Synthesis, structure anatomy, and catalytic properties of Ag14Cu2 nanoclusters co-protected by alkynyl and phosphine ligands.

We report the synthesis, structure anatomy, and catalytic properties of Ag14Cu2(CCArF)14(PPh3)4 (CCArF: 3,5-bis(trifluoromethyl)phenylacetylene) nanoclusters, denoted as Ag14Cu2. Ag14Cu2 has a robust electronic structure with two free valence electrons, and it has a distinctive absorbance feature. Single-crystal X-ray diffraction (SC-XRD) disclosed that Ag14Cu2 possesses an octahedral Ag6 metal kernel capped by two Ag4Cu1(CCArF)7(PPh3)2 metal-ligand units. Remarkably, it exhibits excellent bifunctional catalytic performance for 4-nitrophenol reduction and the electrochemical CO2 reduction reaction (eCO2RR). In 4-nitrophenol reduction, it adopts first-order reaction kinetics with a rate constant of 0.137 min-1, while in the eCO2RR, it shows a CO faradaic efficiency (FECO) of 83.71% and a high current density of 92.65 mA cm-2 at -1.6 V vs. RHE. Moreover, Ag14Cu2 showed robust long-term stability with no significant decay in current density and FECO over 10 h of continuous operation in the eCO2RR. This study not only enriches the potpourri of alkynyl-protected bimetallic AgCu nanoclusters, but also demonstrates the great potential of employing metal nanoclusters for bifunctional catalytic applications.

Clinical features and outcomes of children's interstitial lung disease accompanied with connective tissue disease: A prospective cohort study.

BACKGROUND: Medical complexity of childhood interstitial lung disease (chILD) with connective tissue disease (CTD) poses a considerable challenge to pediatricians. METHODS: Clinical characteristics, laboratory findings, pulmonary function tests (PFTs), treatments and outcomes obtained for patients with CTD-chILD were analyzed in a prospective study. RESULTS: Patients' median age at diagnosis was 7 years old. About 29.4% (15/51) suffered rapidly progressive childhood ILD (RP-chILD) with a high mortality rate (33.3%, 5/15), and the incidence of RP-chILD in juvenile idiopathic inflammatory myopathies was as high as 41.6% and the mortality rate was 30% (3/10). More than 70% patients had decreased diffusion capacity. The mean interval from symptoms-onset to diagnosis was 11.3 months. Compared to chILD with known CTD, the chILD proceeded CTD had a longer diagnosis interval, higher mortality, hospital stays and costs (P < 0.05). Lung imaging (33.3%) and lung function (72.7%) were partially reversible. The average survival time was 68.6 months. Cox univariate analysis showed that HRCT score ≥3, experiencing RP-chILD, cyanosis, acute respiratory distress syndrome (ARDS) and CD4 T cell <200 were significant predictors of death for chILD, whereas Cox multivariate analysis showed that ARDS was significant predictor of death for CTD-chILD, while IVIG support combined with corticosteroids and immunosuppressants was a protective factor. CONCLUSIONS: Care providers should conduct an assessment for CTD in chILD as a longer interval between the diagnosis of chILD and the CTD is associated with increased mortality. Complications as ARDS predict poor outcome in CTD-chILD, while IVIG support combined with corticosteroids and immunosuppressants is a protective factor.

Case report: The clinical utility of metagenomic next-generation sequencing in mucormycosis diagnosis caused by fatal Lichtheimia ramosa infection in pediatric neuroblastoma.

Lichtheimia ramosa (L. ramosa) is an opportunistic fungal pathogen of the order Mucorales that may result in a rare but serious mucormycosis infection. Mucormycosis could be angioinvasive, causing thrombosis and necrosis in the nose, brain, digestive tract, and respiratory tract. The infection is highly lethal, especially in immunocompromised hosts, and the incidence has been on the rise. However, due to its relatively low incidence in pediatric population and the challenges with diagnosis, the awareness and management experience for pediatric mucormycosis are extremely limited, which might lead to poor outcomes. In this study, we comprehensively reviewed the course of a fatal rhinocerebral mucormycosis case in a pediatric neuroblastoma patient receiving chemotherapy. Due to a lack of awareness of the infection, the standard care of amphotericin B treatment was delayed and not administered until the identification of L. ramosa by metagenomic next-generation sequencing (mNGS)-based pan-pathogen detection of the patient's peripheral blood sample. We also reviewed the literature on L. ramosa infection cases reported worldwide between 2010 and 2022, with an analysis of clinical manifestation, prognosis, and epidemiological data. Our study not only highlighted the clinical value of comprehensive mNGS in rapid pathogen detection but also raised awareness of recognizing lethal fungal infection early in immunocompromised hosts including pediatric cancer patients.

