Addition of gadolinium contrast to three-dimensional SSFP MR sequences improves the visibility of coronary artery anatomy in young children.

Objective: This study aims to compare the value of a gadolinium contrast-enhanced 1.5-T three-dimensional (3D) steady-state free precession (SSFP) sequence with that of a noncontrast 3D SSFP sequence for magnetic resonance coronary angiography in a pediatric population. Materials and methods: Seventy-nine patients from 1 month to 18 years old participated in this study. A 3D SSFP coronary MRA at 1.5-T was applied before and after gadolinium-diethylenetriaminepentaaceticacid (DTPA) injection. The detection rates of coronary arteries and side branches were assessed by McNemar's χ2 test. The image quality, vessel length, signal-to-noise ratio (SNR), and contrast-to-noise ratio (CNR) of the coronary arteries were analyzed by the Wilcoxon signed-rank test. The intra- and interobserver agreements were evaluated with a weighted kappa test or an intraclass correlation efficient test. Results: A contrast-enhanced scan detected more coronary arteries than a noncontrast-enhanced scan in patients under 2 years old (P < 0.05). The SSFP sequence with contrast media detected more coronary artery side branches in patients younger than 5 years (P < 0.05). The image quality of all the coronary arteries was better after the injection of gadolinium-DTPA in children younger than 2 years (P < 0.05) but not significantly improved in children older than 2 years (P > 0.05). The contrast-enhanced 3D SSFP protocol detected longer lengths for the left anterior descending coronary artery in children younger than 2 years and the left circumflex coronary artery (LCX) in children younger than 5 years (P < 0.05). SNR and CNR of all the coronary arteries in children younger than 5 years and the LCX and right coronary artery in children older than 5 years enhanced after the injection of gadolinium-DTPA (P < 0.05). The intra- and interobserver agreements were high (0.803-0.998) for image quality, length, SNR, and CNR of the coronary arteries in both pre- and postcontrast groups. Conclusion: The use of gadolinium contrast in combination with the 3D SSFP sequence is necessary for coronary imaging in children under 2 years of age and may be helpful in children between 2 and 5 years. Coronary artery visualization is not significantly improved in children older than 5 years.

Preoperative prediction of sonic hedgehog and group 4 molecular subtypes of pediatric medulloblastoma based on radiomics of multiparametric MRI combined with clinical parameters.

Purpose: To construct a machine learning model based on radiomics of multiparametric magnetic resonance imaging (MRI) combined with clinical parameters for predicting Sonic Hedgehog (SHH) and Group 4 (G4) molecular subtypes of pediatric medulloblastoma (MB). Methods: The preoperative MRI images and clinical data of 95 patients with MB were retrospectively analyzed, including 47 cases of SHH subtype and 48 cases of G4 subtype. Radiomic features were extracted from T1-weighted imaging (T1), contrast-enhanced T1 weighted imaging (T1c), T2-weighted imaging (T2), T2 fluid-attenuated inversion recovery imaging (T2FLAIR), and apparent diffusion coefficient (ADC) maps, using variance thresholding, SelectKBest, and Least Absolute Shrinkage and Selection Operator (LASSO) regression algorithms. The optimal features were filtered using LASSO regression, and a logistic regression (LR) algorithm was used to build a machine learning model. The receiver operator characteristic (ROC) curve was plotted to evaluate the prediction accuracy, and verified by its calibration, decision and nomogram. The Delong test was used to compare the differences between different models. Results: A total of 17 optimal features, with non-redundancy and high correlation, were selected from 7,045 radiomics features, and used to build an LR model. The model showed a classification accuracy with an under the curve (AUC) of 0.960 (95% CI: 0.871-1.000) in the training cohort and 0.751 (95% CI: 0.587-0.915) in the testing cohort, respectively. The location of the tumor, pathological type, and hydrocephalus status of the two subtypes of patients differed significantly (p < 0.05). When combining radiomics features and clinical parameters to construct the combined prediction model, the AUC improved to 0.965 (95% CI: 0.898-1.000) in the training cohort and 0.849 (95% CI: 0.695-1.000) in the testing cohort, respectively. There was a significant difference in the prediction accuracy, as measured by AUC, between the testing cohorts of the two prediction models, which was confirmed by Delong's test (p = 0.0144). Decision curves and nomogram further validate that the combined model can achieve net benefits in clinical work. Conclusion: The combined prediction model, constructed based on radiomics of multiparametric MRI and clinical parameters can potentially provide a non-invasive clinical approach to predict SHH and G4 molecular subtypes of MB preoperatively.

