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Background Prenatal ultrasound is the preferred modality for diagnosing fetal congenital heart disease. Given issues of physician proficiency and hospital distribution, we propose a dynamic sequential cross-sectional scanning (SCS) to explore the feasibility of cardiac screening by sonographers with less than 5 years of experience in ultrasound. Materials and methods Twenty residents were randomly divided into two groups, receiving training in the American Institute of Ultrasound in Medicine (AIUM) fetal echocardiography and the SCS method. According to the needs of training, the professional staff developed the theoretical knowledge question bank, the CHD ultrasonic video disease bank, and the assessment scale. Trainees completed the pre-training examination, theory and skill operation training, and post-training assessment. For the two groups, the theoretical knowledge, skill operation and disease diagnosis were analyzed statistically before and after training. Results After training, the trainees in both groups had significantly improved knowledge and diagnostic abilities, their diagnostic thinking about CHD was clear, and they could identify major or even all structural abnormalities and make a definite diagnosis. In terms of skill operation, both groups could complete all required scanning within the specified time. The scanning time of the SCS group was significantly lower than that of the AIUM group, and the effect of the receptor site in the AIUM group was significantly higher than that in the SCS group. Conclusion SCS can be used as a new rapid fetal cardiac scanning method and try to popularize among echocardiographer.

PurposeWe evaluated the diagnostic utility of abdominal ultrasound (AUS), an adjunct to abdominal X-ray (AXR), for necrotizing enterocolitis (NEC) in congenital heart disease (CHD) patients.Methods86 patients with suspected NEC from 2009 to 2018 were classified as with CHD (n = 18) if they required cardiac intervention versus without CHD (n = 68). Clinical and radiological data were collected, including AXR and AUS concordance. Wilcoxon rank-sum test and Fisher’s exact test were performed.ResultsCHD patients had higher birth weights (p < 0.001) and gestational ages (p < 0.001) than non-CHD patients. CHD patients presented more frequently with hypotension (p = 0.041) and less frequently with bilious emesis (p < 0.001). Overall, CHD patients were less likely to have AUS findings of pneumatosis (33.3 vs. 72.1%; p = 0.005) and decreased mural flow (0 vs. 20.6%; p = 0.035) compared to non-CHD patients. On concordance analysis, CHD patients had 3.9-fold more discordant studies with pneumatosis on AXR but not on AUS (33.3 vs. 8.8%; p = 0.016) compared to non-CHD patients. Urgent surgery was required in 5.6% of CHD patients versus 16.2% of non-CHD patients.ConclusionCHD patients with suspected NEC represent a distinct clinical population. AUS has particular utility in assessing findings of bowel viability in the CHD NEC population, reflecting reduced rates of surgical NEC.

Objective This study aimed to explore the effectiveness of combining fetal heart sequential cross-sectional scanning with drawing methods, mind mapping, and case-based learning (CBL) for training in fetal conotruncal anomalies (CA) screening. Method An experimental control method was employed. Doctors participating in continuing fetal ultrasound education were randomly divided into two groups. Both groups received theoretical instruction on fetal heart embryology, development, and abnormal arterial trunk development. Group A received traditional teaching methods for both theory and skills, with the skills course focusing on routine fetal heart scanning. Group B received a mixed teaching approach incorporating pathological mind mapping, CBL teaching, and drawing methods for both theory and skills courses. Group B’s skills training focused on the sequential cross-sectional scanning method. The effectiveness of the training was evaluated by analyzing the classroom learning results and conducting a questionnaire survey of both groups. Result Group B participants achieved significantly higher scores on both theoretical and skills assessments compared to Group A. The diagnostic accuracy of different diseases in group B was significantly higher than that in group A, except for AVSD and APW. The post-class questionnaire revealed that participants in Group B expressed greater satisfaction with the combined teaching approach. They perceived a significant improvement in their independent learning abilities, diagnostic skills for related diseases, clinical skills, and overall competence compared to the traditional teaching group. The post-training correlation analysis did not identify any association between working years and theoretical or technical performance after training. Conclusion This study demonstrates that combining fetal heart sequential cross-sectional scanning with a variety of teaching methods, including drawing methods, mind mapping, and CBL, can enhance understanding of fetal trunk structure scanning and foster the development of clinical reasoning skills, ultimately leading to improved diagnostic accuracy in the identification and differential diagnosis of conotruncal anomalies.

