Dynamic Contrast-enhanced MRI Quantification of Altered Vascular Permeability in Autoimmune Encephalitis.

Background Blood-brain barrier (BBB) permeability change is a possible pathologic mechanism of autoimmune encephalitis. Purpose To evaluate the change in BBB permeability in patients with autoimmune encephalitis as compared with healthy controls by using dynamic contrast-enhanced (DCE) MRI and to explore its predictive value for treatment response in patients. Materials and Methods This single-center retrospective study included consecutive patients with probable or possible autoimmune encephalitis and healthy controls who underwent DCE MRI between April 2020 and May 2021. Automatic volumetric segmentation was performed on three-dimensional T1-weighted images, and volume transfer constant (Ktrans) values were calculated at encephalitis-associated brain regions. Ktrans values were compared between the patients and controls, with adjustment for age and sex with use of a nonparametric approach. The Wilcoxon rank sum test was performed to compare Ktrans values of the good (improvement in modified Rankin Scale [mRS] score of at least two points or achievement of an mRS score of ≤2) and poor (improvement in mRS score of less than two points and achievement of an mRS score >2) treatment response groups among the patients. Results Thirty-eight patients with autoimmune encephalitis (median age, 38 years [IQR, 29-59 years]; 20 [53%] female) and 17 controls (median age, 71 years [IQR, 63-77 years]; 12 [71%] female) were included. All brain regions showed higher Ktrans values in patients as compared with controls (P < .001). The median difference in Ktrans between the patients and controls was largest in the right parahippocampal gyrus (25.1 × 10-4 min-1 [95% CI: 17.6, 43.4]). Among patients, the poor treatment response group had higher baseline Ktrans values in both cerebellar cortices (P = .03), the left cerebellar cortex (P = .02), right cerebellar cortex (P = .045), left cerebral cortex (P = .045), and left postcentral gyrus (P = .03) than the good treatment response group. Conclusion DCE MRI demonstrated that BBB permeability was increased in all brain regions in patients with autoimmune encephalitis as compared with controls, and baseline Ktrans values were higher in patients with poor treatment response in the cerebellar cortex, left cerebral cortex, and left postcentral gyrus as compared with the good response group. © RSNA, 2024 Supplemental material is available for this article. See also the editorial by Filippi and Rocca in this issue.

Malignancy risk of indeterminate lymph node at the central compartment in patients with thyroid cancer and concomitant sonographic thyroiditis.

BACKGROUND: To evaluate the malignancy risk of sonographic (US) indeterminate lymph node (LN)s at the central compartment in thyroid cancer patients with US-thyroiditis (ST). METHODS: Among the central compartments of suspicious, indeterminate, and probably benign LN US categories, the malignancy rates were compared between ST and non-US-thyroiditis (non-ST) groups. Those of indeterminate category were compared with suspicious and probably benign categories. RESULTS: At 531 central compartments from 349 patients, the malignancy rate was lower in ST group (34.4% [44/128]) than non-ST group (43.4% [175/403]), although statistically not significant (p = 0.08). The malignancy rate of indeterminate category in ST group (35.7% [5/14]) was lower than non-ST group (71.9% [23/32]) (p = 0.047). Within ST group, the malignancy rate of indeterminate category (35.7% [5/14]) did not differ from probably benign category (29.1% [30/103]) (p = 0.756), but was lower than suspicious category (81.8% [9/11]) (p = 0.042). CONCLUSIONS: The malignancy risk of US indeterminate LNs at the central compartment in thyroid cancer patients with US thyroiditis was lower than that in patients without US thyroiditis.

Outcomes after Biventricular Repair Using a Conduit between the Right Ventricle and Pulmonary Artery in Infancy.