A newly identified pyroptosis-related gene signature for predicting prognosis of patients with hepatocellular cancer.

Background: Hepatocellular carcinoma (HCC) is a very heterogeneous illness, making prognosis prediction a huge problem. Pyroptosis, which has recently been shown to be an inflammatory type of programmed cell death, is involved in HCC. Nevertheless, the role of pyroptosis-related genes in HCC has not been fully elucidated. Thus, this study aimed to construct a prognostic signature based on pyroptosis-related genes for HCC. Methods: The messenger RNA expression patterns of HCC patients, as well as the accompanying clinical information, were retrieved from The Cancer Genome Atlas (TCGA) database for this research. After differentially expressed pyroptosis-related Gene in tumor and normal groups were identified, Cox regression analyses were performed to construct a prognostic signature which was then assessed through independent prognostic analysis. Results: A signature consisting of four genes (CASP8, GSDME, NOD2, and PLCG1) was constructed to predict overall survival (OS) for HCC. The signature was identified to be independent by the cox regression analysis and obtained the largest area under the receiver operating characteristic (ROC) curve (AUC) was 0.691, 0.628, and 0.632 for survival at 1, 2, and 3 years, respectively. Conclusions: We discovered that the levels of pyroptosis-related genes expression differed across HCC patients and were associated with both survival and prognosis. This suggested that targeting pyroptosis as a treatment strategy for HCC may be a viable option.

Relationship between bronchopulmonary dysplasia phenotypes with high-resolution computed tomography score in early preterm infants.

Objective: To assess the relationship between high-resolution computed tomography (HRCT) abnormalities and clinical phenotypes of bronchopulmonary dysplasia (BPD). Methods: A retrospective, single-center study was carried out at the Children's Hospital of Fudan University between 2013 and 2020. Preterm infants born at ≤ 32 weeks' gestation who were diagnosed with BPD and had HRCT between 40 and 50 weeks postmenstrual age (PMA)were included in the study. HRCT images from six pulmonary lobes were scored based on seven types of pulmonary lesions from two categories: hyperaeration lesions and parenchymal lesions. The hyperaeration score (HS) included scores of decreased attenuation, mosaic attenuation, and bulla/bleb, while the parenchymal score (PS) included those of linear lesion, consolidation, bronchial wall thickening, and bronchiectasis. All seven scores were summed up to create the total score (TS). One-way ANOVA testing or Kruskal-Wallis testing was adopted for the comparison of HRCT scores with BPD severity and clinical phenotypes. The correlation between HRCT scores and clinical phenotypes was evaluated by Spearman's correlation analysis. Results: A total of 81 cases were included in the study. Cases with more severe BPD had a higher TS (p = 0.01), HS (p = 0.02), PS (p = 0.02), mosaic attenuation score (p = 0.03), bulla/Bleb score (p = 0.03), and linear density score (p = 0.01). TS (r = 0.28), PS (r = 0.35), linear density (r = 0.34), and consolidation (r = 0.24) were correlated with pulmonary hypertension (PH). However, no HRCT score was significantly different between the patients with or without tracheobronchomalacia (TBM). BPD patients with a combination of lung parenchymal disease, PH, and TBM had the highest TS and HS. Conclusion: HRCT scores correlated with BPD severity and PH in our study. HS might be a useful tool in the assessment of BPD severity while linear densities and consolidation might be helpful in predicting PH.

