improvement in standardization was seen in infants. Small differences persist between modern platforms; however, these results support the cautious consideration of comparing inter-platform measurements.

Background: Three-dimensional echocardiography (3DE) evaluation of left ventricular (LV) volume and function in pediatrics compares favorably with cardiac magnetic resonance imaging. The aim of this study was to establish from a multicenter, normal pediatric z-score values of 3DE left ventricular volumes and function. Methods: Six hundred and ninety-eight healthy children (ages 0 to 18 years) were recruited from five centers. LV 3DE was acquired from the 4-chamber view. A vendor independent software analyzed end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), and ejection fraction (EF) using semi-automated quantification. Body surface area (BSA) based z-scores were generated. Intraobserver and interobserver variability were calculated using intraclass correlation (ICC) and repeatability coefficient (RC). Results: Z-scores were generated for ESV, EDV, and SV. The ICC for intraobserver variability for EDV, ESV, and SV were 0.99, 0.99, and 0.99 respectively. The ICC for interobserver variability for EDV, ESV, and SV were 0.98, 0.94, and 0.98 respectively. The RC for intraobserver and interobserver variability for LV EF was 4.39% (95% CI: 3.01, 5.59) and interobserver was 7.08% (95%CI: 5.51, 8.42). Conclusions: We report pediatric Z-scores for normal LV volumes using the semi-automated method from five centers, enhancing its generalizability. 3DE evaluation of LV volumes and EF in pediatric patients is highly reproducible.

Background Strain and synchrony can be calculated from a variety of software packages, but there is a paucity of data with inter-vendor comparisons in children. To test the hypothesis that different packages may affect results, independent of acquisition, we compared values obtained using two commercially available analysis tool (QLAB and TomTec), with several different settings. Methods The study population included 108 children; patients were divided into three groups: (1) normal cardiac structure and conduction; (2) ventricular paced rhythm; and (3) flattened ventricular septum (reflecting right ventricular pressure or volume load lesions). We analyzed the same image acquired from the apical 4-chamber (AP4) and short-axis at the mid-papillary level (SAXM) views in both QLAB (versions 10.5 and 10.8) and TomTec (version 1.2). In QLAB version 10.8, low, medium, and high quantification smoothness settings were employed. In TomTec, images were analyzed with both low and high frame rates. Tracking quality for each package was graded. AP4 and SAXM strain and synchrony values were recorded. A mixed-effects linear regression model was used, with main effect considered significant if the p-value was Results Tracking scores were high for all packages except QLAB 10.5 in the SAXM view. AP4 and SAXM strain values varied significantly between QLAB 10.5 and the other packages. Synchrony values varied widely for all strain values (p Conclusion Synchrony values varied substantially among all packages in children. Strain values varied widely between QLAB 10.5 and all other software packages, recommending avoidance of QLAB 10.5 for future studies. Quantification smoothness settings in QLAB 10.8 resulted in minimal strain differences. In TomTec, low and high frame rate strain values differed only in a subset of patients (flattened septum). These data suggest that reliable comparisons between strain values derived from QLAB and TomTec is possible in certain cases, but that caution should be used especially in different hemodynamics conditions.

To report outcomes in a pilot study of autologous mitochondrial transplantation (MT) in pediatric patients requiring postcardiotomy extracorporeal membrane oxygenation (ECMO) for severe refractory cardiogenic shock after ischemia-reperfusion injury (IRI).A single-center retrospective study of patients requiring ECMO for postcardiotomy cardiogenic shock following IRI between May 2002 and December 2018 was performed. Postcardiotomy IRI was defined as coronary artery compromise followed by successful revascularization. Patients undergoing revascularization and subsequent MT were compared with those undergoing revascularization alone (Control).Twenty-four patients were included (MT, n = 10; Control, n = 14). Markers of systemic inflammatory response and organ function measured 1 day before and 7 days following revascularization did not differ between groups. Successful separation from ECMO-defined as freedom from ECMO reinstitution within 1 week after initial separation-was possible for 8 patients in the MT group (80%) and 4 in the Control group (29%) (P = .02). Median circumferential strain immediately following IRI but before therapy was not significantly different between groups. Immediately following separation from ECMO, ventricular strain was significantly better in the MT group (-23.0%; range, -20.0% to -28.8%) compared with the Control group (-16.8%; range, -13.0% to -18.4%) (P = .03). Median time to functional recovery after revascularization was significantly shorter in the MT group (2 days vs 9 days; P = .02). Cardiovascular events were lower in the MT group (20% vs 79%; P .01). Cox regression analysis showed higher composite estimated risk of cardiovascular events in the Control group (hazard ratio, 4.6; 95% confidence interval, 1.0 to 20.9; P = .04) CONCLUSIONS: In this pilot study, MT was associated with successful separation from ECMO and enhanced ventricular strain in patients requiring postcardiotomy ECMO for severe refractory cardiogenic shock after IRI.

