Feasibility of Three-Dimensional Echocardiographic Analysis in Newborns by Novice Observers.

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.

Common Blood Test Indices for Predicting Transient Abnormal Myelopoiesis-Related Mortality in Infants with Down Syndrome.

Transient abnormal myelopoiesis (TAM) can cause early death in children with Down syndrome, and liver failure is the most common cause of death. The aim of this single-center retrospective study was to identify a quantitative index for predicting TAM-related mortality at the time of diagnosis. Of the 462 children with Down syndrome admitted to our hospital from 1992 to 2021, we studied 12 infants with TAM-related death and 31 survivors who were diagnosed with TAM. In the death and survival groups, the median gestational ages were 34.9 and 37.1 weeks, respectively (p = 0.12). At diagnosis, the white blood cell (WBC) counts were 99.2 and 36.2 × 109/L (p = 0.011), the hemoglobin concentrations were 131 and 159 g/L (p = 0.009), and the serum albumin concentrations were 23 and 31 g/L (p < 0.001), respectively. The areas under the receiver operating characteristic curve for the abilities of the WBC count, hemoglobin, and serum albumin at diagnosis to predict survival were 0.75, 0.76, and 0.85, respectively. The serum albumin concentration threshold of 28 g/L at diagnosis had sensitivity of 0.79 and specificity of 0.82. Gestational age and serum albumin concentration were entered into a logistic regression model. The serum albumin concentration was an independent indicator of TAM-related death (adjusted odds ratio, 0.78; 95% confidence interval, 0.65-0.93; p = 0.005). In conclusion, a low serum albumin concentration at diagnosis may be a good predictor of TAM-related death.

Insufficient development of vessels and alveoli in lungs of infants with trisomy 18-Features of pulmonary histopathological findings from lung biopsy.

The aim of this study was to evaluate the features of pulmonary histopathological changes in cases of trisomy 18 complicated with congenital heart disease and pulmonary arterial hypertension. Twenty-eight patients with trisomy 18 underwent open lung biopsy at the time of primary operation in our hospital between 2008 and 2019. We compared these histopathological findings with those from previously described groups without trisomy 18. Mean age at primary cardiac surgery was 37 days (range, 9-69 days). According to the Heath-Edwards (HE) classification, 1, 8, 12, and 5 patients were graded as 0, 1, 2, and 3, respectively, whereas 2 patients were not classifiable due to medial defects in the small pulmonary arteries (MD). Four (14.3%) and 13 (46.4%) patients presented with MD and hypoplasia of the small pulmonary arteries (HS). Fifteen (53.6%) and 21 (75.0%) patients presented with alveolar hypoplasia (AH) and alveolar wall thickening (AT). MD, HS, and AH in trisomy 18 were present frequently, differing significantly from previous reports. These findings might be associated with congenital inadequate development of vessels and alveoli in the lung, contributing to a high risk of PAH in trisomy 18.