Imaging characteristics of pulmonary BCG/TB infection in patients with chronic granulomatous disease.

In China, tuberculosis (TB) is endemic and the Bacillus Callmette-Güerin (BCG) vaccine is administered to all the newborns, which may lead to BCG infection in patients with chronic granulomatous disease (CGD). Infection of BCG/TB in CGD patients can be fatal and pulmonary is the most affected organ. Our objective was to assess the imaging of pulmonary BCG/TB infection in CGD. We screened 169 CGD patients and identified the patients with pulmonary BCG/TB infection. BCG infection was diagnosis according to the vaccination history, local infection manifestation, acid-fast bacilli staining, specific polymerase chain reaction, and/or spoligotyping. PPD, T-SPOT and acid-fast bacilli staining were used for diagnosis of TB. Totally 58 patients were identified, including TB (n = 7), solely BCG (n = 18), BCG + bacterial (n = 20), and BCG + fungi (n = 13). The onset of BCG disease was much earlier than TB. For those patients only with BCG, lymphadenopathy was the first and most prevalent feature. The most found location was the left axilla, followed by the ipsilateral cervical areas and mediastinal or hilar area. On chest CT, ground-glass opacities, multiple nodules and pulmonary scarring were the most common findings. For TB patients, the pulmonary infections were more serious, including large masses, severe lymphadenopathy, and extensive pulmonary fibrosis. Pulmonary infection of BCG were more common than TB in CGD patients, but much less severe.

Evaluation of comprehensive myocardial contractility in children with Kawasaki disease by cardiac magnetic resonance in a large single center.

BACKGROUND: Children with Kawasaki disease (KD) and coronary artery lesions (CALs) can develop myocardial ischemia, fibrosis, and abnormal contractility. We aimed to assess the association between myocardial mechanical deformation with myocardial fibrosis, ischemia, and CALs. METHODS: In total, 76 KD and 20 healthy volunteers received cardiac magnetic resonance (CMR). Peak systolic left ventricular (LV) longitudinal, radial, and circumferential strain and strain rate [LV strain longitudinal (LVSL), LV strain radial (LVSR), LV strain circumferential (LVSC), LV strain rate longitudinal (LVSRL), LV strain rate radial (LVSRR), and LV strain rate circumferential (LVSRC)], along with late gadolinium enhancement (LGE), perfusion deficit, and CALs in related segments were analyzed. The KD group was subdivided by CALs, perfusion, and LGE results, and strain results were compared with controls and in subgroups. RESULTS: Cardiac fibrosis and ischemia were not confined to the territory of CALs. In a global analysis, strain and strain rates were lower in the KD group, especially in the subgroup with LGE and perfusion deficit. In segmental analysis, LVSR, LVSC, LVSL, and LVSRR decreased in the giant aneurysm group, and a lower LVSR (20.369%±10.603% vs. 26.071%±12.349%) and LVSC (-13.37%±5.365% vs. -15.847%±5.778%) were observed in thrombosed segments. The strain and strain rate were all lower in segments with LGE and perfusion deficit, and no obvious difference was found between groups with and without stenosis. LVSR had a better ability to identify giant aneurysm, thrombosis, stenosis, perfusion deficit, and LGE. CONCLUSIONS: We detected lower strain values in KD patients, which was more pronounced in segments with aneurysm, thrombi, LGE, and perfusion deficit. LVSR is useful to discern patients with higher risk.

Diagnostic performance of magnetic resonance enterography and ultrasound in children with inflammatory bowel diseases: a diagnostic test accuracy meta-analysis.