Liver fibrosis and cirrhosis are one of the critical complications in Fontan patients. However, there are no well-established non-invasive and quantitative techniques for evaluating liver abnormalities in Fontan patients. Intravoxel incoherent motion diffusion-weighted imaging with MRI is a non-invasive and quantitative method to evaluate capillary network perfusion and molecular diffusion. The objective of this study is to assess the feasibility of intravoxel incoherent motion imaging in evaluating liver abnormalities in Fontan children. Five consecutive Fontan patients and four age-matched healthy volunteers were included. Fontan patients were 12.8 ± 1.5 years old at the time of MRI scan. Intravoxel incoherent motion imaging parameters (D, D*, and f values) within the right hepatic lobe were compared. Laboratory test, ultrasonography, and cardiac MRI were also conducted in the Fontan patients. Results of cardiac catheterization conducted within one year of the intravoxel incoherent motion imaging were also examined. In Fontan patients, laboratory test and liver ultrasonography showed almost normal liver condition. Cardiac catheter and MRI showed good Fontan circulation. Cardiac index was 2.61 ± 0.23 L/min/m2. Intravoxel incoherent motion imaging parameters D, D*, and f values were lower in Fontan patients compared with controls (D: 1.1 ± 0.0 versus 1.3 ± 0.2 × 10-3 mm2/second (p = 0.04), D*: 30.8 ± 24.8 versus 113.2 ± 25.6 × 10-3 mm2/second (p < 0.01), and f: 13.2 ± 3.1 versus 22.4 ± 2.4% (p < 0.01), respectively). Intravoxel incoherent motion imaging is feasible for evaluating liver abnormalities in children with Fontan circulation.

Summary Objective The aim of this study was to explore the feasibility of using low dose radiation and low concentration contrast media in enhanced CT examinations in children with congenital heart disease. Materials and Methods Ninety patients with congenital heart disease were randomly divided into three groups of 30 patients each who underwent contrast‐enhanced cardiac scans on a Discovery CT750 HD scanner. Group A received 270 mg I/mL iodixanol, and group B received 320 mg I/mL iodixanol contrast media and was scanned with prospective ECG triggering mode. Group C received 320 mg I/mL iodixanol and was scanned with conventional retrospective ECG gating mode. The same weight‐based contrast injection protocol was used for all three groups. Images were reconstructed using a 30% adaptive statistical iterative reconstruction (ASIR) algorithm and a 50% ASIR in groups A and B and a 30% ASIR in group C. The subjective and objective image quality evaluations, diagnostic accuracies, radiation doses and amounts of contrast media in the three groups were measured and compared. Results All images in the three groups met the diagnostic requirements, with the same diagnostic accuracy and image quality scores greater than 3 in a 4‐point scoring system. However, ventricular enhancement and the objective noise, signal‐to‐noise ratio, contrast‐to‐noise ratio and subjective image quality scores in group C were better than those in groups A and B (all P<.001). The effective radiation dose in groups A and B was 84% lower than that in group C (P<.001); group A received the lowest contrast dose (14% lower than that of groups B and C). Conclusion Enhanced CT scan images with low dose radiation and low concentration contrast media can meet the diagnostic requirements for examining children with congenital heart disease while reducing the potential risk of radiation damage and contrast‐induced nephropathy.