Structure, optical properties, and catalytic applications of alkynyl-protected M4Rh2 (M = Ag/Au) nanoclusters with atomic precision: a comparative study.

We report two atomically precise alloy nanoclusters of Ag4Rh2(CCArF)8(PPh3)2 and Au4Rh2(CCArF)8(PPh3)2 (Ar = 3,5-(CF3)2C6H3, abbreviated as Ag4Rh2 and Au4Rh2, respectively) co-protected by alkynyl and phosphine ligands. Both clusters have identical octahedral metal core configurations and can be termed superatoms with two free electrons. However, they possess different optical features, manifested by totally different absorbance peaks, and drastically different emission peaks, and also, Ag4Rh2 has a much higher fluorescence quantum yield (18.43%) than Au4Rh2 (4.98%). Moreover, Au4Rh2 exhibited markedly superior catalytic performance in the electrochemical hydrogen evolution reaction (HER), manifested by a much lower overpotential at 10 mA cm-2 and better stability. Density functional theory (DFT) calculations revealed that the free energy change of Au4Rh2 for the adsorption of two H* (0.64 eV) is lower than that of Ag4Rh2 for the adsorption of one H* (-0.90 eV) after stripping a single alkynyl ligand from the cluster. In contrast, Ag4Rh2 demonstrated much stronger catalytic capability for catalyzing 4-nitrophenol reduction. The present study provides an exquisite example to understand the structure-property relationship of atomically precise alloy nanoclusters, and emphasizes the importance of fine-tuning of the physicochemical properties and catalytic performance of the metal nanoclusters through modulating the metal core and beyond.

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.

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.

Unrelated umbilical cord blood transplantation for children with hereditary leukodystrophy: A retrospective study.

Objective: To analyze the efficiency of unrelated umbilical cord blood transplantation (UCBT) in the treatment of hereditary leukodystrophy following busulfan- and cyclophosphamide-based myeloablative chemotherapy. Methods: A retrospective study was performed in patients with hereditary leukodystrophy who underwent UCBT after myeloablative chemotherapy between April 2015 and March 2020. Results: The study cohort included 12 pediatric patients (ten males), nine with cerebral adrenoleukodystrophy (ALD) and three with juvenile globoid cell leukodystrophy (GLD). All received HLA-matched or partially mismatched unrelated UCBT. There were no cases of graft rejection. Median neutrophil engraftment time was 20 days [12-33 days] and median platelet engraftment time was 29 days [14-65 days]. Median follow-up was 36 months [1-86 months], and the overall survival rate for patients with cerebral ALD and juvenile GLD after UCBT was 77.8% (7/9) and 100% (3/3), respectively. In patients with ALD, although lipid profiles (serum very-long-chain fatty acid) were improved post-UCBT, six patients demonstrated worse neurologic function score and performance status post-UCBT, and six patients had higher Loes scores at last follow-up compared with baseline. In patients with juvenile GLD, all patients showed stable neurologic function score and performance status despite the Loes score of one patient increased slightly after transplantation. Conclusion: In patients with cerebral ALD, patients with no or mild neurological symptoms can benefit from UCBT, while UCBT cannot reverse advanced disease. In patients with juvenile GLD, UCBT is safe and contributes to stabilize neurological function.

Case report: A study on the de novo KMT2D variant of Kabuki syndrome with Goodpasture's syndrome by whole exome sequencing.

Kabuki syndrome (KS) is a rare genetic disorder characterized by dysmorphic facial features, skeletal abnormalities, and intellectual disability. KMT2D and KDM6A were identified as the main causative genes. To our knowledge, there exist no cases of KS, which were reported with pneumorrhagia. In this study, a 10-month-old male was diagnosed to have KS with typical facial features, skeletal anomalies, and serious postnatal growth retardation. Whole exome sequencing of the trio family revealed the presence of a de novo KMT2D missense variant (c.15143G > A, p. R5048H). The child was presented to the pediatric emergency department several times because of cough, hypoxemia, and anemia. After performing chest CT and fiberoptic bronchoscopy, we found that the child had a pulmonary hemorrhage. During research on the cause of pulmonary hemorrhage, the patient's anti-GBM antibodies gradually became positive, and the urine microalbumin level was elevated at the age of 12-month-old. After glucocorticoids and immunosuppressant therapy, the patient became much better. But he had recurrent pulmonary hemorrhage at the age of 16 months. Therefore, the patient underwent digital subtraction angiography (DSA). However, the DSA showed three abnormal bronchial arteries. This single case expands the phenotypes of patients with KS and Goodpasture's syndrome, which were found to have a de novo KMT2D missense variant.

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.

Engineering Gold Nanostructures for Cancer Treatment: Spherical Nanoparticles, Nanorods, and Atomically Precise Nanoclusters.