OBJECTIVES: Magnetic resonance enterography (MRE) and ultrasound (US) can be used to diagnose inflammatory bowel diseases (IBD) in children. This meta-analysis aimed to determine the diagnostic performance of MRE and US in pediatric patients with IBD. METHODS: PubMed, Embase, and the Cochrane Library were searched for eligible studies published up to June 1, 2020. The outcomes were the performances of MRE and US at the segment and patient levels. Pooled sensitivity, specificity, positive and negative likelihood ratios (PLR and NLR), diagnostic odds ratio (DOR), and the area under the summary receiver operating characteristic curves value (SROC) were analyzed. RESULTS: Eight studies (340 children) were included. Compared with the reference standard, MRE showed pooled sensitivity of 93.0% (95% confidence interval (CI): 90.0-95.4%), specificity of 94.6% (95% CI: 92.1-96.5%), PLR of 11.146 (95% CI: 5.027-24.713), NLR of 0.094 (95% CI: 0.057-0.155), and DOR of 134.21 (95% CI: 40.72-442.29), with a SROC of 0.9721. Similar results were observed at the patient and segment levels. Compared with the reference standard, US had pooled sensitivity of 84.1% (95% CI: 69.9-93.4%), specificity of 82.9% (95% CI: 66.4-93.4%), PLR of 4.924 (95% CI: 2.351-10.310), NLR of 0.207 (95% CI: 0.103-0.413), and DOR of 25.919 (95% CI: 7.63-88.07), but only two studies were included. US (reader 1) had a similar diagnostic value to US (reader 2). CONCLUSIONS: The present meta-analysis shows that MRE has good performance in detecting IBD in pediatric patients. Only two studies used US, and additional studies are necessary to confirm the diagnostic performance of US for IBD in children. KEY POINTS: • MRE has good performance in the detection of IBD in pediatric patients. • Similar results were observed at the patient and segment levels for MRE. • Only two studies were included for US, without differentiating patient/segment.

Midterm Outcomes of Angioplasty for Pediatric Renovascular Hypertension.

PURPOSE: To evaluate the midterm outcomes of percutaneous transluminal renal angioplasty (PTRA) for pediatric renovascular hypertension (RVH). MATERIALS AND METHODS: The clinical data of patients who underwent PTRA for RVH in the authors' hospital from 2012 to 2019 were retrospectively analyzed. Postprocedural blood pressure, glomerular filtration rate (GFR) of the affected kidney, restenosis, and complications were closely monitored. RESULTS: PTRA was performed in a total of 30 children (20 boys and 10 girls), with a mean age of 7.3 years ± 0.7 (range, 40 days to 13.9 years) and a mean weight of 25.0 kg ± 2.3 (range, 3.4-53 kg). The median follow-up period was 26.5 months (range, 1 month to 7.5 years). Technical success was achieved in 26 (86.7%) of the 30 patients. Restenosis developed in 3 patients (10.0%). Only 1 patient underwent stent implantation, and the stent fractured 8 months later, requiring further intervention. There were no other complications. In terms of clinical benefit of blood pressure control after the initial PTRA procedure, 15 patients (50%) were cured and 7 patients (23.3%) showed improvement. There was no significant difference in the etiology, lesion location, and lesion length between patients with clinical benefit and failure (P = .06, P = .202, and P = .06, respectively). GFR of the affected kidney was significantly improved from 19.9 mL/min ± 11.2 to 38.1 mL/min ± 11.9 at the 6-month follow-up after PTRA (P < .001). CONCLUSIONS: The overall results of PTRA for pediatric RVH caused by different etiologies are promising. PTRA not only provided a clinical benefit of blood pressure control in 73.3% of the patients but also significantly improved the function of the affected kidney.

Early detection relationship of cerebral palsy markers using brain structure and general movements in infants born <32 weeks gestational age.