Background: This study investigated the outcomes of biventricular repair using right ventricle to pulmonary artery (RV-PA) conduit placement in patients aged <1 year. Methods: Patients aged <1 year who underwent biventricular repair using an RV-PA conduit between 2011 and 2020 were included in this study. The outcomes of interest were death from any cause, conduit reintervention, and conduit dysfunction (peak velocity of ≥3.5 m/sec or moderate or severe regurgitation). Results: In total, 141 patients were enrolled. The median age at initial conduit implantation was 6 months. The median conduit diameter z-score was 1.3. The overall 5-year survival rate was 89.6%. In the multivariable analysis, younger age (p=0.006) and longer cardiopulmonary bypass time (p=0.001) were risk factors for overall mortality. During follow-up, 61 patients required conduit reintervention, and conduit dysfunction occurred in 68 patients. The 5-year freedom from conduit reintervention and dysfunction rates were 52.9% and 45.9%, respectively. In the multivariable analysis, a smaller conduit z-score (p<0.001) was a shared risk factor for both conduit reintervention and dysfunction. Analysis of variance demonstrated a nonlinear relationship between the conduit z-score and conduit reintervention or dysfunction. The hazard ratio was lowest in patients with a conduit z-score of 1.3 for reintervention and a conduit z-score of 1.4 for dysfunction. Conclusion: RV-PA conduit placement can be safely performed in infants. A significant number of patients required conduit reintervention and had conduit dysfunction. A slightly oversized conduit with a z-score of 1.3 may reduce the risk of conduit reintervention or dysfunction.

Surgical Management of Coronary Artery Fistulas in Children.

Background: This study investigated the surgical outcomes associated with coronary artery fistulas (CAFs) in children. Methods: We retrospectively reviewed the medical records of 23 pediatric patients who underwent surgical closure of CAFs between 1995 and 2021. At presentation, 7 patients (30.4%) exhibited symptoms. Associated cardiac anomalies were present in 8 patients. Fourteen fistulas originated from the right coronary artery and 9 from the left. The most common drainage site was the right ventricle, followed by the right atrium and the left ventricle. The median follow-up duration was 9.3 years (range, 0.1-25.6 years). Results: The median age and body weight at repair were 3.1 years (range, 0-13.4 years) and 14.4 kg (range, 3.1-42.2 kg), respectively. Cardiopulmonary bypass was used in 17 cases (73.9%), while cardioplegic arrest was employed in 14 (60.9%). Epicardial CAF ligation was utilized in 10 patients (43.5%), the transcoronary approach in 9 (39.1%), the endocardial approach in 2 (8.7%), and other methods in 2 patients (8.7%). The application of cardioplegic arrest during repair did not significantly impact the duration of postoperative intensive care unit stay or overall hospital stay. One in-hospital death and 1 late death were recorded. The overall survival rate was 95.7% at 10 years and 83.7% at 15 years. A residual fistula was detected in 1 patient. During the follow-up period, no surviving patient experienced cardiovascular symptoms or coronary events. Conclusion: Surgical repair of CAF can be performed safely with or without cardioplegic arrest, and it is associated with a favorable prognosis in children.

Added value of dynamic contrast-enhanced MR imaging in deep learning-based prediction of local recurrence in grade 4 adult-type diffuse gliomas patients.

Local recurrences in patients with grade 4 adult-type diffuse gliomas mostly occur within residual non-enhancing T2 hyperintensity areas after surgical resection. Unfortunately, it is challenging to distinguish non-enhancing tumors from edema in the non-enhancing T2 hyperintensity areas using conventional MRI alone. Quantitative DCE MRI parameters such as Ktrans and Ve convey permeability information of glioblastomas that cannot be provided by conventional MRI. We used the publicly available nnU-Net to train a deep learning model that incorporated both conventional and DCE MRI to detect the subtle difference in vessel leakiness due to neoangiogenesis between the non-recurrence area and the local recurrence area, which contains a higher proportion of high-grade glioma cells. We found that the addition of Ve doubled the sensitivity while nonsignificantly decreasing the specificity for prediction of local recurrence in glioblastomas, which implies that the combined model may result in fewer missed cases of local recurrence. The deep learning model predictive of local recurrence may enable risk-adapted radiotherapy planning in patients with grade 4 adult-type diffuse gliomas.