Cancer is a major global health issue and is a leading cause of mortality. It has been documented that various conventional treatments can be enhanced by incorporation with nanomaterials. Thanks to their rich optical properties, excellent biocompatibility, and tunable chemical reactivities, gold nanostructures have been gaining more and more research attention for cancer treatment in recent decades. In this review, we first summarize the recent progress in employing three typical gold nanostructures, namely spherical Au nanoparticles, Au nanorods, and atomically precise Au nanoclusters, for cancer diagnostics and therapeutics. Following that, the challenges and the future perspectives of this field are discussed. Finally, a brief conclusion is summarized at the end.

Early severity prediction of BPD for premature infants from chest X-ray images using deep learning: A study at the 28th day of oxygen inhalation.

BACKGROUND AND OBJECTIVE: Bronchopulmonary dysplasia is a common respiratory disease in premature infants. The severity is diagnosed at the 56th day after birth or discharge by analyzing the clinical indicators, which may cause the delay of the best treatment opportunity. Thus, we proposed a deep learning-based method using chest X-ray images of the 28th day of oxygen inhalation for the early severity prediction of bronchopulmonary dysplasia in clinic. METHODS: We first adopted a two-step lung field extraction method by combining digital image processing and human-computer interaction to form the one-to-one corresponding image and label. The designed XSEG-Net model was then trained for segmenting the chest X-ray images, with the results being used for the analysis of heart development and clinical severity. Therein, Six-Point cardiothoracic ratio measurement algorithm based on corner detection was designed for the analysis of heart development; and the transfer learning of deep convolutional neural network models were used for the early prediction of clinical severities. RESULTS: The dice and cross-entropy loss value of the training of XSEG-Net network reached 0.9794 and 0.0146. The dice, volumetric overlap error, relative volume difference, precision, and recall were used to evaluate the trained model in testing set with the result being 98.43 ± 0.39%, 0.49 ± 0.35%, 0.49 ± 0.35%, 98.67 ± 0.40%, and 98.20 ± 0.47%, respectively. The errors between the Six-Point cardiothoracic ratio measurement method and the gold standard were 0.0122 ± 0.0084. The deep convolutional neural network model based on VGGNet had the promising prediction performance, with the accuracy, precision, sensitivity, specificity, and F1 score reaching 95.58 ± 0.48%, 95.61 ± 0.55%, 95.67 ± 0.44%, 96.98 ± 0.42%, and 95.61±0.48%, respectively. CONCLUSIONS: These experimental results of the proposed methods in lung field segmentation, cardiothoracic ratio measurement and clinic severity prediction were better than previous methods, which proved that this method had great potential for clinical application.

Multi-site infection by methicillin-resistant Staphylococcus aureus in a six-year old girl: a case report.

BACKGROUND: Community-acquired Methicillin-resistant Staphylococcus aureus (CA-MRSA) is an emerging pathogen that leads to severe outcomes, especially in pediatric patients with multiple sites infection. CASE PRESENTATION: We report a case of multiple sites and life-threatening infection caused by CA-MRSA in a 6-year-old girl who manifested sepsis, myelitis, purulent arthritis, purulent meningitis, hydropericardium, pneumonia, and empyema. The girl exhibited good response to the combination therapy of linezolid and rifampicin after treatment failure of vancomycin with maximum dose due to its serum concentration unable to reach therapeutic goal. We performed pleural effusion and hydropericardium effusion drainage and treated left lower limb infection using interdisciplinary approaches. CONCLUSION: This case highlights the need to be aware of CA-MRSA infection, which requires accurate diagnosis, identification of infected sites, appropriate antibiotic treatment, and surgical debridement.

Respiratory symptoms as initial manifestations of interstitial lung disease in clinically amyopathic juvenile dermatomyositis: a case report with literature review.

BACKGROUND: Clinically amyopathic juvenile dermatomyositis (CAJDM) is a clinical subgroup of juvenile dermatomyositis (JDM), characterized by JDM rashes with little or no clinically evident muscle weakness. Interstitial lung disease (ILD) is an uncommon but potentially fatal complication of juvenile dermatomyositis (JDM). While adults with dermatomyositis-associated ILD usually present respiratory symptoms before or at the same time as skin muscle manifestations, only a few studies have covered the onset of respiratory symptoms of ILD in JDM patients, especially CAJDM. There is currently no clear effective treatment regime or any prognostic factors for CAJDM-associated ILD. CASE PRESENTATION: Here, we report the first case of a CAJDM patient who presented with respiratory symptoms as the initial manifestation. A 10-year-old male patient presented to the hospital with a complaint of progressive cough and chest pain. Violaceous macule and papules appeared a few days later and he was positive for anti-Ro-52 antibodies. Imaging showed diffuse interstitial infiltration in both lungs and lung function tests showed restrictive and obstructive ventilatory dysfunction. Muscular abnormalities were excluded by thigh magnetic resonance imaging (MRI) and electromyography. Skin biopsy showed pathognomonic findings consistent with DM. Lung biopsy indicated chronic inflammation of the mucosa. This patient was finally diagnosed with CAJDM complicated by ILD and prescribed methylprednisolone, immunoglobulin, prednisolone and mycophenolate mofetil (MMF) for treatment. The patient's cutaneous and respiratory manifestations were largely improved. We retrospectively reviewed this and another six cases with CAJDM-associated ILD reported previously to better understand its clinical characteristics and effective management. CONCLUSIONS: Initial respiratory symptoms with rapid progression in patients presenting Gottron papules should be considered manifestations of CAJDM-associated ILD. We also found a combination of corticosteroids, IVIG and MMF to be an effective method of arresting the progress of CAJDM-associated ILD and improving the prognosis of the patients.