AIM: To detect early brain structural and clinical functional markers of brain injury and development based on a magnetic resonance imaging (MRI) scoring system and a general movement assessment (GMA) for preterm infants later diagnosed with cerebral palsy (CP). STUDY DESIGN: Retrospective cohort study. General movements (GMs) were scored according to a semiquantitative scoring system: the GMs optimality score (GMOS) at preterm and term ages and the Motor Optimality Score (MOS) at the corrected age of 3 months after birth. Brain magnetic resonance imaging (MRI) at term-equivalent age was scored using an MRI scoring system. We analyzed the relationship between the early degree of cerebral white matter (WM) abnormality and the GMOS and the MOS for infants born <32 weeks gestational age later diagnosed with CP in a comparison group of neurotypical controls. SUBJECTS: Sixteen preterm infants were included in this study who underwent MRI and GMs assessment. 8 out of the 16 preterm infants were later diagnosed with CP, while the other 8 infants with normal motor development (N) were placed into the control group. Their median gestational age was 30w6d and 27w6d for each group respectively. RESULTS: The cerebral WM MRI scores were significantly higher in the CP group than in the control group (p < 0.01). The GMOS and MOS were significantly higher in the control group than in the CP group (p < 0.05). The MOS showed a strong correlation to the cerebral WM MRI score (r = -0.88) and the subscale of cerebral WM items (the cystic degeneration and the focal signal abnormalities) of the MRI score (r = -0.94) in the CP group. The MOS also showed a correlation with corrected biparietal diameter (cBPD) in the preterm infant group with CP (r = 0.75). Results of linear regression analyses between term MRI and GMs measures in preterm infants with CP are presented. Cerebral WM scores were associated with the MOS (ß = -0.63; 95%CI = -0.97, -0.29; p < 0.01). Cerebral WM injury, including the subscale of cystic degeneration and focal signal abnormalities was closely associated with the MOS (ß = -0.83; 95%CI = -1.13, -0.54; p < 0.001). CONCLUSION: Cerebral WM scores show a strong association with a decreased motor performance on the MOS in preterm infants later diagnosed with CP. Severe white matter injury and significantly decreased MOS scores may provide useful early markers and strong evidence to early predict the risk of later development of cerebral palsy in preterm infants.

Cardiac magnetic resonance feature tracking of the right ventricle in convalescent Kawasaki disease in a large single center.

BACKGROUND: The changes in right ventricular (RV) contractility of Kawasaki disease (KD) still remain unclear. HYPOTHESIS: We aimed to determine whether RV systolic dysfunction can be detected by cardiac magnetic resonance (CMR) feature tracking and to find its association with coronary artery lesions (aneurysm, thrombosis and stenosis). METHODS: Peak systolic myocardial longitudinal, radial and circumferential strain and the strain rate (RVSL, RVSR, RVSC, RVSRL, RVSRR and RVSRC) in the global RV and three levels (basal, middle and apical) were measured in 66 patients with convalescent KD. A total of 20 controls were included. Comparisons were made with controls and among KD subgroups divided with coronary artery lesions. RESULTS: RVSC (-10.575% vs. -10.760%), RVSL (-18.150% vs. -18.712%) and RVSRC (-0.815/s vs. -0.924/s) were slightly lower in KD group without significant difference. All the strain and strain rate presented lowest in the basal level. In subgroup comparison, lower RVSL and RVSRL were observed in the giant coronary artery aneurysm (CAA) group; RVSR (15.844% vs. 16.897%), RVSRR (1.245/s vs. 1.322/s) and RVSRC (-0.715/s vs. -0.895/s) were lower in thrombosed group; RVSRL (-1.27/s vs. -1.503/s) were lower in stenosis group. All the comparison in subgroups did not reach significant difference. From the analysis of receiver operating characteristic curve, RVSRL had a better ability to identify KD with giant CAA and stenosis. For the identification of thrombosis, RVSRC had a better ability. CONCLUSIONS: Lower strain and strain rates of RV were detected in convalescent KD. More pronounced in those with persisting coronary artery lesions.

Imaging findings of pulmonary manifestations of chronic granulomatous disease in a large single center from Shanghai, China (1999-2018).