Background Strain rate (SR) parameters derived from two-dimensional speckle-tracking echocardiography have prognostic value in children with heart disease. Routine use is hindered by a lack of normative data. The aim of this study was to determine reference values and Z scores for left ventricular systolic and diastolic SR in a large cohort of healthy children. Methods Echocardiograms from 577 subjects ≤18 years of age (mean age, 9.6 ± 5.6 years; range, 1 day to 18.0 years; 46% female) with structurally and functionally normal hearts were retrospectively included. Left ventricular longitudinal and circumferential systolic and early and late diastolic SR were measured using two-dimensional speckle-tracking echocardiography from the apical four-chamber and short-axis mid-papillary views. Associations with age and body surface area were assessed using Spearman correlation and generalized additive modeling. The relationship between systolic SR and wall stress (afterload) was examined. Analyses were conducted with and without correction for heart rate. Multivariable linear regression modeling was used to identify independent factors associated with the SR parameters. Z score equations were derived from a selected best-fit parametric model. Results All SR parameters differed significantly by age group. The magnitude of all SR values decreased with increasing age and body surface area. Systolic SR magnitude was inversely related to wall stress in children ≤7 years of age but not did not vary significantly in the older age groups. All relationships were maintained after heart rate correction. SR measurements had very good or excellent agreement. Conclusion Longitudinal and circumferential systolic and diastolic SR parameters are presented from a large cohort of healthy children using two-dimensional speckle-tracking echocardiography from the Philips platform. SR values differ significantly by age and body surface area. These results suggest that the myocardium becomes less sensitive to afterload with maturity. Z score equations based on age are presented, which should promote further clinical and research use.

Strain and synchrony are associated with clinical outcomes in children with heart diseases. Robust normative data for these values, measured by 2-dimensional speckle tracking echocardiography (2DSTE), are limited. Therefore, we aimed to derive normal ranges and z-scores of 2DSTE strain and synchrony parameters in children. Subjects were21 years old with structurally and functionally normal hearts. High frame-rate 2-dimensional echocardiographic images were retrospectively analyzed to measure longitudinal (LS) and circumferential (CS) strain and synchrony; views used were apical 4, 2, and 3-chamber (AP 4, 2, 3) and mid-papillary short-axis (SAX-M). Synchrony measures included standard deviation of time to peak strain, maximal wall delay, and cross-correlation mean segmental delay; these were calculated without and with heart rate (HR) correction (divided by √RR). Z-score equations were created for AP4 and SAX-M strain components. n = 312 subjects (40% female) were included (age 3 days to 20.5 years). Mean strain values (%) were: AP4 -24.4 ± 3.2, AP2 -24.2 ± 3.3, AP3 -24.6 ± 3.4, SAX-M -25.8 ± 3.4. Significant differences between ages were present for all strain components (AP4 p0.001; AP2 p = 0.003; AP3 p = 0.014; SAX-M p = 0.01). LS components decreased with increasing age and body surface area (p0.001 for all); CS did not. Longitudinal, but not circumferential, synchrony parameters decreased with age; however, these were nonsignificant after HR correction. In conclusion, normal pediatric 2DSTE strain and synchrony parameters and z-scores are reported to provide a foundation for incorporation into clinical practice. LS decline with age whereas CS does not. Age-related decreases in LS synchrony were mostly nonsignificant when corrected for HR.