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.

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.

Changes to the geometry and fluid mechanics of the carotid siphon in the pediatric Moyamoya disease.

BACKGROUND: The Moyamoya disease is a cerebrovascular disease that causes occlusion of the distal end of the internal carotid artery, leading to the formation of multiple tiny collateral arteries. To date, the pathogenesis of Moyamoya is unknown. Improved understanding of the changes to vascular geometry and fluid mechanics of the carotid siphon during disease may improve understanding of the pathogenesis, prognosis techniques and disease management. METHODS: A retrospective analysis of Magnetic Resonance Angiography (MRA) images was performed for Moyamoya pediatric patients (MMD) (n = 23) and control (Ctrl) pediatric patients (n = 20). The Ctrl group was composed of patients who complained of headache and had normal MRA. We performed segmentation of MRA images to quantify geometric parameters of the artery. Computational fluid dynamics (CFD) was performed to quantify the hemodynamic parameters. RESULTS: MMD internal carotid and carotid siphons were smaller in cross-sectional areas, and shorter in curved vascular length. Vascular curvature remained constant over age and vascular size and did not change between Ctrl and MMD, but MMD carotid siphon had lower tortuosity in the posterior bend, and higher torsion in the anterior bend. Wall shear stress and secondary flows were significantly lower in MMD, but the ratio of secondary flow kinetic energy to primary flow kinetic energy were similar between MMD and Ctrl. CONCLUSION: There were alterations to both the geometry and the flow mechanics of the carotid siphons of Moyamoya patients but it is unclear whether hemodynamics is the cause or the effect of morphological changes observed.

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.

Successful resection of a primary cardiac fibroma in a neonate: report of a case.

During the fetal-neonatal period, a primary cardiac tumor may be completely asymptomatic and such tumors may be incidentally discovered by echocardiography. A four-hour-old male was diagnosed to have a cardiac tumor by post-natal echocardiography and was observed closely. Surgery was indicated immediately at the 3 week follow-up examination when the tumor was found to have obstructed the right ventricle outflow. The tumor was resected successfully and its histopathology indicated that it was a fibroma. Follow-up echocardiograms and magnetic resonance imaging 5 months postoperatively demonstrated no evidence of any remaining tumor and his RV function was good.

[Clinical characteristics and imaging evaluation in children with renovascular hypertension].

OBJECTIVE: To characterize the clinical and angiographic features in children with renovascular hypertension. METHOD: Clinical data of 14 children (7 male, 7 female; age 0.8-14 years, mean 8.7 years), who were diagnosed with renovascular hypertension by renal angiography in our institute from January 2005 to December 2012 were collected and retrospectively analyzed. RESULT: The mean blood pressure at the diagnosis was 187/127 mm Hg. Chief complaints of symptomatic patients were headache (29%, 4/14), hypertensive encephalopathy (36%, 5/14), signs of congestive heart failure (14%, 2/14) and hematemesis (7%, 1/14). Renovascular hypertension was found incidentally in 14% (2/14) of patients who were asymptomatic. Conventional renal angiography elucidated the anatomical distribution of lesions in the renal arterial system. It was found that 14% (2/14) of patients had bilateral disease, 50% (7/14) had single stenosis at main or accessory renal artery, while multiple stenoses was seen in 43% (6/14) of children, with involvement of segmental renal artery and small interlobar or arcuate vessels. Compared with catheter angiography, 50% (7/14) of patients with renovascular hypertension, especially intrarenal arterial disease, were missed on computed tomography angiography or magnetic resonance angiography. CONCLUSION: It is mandatory to emphasize blood pressure measurement in pediatric clinical practice for early recognition of renovascular hypertension. As children with renovascular hypertension display involvement of multiple arteries, including in smaller intrarenal arteries, digital subtraction angiography is the only method that can reliably diagnose pediatric renovascular hypertension.

Multidetector CT angiography and 3D reconstruction in young children with coarctation of the aorta.

The purpose of this study was to assess the reliability of multidetector CT (MDCT) angiography and three-dimensional (3D) reconstruction in patients with coarctation of the aorta. Sixteen patients (median age, 2 months; range, 15 days to 28 months) with suspected coarctation of the aorta underwent both color Doppler echocardiography (CDE) and MDCT. In addition to the CT axial slices, 3D reconstructions such as volume rendering and multiple planar reformation were used to diagnose coarctation and associated cardiac abnormalities. The sensitivities of diagnosis were evaluated compared with MDCT and echocardiography and surgical findings. Sixteen cases were diagnosed as coarctation of the aorta by MDCT. This included hypoplasia of the aortic arch (n = 5). The results were confirmed at the time of surgery. Diagnosis sensitivities of coarctation were 87.5% for axial and 100% for both multiplanar and 3D volume-rendered images. Moreover, MDCT was able to clearly display the location and extent of the coarctation. Focal defects were observed in 14 cases, and diffuse defects in 2 cases. The sensitivity of MDCT diagnosis for coarctation of the aorta was 100%, which was higher than that of CDE (87.5%). Ventricular septum defect was diagnosed by MDCT in 11 cases, with 1 missed, and the sensitivity was 91.7% (11/12), which was lower than that of CDE (100%; 12/12). In addition, six patients had patent ductus arteriosus. Two patients in this study had collateral vessel formation. We conclude that CTA with 3D reconstruction represents a reliable noninvasive technique for the assessment of coarctation. It may serve as a noninvasive diagnostic tool before intervention or surgical treatment.