Chronic granulomatous disease (CGD) is characterized by recurrent infections and granuloma formation in multiple organs, especially the lung. We aimed to investigate pulmonary manifestations by computed tomography (CT). In total, 100 patients with 117 episodes of pulmonary infection were included. Chest CT scans of every episode were analyzed. Random nodules were the most common findings (79.49%), followed by ground-grass opacities (74.36%), focal consolidations (62.39%), and masses (59.83%). Cavities (12.82%) and multiple small abscesses (17.09%) could be found in the consolidations and masses. CT revealed interstitial pneumonia with tree-in-bud opacities (17.09%), interlobular septal thickening (23.08%) and emphysema (35.04%), which were more severe in the bilateral upper lobes. Mediastinal and hilar lymphadenopathy (78.63%) and axillary lymphadenopathy (65.81%) were common. Fungal infection (n = 27) was the most common and presented with multiple nodules and masses. Approximately 1/4 of fungal infections had interstitial pneumonia. In Staphylococcus aureus (n = 6) and Klebsiella pneumoniae (n = 3) infections, large areas of consolidation were common. In tuberculosis infection, the pulmonary infections were more severe and complex. For Bacillus Calmette-Guérin disease, left-sided axillary lymphadenopathy was a characteristic manifestation. CT images of CGD demonstrated variable pulmonary abnormalities. The main infectious organisms have unique imaging features.

Esophageal Lung in Association with Pulmonary Vascular Anomalies.

We present a 7.8-month-old girl with recurrent pneumonia, growth retardation, and tachypnea. Computed tomography revealed a particularly unusual esophageal lung, in association with pulmonary vascular anomalies of right pulmonary veins atresia and right pulmonary artery hypoplasia.

A novel TSC2 missense variant associated with a variable phenotype of tuberous sclerosis complex: case report of a Chinese family.

BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder characterized by the development of hamartomas in multiple organs, including the brain, heart, skin, kidney, lung and retina. A diagnosis of TSC is established with a recently revised clinical/radiological set of criteria and/or a causative mutation in TSC1 or TSC2 gene. CASE PRESENTATION: We report a Chinese TSC family with two siblings presenting with multiple hypomelanotic macules, cardiac rhabdomyomas and cortical tubers associated with a small subependymal nodule. The older child had seizures. A novel heterozygous missense variant in the TSC2 gene (c.899G > T, p.G300 V) was identified and shown to be inherited from their father as well as paternal grandfather, both of whom presented with variable TSC-associated signs and symptoms. CONCLUSION: We identified a novel heterozygous TSC2 variant c.899G > T as the causative mutation in a Chinese family with TSC, resulting in wide intrafamilial phenotypic variability. Our study illustrates the importance of clinical evaluation and genetic testing for family members of the patient affected with TSC.

High-Performance, Self-Powered Photodetectors Based on Perovskite and Graphene.

An ideal photodetector must exhibit a fast and wide tunable spectral response, be highly responsive, have low power consumption, and have a facile fabrication process. In this work, a self-powered photodetector with a graphene electrode and a perovskite photoactive layer is assembled for the first time. The graphene electrode is prepared using a solution transfer process, and the perovskite layer is prepared using a solution coating process, which makes the device low cost. Graphene can form a Schottky junction with TiO2 to efficiently separate/transport photogenerated excitons at the graphene/perovskite interface. Unlike the conventional photovoltaic structure, in this photodetector, both photogenerated electrons and holes are transported along the same direction to graphene, and electrons tunneled into TiO2 are collected by the cathode and holes transported by graphene are collected by the anode; therefore, the photodetector is self-powered. The photodetector has a broad range of detection, from 260 to 900 nm, an ultrahigh on-off ratio of 4 × 106, rapid response to light on-off (<5 ms), and a high level of detection of ∼1011 Jones. The high performance is primarily due to the unique charge-transport property of graphene and strong light absorption properties of perovskite. This work suggests a new method for the production of self-powered photodetectors with high performance and low power consumption on a large scale.

Magnetic Field-Assisted Perovskite Film Preparation for Enhanced Performance of Solar Cells.