Background Dual-phase 3-dimensional whole-heart acquisition allows simultaneous imaging during systole and diastole. Respiratory navigator gating and tracking of the diaphragm is used with limited accuracy. Prolonged scan time is common, and navigation often fails in patients with erratic breathing. Image-navigation (iNAV) tracks movement of the heart itself and is feasible in single phase whole heart imaging. To evaluate its diagnostic ability in congenital heart disease, we sought to apply iNAV to dual-phase sequencing. Methods Healthy volunteers and patients with congenital heart disease underwent dual-phase imaging using the conventional diaphragmatic-navigation (dNAV) and iNAV. Acquisition time was recorded and image quality assessed. Sharpness and length of the right coronary (RCA), left anterior descending (LAD), and circumflex (LCx) arteries were measured in both cardiac phases for both approaches. Qualitative and quantitative analyses were performed in a blinded and randomized fashion. Results In volunteers, there was no significant difference in vessel sharpness between approaches ( p  > 0.05). In patients, analysis showed equal vessel sharpness for LAD and RCA ( p  > 0.05). LCx sharpness was greater with dNAV ( p  < 0.05). Visualized length with iNAV was 0.5 ± 0.4 cm greater than that with dNAV for LCx in diastole (p < 0.05), 1.0 ± 0.3 cm greater than dNAV for LAD in diastole ( p  < 0.05), and 0.8 ± 0.7 cm greater than dNAV for RCA in systole ( p  < 0.05). Qualitative scores were similar between modalities ( p  = 0.71). Mean iNAV scan time was 5:18 ± 2:12 min shorter than mean dNAV scan time in volunteers ( p  = 0.0001) and 3:16 ± 1:12 min shorter in patients ( p  = 0.0001). Conclusions Image quality of iNAV and dNAV was similar with better distal vessel visualization with iNAV. iNAV acquisition time was significantly shorter. Complete cardiac diagnosis was achieved. Shortened acquisition time will improve clinical applicability and patient comfort.

A forward genetic screen in fetal mice to identify genes involved in congenital heart disease (CHD) reveals that a large proportion of genes associated with CHD are related to cilia and cilia-transduced cell signalling, with potential implications for the human disease. Cilia defects in congenital heart disease The identification of genes causing congenital heart disease (CHD) has been challenging, in part because of the difficulty of distinguishing pathogenic mutations from random sequence genetic variability. Cecilia Lo and colleagues have therefore used a large-scale mouse forward genetic screen with chemical mutagenesis to recover mutations causing congenital heart disease. They identify 218 mouse models of the condition and, using whole-exome sequencing, 91 recessive mutations in 61 genes. A larger than expected proportion of these genes was found to be related to cilia and cilia-transduced cell signalling. Congenital heart disease (CHD) is the most prevalent birth defect, affecting nearly 1% of live births 1 ; the incidence of CHD is up to tenfold higher in human fetuses 2 , 3 . A genetic contribution is strongly suggested by the association of CHD with chromosome abnormalities and high recurrence risk 4 . Here we report findings from a recessive forward genetic screen in fetal mice, showing that cilia and cilia-transduced cell signalling have important roles in the pathogenesis of CHD. The cilium is an evolutionarily conserved organelle projecting from the cell surface with essential roles in diverse cellular processes. Using echocardiography, we ultrasound scanned 87,355 chemically mutagenized C57BL/6J fetal mice and recovered 218 CHD mouse models. Whole-exome sequencing identified 91 recessive CHD mutations in 61 genes. This included 34 cilia-related genes, 16 genes involved in cilia-transduced cell signalling, and 10 genes regulating vesicular trafficking, a pathway important for ciliogenesis and cell signalling. Surprisingly, many CHD genes encoded interacting proteins, suggesting that an interactome protein network may provide a larger genomic context for CHD pathogenesis. These findings provide novel insights into the potential Mendelian genetic contribution to CHD in the fetal population, a segment of the human population not well studied. We note that the pathways identified show overlap with CHD candidate genes recovered in CHD patients 5 , suggesting that they may have relevance to the more complex genetics of CHD overall. These CHD mouse models and >8,000 incidental mutations have been sperm archived, creating a rich public resource for human disease modelling.

The purpose of this study was to use hypnosis in patients with congenital heart disease undergoing transesophageal echocardiography (TEE). From January 2016 to July 2017, 50 adult patients undergoing TEE were randomly assigned to two groups: TEE in hypnosis (n = 23), TEE in sedation (n = 27). Vital parameters (heart rate [HR], blood pressure [BP], oxygen saturation [SO2] before, during and after the procedure) and drug administration were recorded. The State-Trait Anxiety Inventory was performed before and after TEE, the memory and experience of TEE through a structured interview were assessed. All patients in the hypnosis group performed TEE without any sedation. As for anxiety before TEE, no significant differences were observed between groups; after TEE all patients were less anxious than at the beginning (p<0.001) with a greater decrease in patients of the hypnosis group (p<0.001). Before TEE, there were no significant differences also in HR, BP and SO2. During TEE in both groups a similar increase in HR and BP was found (p<0.001), whereas SO2 values remained stable. In the responses to the structured interview, 94% of patients in the sedation group remembered everything vs 36% of the hypnosis group (p<0.05). No differences were found in the other answers between the two groups. Hypnosis in TEE is useful to improve the emotional experience of patients with congenital heart disease.

