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Cardiac dysfunction is associated with mortality in children with hypoplastic left heart syndrome (HLHS). We evaluated the ability of qualitative and quantitative RV functional parameters to predict outcomes in HLHS patients. In this retrospective, single-center study, echocardiograms from 3 timepoints (pre-stage 1 palliation, 4–8 weeks post-stage 1 palliation, and pre-Glenn) were analyzed in infants with HLHS. Patients were stratified into two groups based on outcome of transplant-free survival post-Glenn (survivors) versus mortality or transplantation prior to Fontan (non-survivors). Images were retrospectively reviewed to obtain RV global longitudinal strain (RVGLS), RV-free wall strain (RVFWS), fractional area change (FAC), tricuspid annular systolic plane excursion (TAPSE), tissue motion annular displacement of the tricuspid valve (TMAD-TV) and qualitative systolic function assessment during the predetermined timepoints. An equal variance t-test and chi-square were used to determine significant differences and ROC curve analysis was performed to derive optimal cutoff values to predict mortality/transplant. A total of 47 patients met inclusion criteria, of which, 21 patients met composite endpoint. There were no significant differences in any RV functional parameter during the pre- or post-stage 1 palliation timepoints. The absolute values of RVFWS, RVGLS, and TMAD-TV were significantly greater in survivors than non-survivors during the pre-Glenn timepoint. A pre-Glenn RVGLS > −15.6 (AUC 0.79), RVFWS > −18.6 (AUC 0.75), and TMAD-TV < 12.6% (AUC 0.82) were sensitive and specific for predicting death or need for transplantation prior to Fontan completion. RVGLS, RVFWS, and TMAD-TV may help identify higher-risk HLHS patients during the interstage period. [ABSTRACT FROM AUTHOR]

Early detection of cardiac involvement in Juvenile Dermatomyositis (JDM) is difficult due to the absence of clinical signs and symptoms, with systolic dysfunction often emerging in late stages and associated with a poor prognosis. This study aimed to employ two-dimensional speckle-tracking echocardiography (STE) for subclinical assessment of left ventricular (LV) systolic failure in JDM and explore potential associations between impaired LV systolic function (LV-GLS) and disease activity. A prospective study enrolled 20 healthy volunteers and 26 JDM patients (< 18 years old) without cardiac symptoms. Clinical data were collected from medical records, and echocardiograms were conducted by a pediatric cardiologist. Our study cohort demonstrated similar age to controls (13.5 ±.6 vs. 13.8 ± 4.7; p = 0.465). Median illness duration at echocardiography was 5 (1.5–17.5) years, and conventional echocardiography indicated normal LV ejection fraction (> 55%) in all participants. However, STE revealed lowered LV GLS in JDM patients (− 22.2 ± 4.1% vs. − 26.5 ± 5.3% p = 0.022). Pulse steroid users displayed lower GLS average values compared to non-users (β = 4.99, 95% CI 1.34–8.64, p = 0.009). Negative correlations existed between LV-GLS and age at diagnosis (r = − 0.499; p = 0.011), diastolic parameters (E/E′ ratio) and age at diagnosis (r = − 0.469; p = 0.018), as well as RV global strain and age at diagnosis (r = − 0.443; p = 0.024). Employing STE in JDM patients facilitated the identification of preclinical cardiac dysfunction. Given JDM patients' younger age, early myocardial damage detection through STE may impact treatment decisions and long-term cardiovascular prognosis. [ABSTRACT FROM AUTHOR]

Multisystem inflammatory syndrome in children (MIS-C), is a rare but severe, hyperinflammatory complication of COVID-19, in which cardiovascular abnormalities are frequently detected. In this prospective study, we describe the echocardiographic findings in patients with MIS-C, with the use of conventional Echocardiography and Speckle-Tracking Echocardiography (STE) with Left Ventricular (LV) Global Longitudinal Strain (GLS) analysis, in the acute and follow-up phase. In total, 25 MIS-C patients [64% females, mean (± SD) age: 8.3 (± 3.72) years] were included. In the acute phase, median (IQR) Troponin and NT-proBNP and mean heart rate, were 8.07 (14.52) pg/mL, 2875.00 (7713.00) pg/mL, and 102.87 (± 22.96) bpm, respectively. Median (IQR) LV Ejection Fraction (LVEF) was 66 (8)% and LVEF impairment was detected in 2/25 (8%) patients. On follow-up (mean time interval:9.50 ± 4.59 months), heart rate was significantly lower, with a mean value of 90.00 (± 14.56) bpm (p-value = 0.017). Median (IQR) LVEF was 66.00 (6.70)% (p-value = 0.345) and all 25 participants had normal LVEF. In 14/25 patients, additional LV-GLS analysis was performed. During the acute phase, mean LV-GLS was − 18.02 (± 4.40)%. LV-GLS was abnormal in 6/14 patients (42.9%) and among them, only one patient had reduced LVEF. On follow-up (median (IQR) time interval:6.93 (3.66) months), mean LV-GLS was -20.31 (± 1.91)% (p-value = 0.07) and in 1/14 patient (7.1%), the LV-GLS impairment persisted. In conclusion, in the acute and follow-up phase, we detected abnormal LV-GLS values in some patients, in the presence of normal LVEF, indicating that STE-GLS is a valuable tool for identifying subclinical myocardial injury in MIS-C. [ABSTRACT FROM AUTHOR]