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.

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.

Differential Effect of the Ratio of Right Ventricular Volume to Left Ventricular Volume in Children with Repaired Tetralogy of Fallot.

We aimed to compare the diagnostic value of indexed right ventricular end-diastolic volume (RVEDVi) and the ratio of right ventricle volume to left ventricle volume (RV/LV ratio) in prediction of the severity of pulmonary regurgitation (PR) expressed as the PR fraction (PRF) after surgery of tetralogy of Fallot (TOF). Forty-one patients with repaired TOF were included in the study. RVEDVi, LVEDVi, RV/LV ratio, PRF and ejection fraction were measured with magnetic resonance imaging. A PRF of more than 20% was considered significant. The predictive capability of two markers (RVEDVi and RV/LV ratio) for significant PR was compared using multivariate linear regression analysis and receiver operating characteristic (ROC) analysis. Both the RV/LV ratio and RVEDVi showed a correlation with PRF (r = 0.526/0.321, p = 0.001/0.041) in the correlation analysis, but in multivariate regression analysis the only independent predictor of PRF was the RV/LV ratio (F = 14.890, p = 0.001). ROC analysis revealed that a better discrimination of significant PR (>20%) from slight types (=20%) PR can be reached with the RV/LV ratio than RVEDVi (AUC = 0.805/0.709, p = 0.01). The RV/LV ratio was better than RVEDVi at differentiating mild from moderate PR (p = 0.006 vs. p = 0.153), and proved superior over RVEDVi in predicting PR based on the PRF criterion.

Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection.

BACKGROUND: Obstructed total anomalous pulmonary venous connection (TAPVC) is frequently misdiagnosed as pulmonary disease and without operative correction early death is common. It is important to make a correct diagnosis before surgery. OBJECTIVE: The purpose of this study was to describe the chest radiographic features of obstructed TAPVC and compare CT angiography with transthoracic echocardiography in the evaluation of obstructed TAPVC. MATERIALS AND METHODS: Eighteen children with obstructed TAPVC were assessed. Their clinical and imaging data were retrospectively reviewed. The characteristic radiographic findings were analyzed and compared with surgical results, and the diagnostic accuracy of CT angiography and transthoracic echocardiography was evaluated in terms of pulmonary venous drainage and obstruction detection. RESULTS: The common radiographic features included pulmonary venous congestion or edema or both (16 of 18 cases, 89%), and absence of cardiomegaly (12 of 18 cases, 67%). CT angiography correctly diagnosed TAPVC and clearly revealed the draining sites in all children (five with supracardiac TAPVC, three with cardiac TAPVC, eight with infracardiac TAPVC and two with mixed TAPVC). The diagnostic agreement between CT angiography and surgery was 100%. Transthoracic echocardiography only correctly revealed the draining sites in 11 children (5 with supracardiac TAPVC, 2 with cardiac TAPVC and 4 with infracardiac TAPVC). The diagnostic agreement between transthoracic echocardiography and surgery was 61%. The diagnostic accuracy of CT angiography was higher than that of transthoracic echocardiography (P = 0.0156). Thirty-four sites of obstruction were correctly detected by CT angiography (11 in the mediastinum, 1 at the diaphragmatic level, 9 below the diaphragm and 13 stenotic individual pulmonary veins in the lung). The diagnostic agreement between CT angiography and surgery was 92%. Transthoracic echocardiography only correctly detected 15 sites of obstruction (11 in the mediastinum, 1 at the diaphragmatic level and 3 below the diaphragm). The diagnostic agreement between transthoracic echocardiography and surgery was 41%. The rate of detection for sites of obstruction with transthoracic echocardiography was much lower than that of CT angiography (P = 0.0002). CONCLUSION: In children with obstructed TAPVC, plain radiographs usually show a characteristic pattern of pulmonary venous congestion or edema, or both, and a normal cardiac silhouette. CT angiography is superior to transthoracic echocardiography in the evaluation of pulmonary venous drainage and obstruction, especially in children with infracardiac and mixed TAPVC.