Outcomes after Mechanical Aortic Valve Replacement in Children with Congenital Heart Disease.

Background: The optimal choice of valve substitute for aortic valve replacement (AVR) in pediatric patients remains a matter of debate. This study investigated the outcomes following AVR using mechanical prostheses in children. Methods: Forty-four patients younger than 15 years who underwent mechanical AVR from March 1990 through March 2023 were included. The outcomes of interest were death or transplantation, hemorrhagic or thromboembolic events, and reoperation after mechanical AVR. Adverse events included any death, transplant, aortic valve reoperation, and major thromboembolic or hemorrhagic event. Results: The median age and weight at AVR were 139 months and 32 kg, respectively. The median follow-up duration was 56 months. The most commonly used valve size was 21 mm (14 [31.8%]). There were 2 in-hospital deaths, 1 in-hospital transplant, and 1 late death. The overall survival rates at 1 and 10 years post-AVR were 92.9% and 90.0%, respectively. Aortic valve reoperation was required in 4 patients at a median of 70 months post-AVR. No major hemorrhagic or thromboembolic events occurred. The 5- and 10-year adverse event-free survival rates were 81.8% and 72.2%, respectively. In univariable analysis, younger age, longer cardiopulmonary bypass time, and smaller valve size were associated with adverse events. The cut-off values for age and prosthetic valve size to minimize the risk of adverse events were 71 months and 20 mm, respectively. Conclusion: Mechanical AVR could be performed safely in children. Younger age, longer cardiopulmonary bypass time and smaller valve size were associated with adverse events. Thromboembolic or hemorrhagic complications might rarely occur.

Deep learning based on dynamic susceptibility contrast MR imaging for prediction of local progression in adult-type diffuse glioma (grade 4).

Adult-type diffuse glioma (grade 4) has infiltrating nature, and therefore local progression is likely to occur within surrounding non-enhancing T2 hyperintense areas even after gross total resection of contrast-enhancing lesions. Cerebral blood volume (CBV) obtained from dynamic susceptibility contrast perfusion-weighted imaging (DSC-PWI) is a parameter that is well-known to be a surrogate marker of both histologic and angiographic vascularity in tumors. We built two nnU-Net deep learning models for prediction of early local progression in adult-type diffuse glioma (grade 4), one using conventional MRI alone and one using multiparametric MRI, including conventional MRI and DSC-PWI. Local progression areas were annotated in a non-enhancing T2 hyperintense lesion on preoperative T2 FLAIR images, using the follow-up contrast-enhanced (CE) T1-weighted (T1W) images as the reference standard. The sensitivity was doubled with the addition of nCBV (80% vs. 40%, P = 0.02) while the specificity was decreased nonsignificantly (29% vs. 48%, P = 0.39), suggesting that fewer cases of early local progression would be missed with the addition of nCBV. While the diagnostic performance of CBV model is still poor and needs improving, the multiparametric deep learning model, which presumably learned from the subtle difference in vascularity between early local progression and non-progression voxels within perilesional T2 hyperintensity, may facilitate risk-adapted radiotherapy planning in adult-type diffuse glioma (grade 4) patients.

Transitional dendritic cells are distinct from conventional DC2 precursors and mediate proinflammatory antiviral responses.

High-dimensional approaches have revealed heterogeneity amongst dendritic cells (DCs), including a population of transitional DCs (tDCs) in mice and humans. However, the origin and relationship of tDCs to other DC subsets has been unclear. Here we show that tDCs are distinct from other well-characterized DCs and conventional DC precursors (pre-cDCs). We demonstrate that tDCs originate from bone marrow progenitors shared with plasmacytoid DCs (pDCs). In the periphery, tDCs contribute to the pool of ESAM+ type 2 DCs (DC2s), and these DC2s have pDC-related developmental features. Different from pre-cDCs, tDCs have less turnover, capture antigen, respond to stimuli and activate antigen-specific naïve T cells, all characteristics of differentiated DCs. Different from pDCs, viral sensing by tDCs results in IL-1ß secretion and fatal immune pathology in a murine coronavirus model. Our findings suggest that tDCs are a distinct pDC-related subset with a DC2 differentiation potential and unique proinflammatory function during viral infections.