Patients with congenital heart disease (CHD) demonstrate altered structural brain network connectivity. However, there is large variability between reported results and little information is available to identify those patients at highest risk for brain alterations. Thus, we aimed to investigate if network connectivity measures were associated with the individual patient's cumulative load of clinical risk factors and with family‐environmental factors in a cohort of adolescents with CHD. Further, we investigated associations with executive function impairments. In 53 adolescents with CHD who underwent open‐heart surgery during infancy, and 75 healthy controls, diffusion magnetic resonance imaging and neuropsychological assessment was conducted at a mean age of 13.2 ± 1.3 years. Structural connectomes were constructed using constrained spherical deconvolution tractography. Graph theory and network‐based statistics were applied to investigate network connectivity measures. A cumulative clinical risk (CCR) score was built by summing up binary risk factors (neonatal, cardiac, neurologic) based on clinically relevant thresholds. The role of family‐environmental factors (parental education, parental mental health, and family function) was investigated. An age‐adjusted executive function summary score was built from nine neuropsychological tests. While network integration and segregation were preserved in adolescents with CHD, they showed lower edge strength in a dense subnetwork. A higher CCR score was associated with lower network segregation, edge strength, and executive function performance. Edge strength was particularly reduced in a subnetwork including inter‐frontal and fronto‐parietal‐thalamic connections. There was no association with family‐environmental factors. Poorer executive functioning was associated with lower network integration and segregation. We demonstrated evidence for alterations of network connectivity strength in adolescents with CHD — particularly in those patients who face a cumulative exposure to multiple clinical risk factors over time. Quantifying the cumulative load of risk early in life may help to better predict trajectories of brain development in order to identify and support the most vulnerable patients as early as possible. Adolescents with congenital heart disease demonstrate altered brain networks—particularly those who face a cumulative exposure to multiple risk factors over time. Early assessment of risk load could help predict brain development and support the most vulnerable patients early on.

Background Brain injury and neurodevelopmental impairment remain a concern in children with complex congenital heart disease (CHD). A practice guideline on neuromonitoring, neuroimaging, and neurodevelopmental follow-up in CHD patients undergoing cardiopulmonary bypass surgery is lacking. The aim of this survey was to systematically evaluate the current practice in centers across Europe. Methods An online-based structured survey was sent to pediatric cardiac surgical centers across Europe between April 2019 and June 2020. Results were summarized by descriptive statistics. Results Valid responses were received by 25 European centers, of which 23 completed the questionnaire to the last page. Near-infrared spectroscopy was the most commonly used neuromonitoring modality used in 64, 80, and 72% preoperatively, intraoperatively, and postoperatively, respectively. Neuroimaging was most commonly performed by means of cranial ultrasound in 96 and 84% preoperatively and postoperatively, respectively. Magnetic resonance imaging was obtained in 72 and 44% preoperatively and postoperatively, respectively, but was predominantly reserved for clinically symptomatic patients (preoperatively 67%, postoperatively 64%). Neurodevelopmental follow-up was implemented in 40% of centers and planned in 24%. Conclusions Heterogeneity in perioperative neuromonitoring and neuroimaging practice in CHD in centers across Europe is large. The need for neurodevelopmental follow-up has been recognized. A clear practice guideline is urgently needed. Impact There is large heterogeneity in neuromonitoring, neuroimaging, and neurodevelopmental follow-up practices among European centers caring for neonates with complex congenital heart disease. This study provides a systematic evaluation of the current neuromonitoring, neuroimaging, and neurodevelopmental follow-up practice in Europe. The results of this survey may serve as the basis for developing a clear practice guideline that could help to early detect and prevent neurological and neurodevelopmental sequelae in neonates with complex congenital heart disease.