There is minimal information available regarding the early effects of bronchial asthma (BA) and its treatment on cardiac function in children. We used two-dimensional speckle-tracking echocardiography (2D-STE) to evaluate cardiac function before and after the treatment of childhood BA. We enrolled 44 children with moderate and severe BA who had not been treated over the preceding 3 months or who were newly diagnosed. All children received the same treatment (that recommended by the Global Initiative for Asthma [GINA] in 2017). All children also underwent transthoracic 2D-STE before treatment and 6 months later. Clinical data were compared before and after treatment. After treatment, significant increases were evident in right ventricular (RV) systolic and diastolic strain, as well as the systolic strain rate. Before and after treatment, the RV global longitudinal systolic strains were − 22.8 ± 3.6 and − 25.1 ± 4.5, respectively (p = 0.036); the RV global longitudinal diastolic strains were − 18.5 ± 6.0 and − 21.5 ± 5.2, respectively (p = 0.038); and the RV systolic strain rates were − 1.26 ± 0.4 and − 1.12 ± 0.3, respectively (p = 0.025). After treatment, significant increases were observed in the right atrial (RA) peak longitudinal strain and strain rate. Before and after treatment, the RA peak atrial longitudinal strains were 32.5 ± 10.8 and 44.7 ± 11.2, respectively (p = 0.042) and the RA longitudinal strain rates were − 1.6 ± 0.3 and − 2.0 ± 0.5, respectively (p = 0.041). RV and RA subclinical dysfunction may develop in children with early-stage BA. However, asthma treatment appears to improve such dysfunction. In children with BA, clinical and subclinical changes in cardiac functions can be easily detected via 2D-STE. [ABSTRACT FROM AUTHOR]

Three-dimensional echocardiography (3DE) provides better interobserver agreement than conventional methods. However, more evidence of whether there is good agreement between novice and experienced observers, especially in pediatric 3DE analysis, is required. We conducted 3DE analysis training in novice observers and investigated the agreement when analyzing 3DE images between novice and experienced observers. One experienced and 4 novice observers independently analyzed 60 3DE images obtained from neonates. The left and right ventricular end-diastolic volume (LVEDV and RVEDV), end-systolic volume (LVESV and RVESV), ejection fraction (LVEF and RVEF), left ventricular global longitudinal strain, and global circumferential strain (LVGLS and LVGCS) were calculated. The novices received hands-on instruction in the analysis procedure before the analysis and received further feedback after their first 40 analyses. Agreement between the novices and the experienced observer was evaluated by the intra-class correlation coefficient (ICC) and percentage difference in 3 groups of 20 images each (images 1–20, 21–40, and 41–60). The ICC of LVEDV was > 0.85 from the first 20 images and increased with experience. The ICCs for RVEDV and RVESV were low from the first 20 images but increased linearly, reaching an ICC > 0.9 in 3 of the 4 novice observers in the last 20 images. The range of the percentage difference was small for LVEDV, RVEDV, LVEF, and RVEF. Novices show an experienced level of 3DE analysis capability in LVEDV and RVEDV after analyzing 40 to 60 images. Training of novices is effective for analyzing LVEDV and RVEDV in 3DE images. [ABSTRACT FROM AUTHOR]

As the important role of longitudinal shortening in ventricular function has been well recognized over the past decade, evaluation of longitudinal systolic function of the left ventricle has become a subject of growing interest. Tissue motion annular displacement of the mitral valve (TMAD) is a new parameter of longitudinal systolic function. Although some studies have reported that this new parameter correlates with left ventricular ejection fraction (LVEF) in adults, little is known about TMAD in normal children. In this work, we investigated 94 children with no history of cardiovascular disease. TMAD was measured in the apical four-chamber view using the two-dimensional speckle tracking technique. Three points for tracking were selected in a diastolic frame: the lateral mitral valve annulus, medial mitral valve annulus, and left ventricular apex. The value was expressed as the percentage of displacement of the midpoint of the mitral valve annulus, using software to correct for left ventricular length at end-diastole. Pearson’s coefficient was used to estimate the correlation between TMAD and left ventricular systolic function parameters including the biplane modified Simpson method-derived ejection fraction and global longitudinal strain (GLS). We also analyzed the correlation between TMAD and heart rate (HR), height, age, and body surface area (BSA). TMAD was found to correlate significantly with LVEF (r = 0.71, p < 0.01) and GLS (r = −0.77, p < 0.01). However, no correlation was revealed for HR (r = −0.14, p = 0.19), height (r = −0.17, p = 0.10), age (r = −0.19, p = 0.09), or BSA (r = −0.19, p = 0.08). These results indicate that TMAD is useful for assessing LVEF and longitudinal systolic function in normal children, and is not influenced by changes in HR, height, age, or BSA. [ABSTRACT FROM AUTHOR]