Deep learning-based reconstruction for acceleration of lumbar spine MRI: a prospective comparison with standard MRI.

OBJECTIVE: To compare the image quality and diagnostic performance between standard turbo spin-echo MRI and accelerated MRI with deep learning (DL)-based image reconstruction for degenerative lumbar spine diseases. MATERIALS AND METHODS: Fifty patients who underwent both the standard and accelerated lumbar MRIs at a 1.5-T scanner for degenerative lumbar spine diseases were prospectively enrolled. DL reconstruction algorithm generated coarse (DL_coarse) and fine (DL_fine) images from the accelerated protocol. Image quality was quantitatively assessed in terms of signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) and qualitatively assessed using five-point visual scoring systems. The sensitivity and specificity of four radiologists for the diagnosis of degenerative diseases in both protocols were compared. RESULTS: The accelerated protocol reduced the average MRI acquisition time by 32.3% as compared to the standard protocol. As compared with standard images, DL_coarse and DL_fine showed significantly higher SNRs on T1-weighted images (T1WI; both p < .001) and T2-weighted images (T2WI; p = .002 and p < 0.001), higher CNRs on T1WI (both p < 0.001), and similar CNRs on T2WI (p = .49 and p = .27). The average radiologist assessment of overall image quality for DL_coarse and DL_fine was higher on sagittal T1WI (p = .04 and p < .001) and axial T2WI (p = .006 and p = .01) and similar on sagittal T2WI (p = .90 and p = .91). Both DL_coarse and DL_fine had better image quality of cauda equina and paraspinal muscles on axial T2WI (both p = .04 for cauda equina; p = .008 and p = .002 for paraspinal muscles). Differences in sensitivity and specificity for the detection of central canal stenosis and neural foraminal stenosis between standard and DL-reconstructed images were all statistically nonsignificant (p ≥ 0.05). CONCLUSION: DL-based protocol reduced MRI acquisition time without degrading image quality and diagnostic performance of readers for degenerative lumbar spine diseases. CLINICAL RELEVANCE STATEMENT: The deep learning (DL)-based reconstruction algorithm may be used to further accelerate spine MRI imaging to reduce patient discomfort and increase the cost efficiency of spine MRI imaging. KEY POINTS: • By using deep learning (DL)-based reconstruction algorithm in combination with the accelerated MRI protocol, the average acquisition time was reduced by 32.3% as compared with the standard protocol. • DL-reconstructed images had similar or better quantitative/qualitative overall image quality and similar or better image quality for the delineation of most individual anatomical structures. • The average radiologist's sensitivity and specificity for the detection of major degenerative lumbar spine diseases, including central canal stenosis, neural foraminal stenosis, and disc herniation, on standard and DL-reconstructed images, were similar.

Duplex ultrasound abnormalities of the lower limb veins might precede clinical venous reflux signs in post-Fontan adolescents and young adults.