Perovskite solar cells (PSCs) are promising low-cost photovoltaic technologies with high power conversion efficiency (PCE). The crystalline quality of perovskite materials is crucial to the photovoltaic performance of the PSCs. Herein, a simple approach is introduced to prepare high-quality CH3NH3PbI3 perovskite films with larger crystalline grains and longer carriers lifetime by using magnetic field to control the nucleation and crystal growth. The fabricated planar CH3NH3PbI3 solar cells have an average PCE of 17.84% and the highest PCE of 18.56% using an optimized magnetic field at 80 mT. In contrast, the PSCs fabricated without the magnetic field give an average PCE of 15.52% and the highest PCE of 16.72%. The magnetic field action produces an ordered arrangement of the perovskite ions, improving the crystallinity of the perovskite films and resulting in a higher PCE.

Air and thermally stable perovskite solar cells with CVD-graphene as the blocking layer.

The poor air and thermal stability of organic-inorganic halide perovskite solar cells have hindered their real applications. Here we report the insertion of a chemical vapor deposited graphene between the Au electrode and spiro-OMeTAD in planar perovskite solar cells to block the diffusion of air and Au into the perovskite layer, where the single layer graphene is transferred into the devices by a simple laminated process. After ageing in 45% humidity air for 96 h or thermal annealing at 80 °C for 12 h, more than 94% PCE of the devices with graphene can be maintained, which is much better than that of devices without graphene (∼57%). The improved stability of devices with the graphene layer is attributed to the reduction of carrier recombination from decomposition of the perovskite layer in air or Au doping into the perovskite layer under annealing treatment. Therefore, graphene is a promising ultra-thin barrier against air and metal diffusion, and has potential applications in photovoltaic devices, integrated circuit chips and light emitting diodes.

Anomalous origin of the left coronary artery from the pulmonary artery in children: diagnostic use of multidetector computed tomography.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly. It is important to demonstrate the anomalous origin of the left coronary artery and its course before surgery. OBJECTIVE: To explore the clinical diagnostic use of multidetector CT coronary angiography in detecting anomalous origin of the left coronary artery from the pulmonary artery in children. MATERIALS AND METHODS: Nine children (2 boys, 7 girls) ages 2 months to 9 years with surgically confirmed anomalous origin of the left coronary artery from the pulmonary artery were studied. Clinical data, transthoracic echocardiography and CT coronary angiography images were retrospectively analyzed. RESULTS: Transthoracic echocardiography correctly diagnosed anomalous origin of the left coronary artery from the pulmonary artery in 7 of 9 patients (95% CI: 40-97%). CT coronary angiography revealed the anomalous origin of the left coronary artery in all children (95% CI: 66-100%). In a 4-year-old girl and a 9-year-old girl, CT coronary angiography showed dilation of the right coronary artery and collateral circulation between the right and the left coronary arteries. CONCLUSION: CT coronary angiography is a useful method to show the anomalous origin of the coronary artery in children with anomalous origin of the left coronary artery from the pulmonary artery, especially for patients in whom origin of the left coronary artery cannot be detected by transthoracic echocardiography.

Interventional embolization of congenital intrahepatic shunts in children.

BACKGROUND: Congenital intrahepatic shunts in children are rare and precise treatment strategies have not been established. OBJECTIVE: The purpose of this study was to present our experience with transcatheter embolization in a descriptive case series of congenital intrahepatic shunts in children. MATERIALS AND METHODS: We retrospectively studied 10 children with symptomatic congenital intrahepatic shunts who were treated with embolization at our institute between January 2008 and December 2014. RESULTS: Seven children had arteriovenous shunts in association with hepatic hemangiomas, two had arterioportal shunts and one had portosystemic shunts. The major presentations were congestive heart failure and severe anemia in the groups with arteriovenous and arterioportal shunts, respectively. Following embolization, two children died in the arteriovenous group, one from progressive liver dysfunction and the other from abdominal compartment syndrome. One child in the arterioportal group required liver transplantation after initial embolization. With mean post-procedure follow-up of 15 months (range 4-54 months), all the remaining children were well, with resolution of the symptoms. CONCLUSION: Interventional embolization provides an alternative to surgical ligation or hepatic resection in children with clinically significant intrahepatic shunts. For lesions with complex morphology, embolization may be inadequate and liver transplantation might be the only therapeutic option.