OBJECTIVE: After the Fontan operation (ie, direct anastomosis of the caval veins to the pulmonary arteries, constituting right ventricular bypass circulation), high central venous pressure can lead to peripheral venous stasis and venous valvar insufficiency. We hypothesized that post-Fontan patients are at a higher risk of developing lower extremity venous lesions detectable using duplex ultrasound, even if clinical signs of chronic venous disease might not be evident. METHODS: A total of 87 transplantation-free survivors after the Fontan procedure who reached adolescence or young adulthood (current age, 15-30 years) participated in a leg vein duplex ultrasound study. The median age at the Fontan procedure, median age at the vein study, and median interval between the two were 3.65 years (interquartile range [IQR], 3.1-5.3 years), 21.7 years (IQR, 18.9-24.7 years), and 16.6 years (IQR, 14.9-19.4 years), respectively. Duplex ultrasound scanning was performed using a venous ultrasound imaging system (Logiq P7; GE Healthcare). The patients were categorized according to the presence of venous reflux (VR) in the superficial, deep, or perforating venous systems: no VR, superficial VR (SVR), deep VR (DVR), perforating VR (PVR), and a combination of multiple venous systems. Correlation of the duplex ultrasound-detected venous lesions with clinical severity using the modified CEAP (clinical, etiological, anatomical, pathophysiological) classification was analyzed using Spearman's correlation analysis. RESULTS: Leg pain was reported by 48 of 87 patients (55.2%). The duplex ultrasound findings for the cohort were no VR in 21 patients (24.1%), SVR in 22 (25.3%), isolated PVR in 21 (24.1%), and reflux of multiple venous systems in 23 patients, including SVR and PVR in 19, DVR and PVR in 1, and SVR, PVR, and DVR in 3 patients. Although the patients with advanced venous lesions detected by duplex ultrasound tended to have a higher CEAP clinical class (P < .001), VR of any venous system on duplex ultrasound was present even in patients with a lower CEAP clinical class. The CEAP clinical class was C0 for 66 patients (76%), and VR of any venous system was present on duplex ultrasound in 66 patients (76%). CONCLUSIONS: The prevalence of lower extremity venous lesions detected by duplex ultrasound is strikingly high in post-Fontan adolescents and young adults, and duplex ultrasound-detected venous abnormalities can precede clinical manifestations. Early detection and timely intervention for leg vein problems are mandatory for post-Fontan patients, especially for those considered to have risk factors for developing chronic lower extremity venous disease.

Extracorporeal Cardiopulmonary Resuscitation in Infants: Outcomes and Predictors of Mortality.

Background: Extracorporeal cardiopulmonary resuscitation (E-CPR) plays an indispensable role when resuscitation fails; however, extracorporeal life support (ECLS) in infants is different from that in adults. The objective of this study was to evaluate the outcomes of E-CPR in infants. Methods: A single-center retrospective study was conducted, analyzing 51 consecutive patients (age <1 year) who received E-CPR for in-hospital cardiac arrest between 2010 and 2021. Results: The median age and body weight was 51 days (interquartile range [IQR], 17-111 days) and 3.4 kg (IQR, 2.9-5.1 kg), respectively. The cause of arrest was cardiogenic in 45 patients (88.2%), and 48 patients (94.1%) had congenital cardiac anomalies. The median conventional cardiopulmonary resuscitation (C-CPR) time before the initiation of ECLS was 77 minutes (IQR, 61-103 minutes) and duration of ECLS was 7 days (IQR, 3-12 days). There were 36 in-hospital deaths (70.6%), and another patient survived after heart transplantation. In the multivariate analysis, single-ventricular physiology (odds ratio [OR], 5.05; p=0.048), open sternum status (OR, 8.69; p=0.013), and C-CPR time (OR, 1.47 per 10 minutes; p=0.021) were significant predictors of in-hospital mortality. In a receiver operating characteristic curve, the optimal cut-off of C-CPR time was 70.5 minutes. The subgroup with early E-CPR (C-CPR time <70.5 minutes) showed a tendency for lower in-hospital mortality tendency (54.5% vs. 82.8%, p=0.060), albeit not statistically significant. Conclusion: If resuscitation fails in an infant, E-CPR could be a life-saving option. It is crucial to improve C-CPR quality and shorten the time before ECLS initiation.

Characteristics of Patients with Surgical Closure of an Atrial Septal Defect during Infancy.

Background: Surgical closure of an atrial septal defect (ASD) is infrequently indicated during infancy. We evaluated the clinical characteristics and outcomes of patients who underwent surgical ASD closure during infancy. Methods: A single-center retrospective review was performed for 39 patients (19 males) who underwent surgical ASD closure during infancy between 1993 and 2020. The median body weight percentile at the time of operation was 9.3. Results: During a median follow-up of 60.9 months, 4 late deaths occurred due to chronic respiratory failure. A preoperative history of bronchopulmonary dysplasia (BPD) was the only risk factor for late mortality identified in Cox regression (hazard ratio, 3.54; 95% confidence interval [CI], 1.75-163.04; p=0.015). The 5-year survival rate was significantly lower in patients with preoperative history of BPD (97.0% vs. 50.0%, p<0.001) and preoperative ventilatory support (97.1% vs. 40.4%, p<0.001). There were significant postoperative increases in left ventricular end-diastolic (p=0.017), end-systolic (p=0.014), and stroke volume (p=0.013) indices. A generalized estimated equation model showed significantly better postoperative improvement in body weight percentiles in patients with lower weight percentiles at the time of operation (<10th percentile, p=0.01) and larger indexed ASD diameter (≥45 mm/m2, p=0.025). Conclusion: Patients with ASD necessitating surgical closure during infancy are extremely small preoperatively and remain small even after surgical closure. However, postoperative somatic growth was more prominent in smaller patients with larger defects, which may be attributable to an increase in postoperative cardiac output due to changes in ventricular septal configuration. The benefits of ASD closure in patients with BPD are undetermined.

Deep learning based automatic detection algorithm for acute intracranial haemorrhage: a pivotal randomized clinical trial.

Acute intracranial haemorrhage (AIH) is a potentially life-threatening emergency that requires prompt and accurate assessment and management. This study aims to develop and validate an artificial intelligence (AI) algorithm for diagnosing AIH using brain-computed tomography (CT) images. A retrospective, multi-reader, pivotal, crossover, randomised study was performed to validate the performance of an AI algorithm was trained using 104,666 slices from 3010 patients. Brain CT images (12,663 slices from 296 patients) were evaluated by nine reviewers belonging to one of the three subgroups (non-radiologist physicians, n = 3; board-certified radiologists, n = 3; and neuroradiologists, n = 3) with and without the aid of our AI algorithm. Sensitivity, specificity, and accuracy were compared between AI-unassisted and AI-assisted interpretations using the chi-square test. Brain CT interpretation with AI assistance results in significantly higher diagnostic accuracy than that without AI assistance (0.9703 vs. 0.9471, p < 0.0001, patient-wise). Among the three subgroups of reviewers, non-radiologist physicians demonstrate the greatest improvement in diagnostic accuracy for brain CT interpretation with AI assistance compared to that without AI assistance. For board-certified radiologists, the diagnostic accuracy for brain CT interpretation is significantly higher with AI assistance than without AI assistance. For neuroradiologists, although brain CT interpretation with AI assistance results in a trend for higher diagnostic accuracy compared to that without AI assistance, the difference does not reach statistical significance. For the detection of AIH, brain CT interpretation with AI assistance results in better diagnostic performance than that without AI assistance, with the most significant improvement observed for non-radiologist physicians.

Quantifying infarct core volume in ischemic stroke: What is the optimal threshold and parameters of computed tomography perfusion?

OBJECTIVE: Although computed tomography perfusion (CTP) is used to select and guide decision-making processes in patients with acute ischemic stroke, there is no clear standardization of the optimal threshold to predict ischemic core volume accurately. The infarct core volume with a relative cerebral blood flow(rCBF) threshold of < 30% is commonly used. We aimed to assess the volumetric agreement of the infarct core volume with different CTP parameters and thresholds using CTP software (RAPID, VITREA) and the infarct volume on diffusion-weighted imaging (DWI), with a short interval time (within 60 min) between CTP and follow-up DWI. MATERIALS AND METHODS: This retrospective study included 42 acute ischemic stroke patients with occlusion of the large artery in the anterior circulation between April 2017-November 2020. RAPID identified infarct core as tissue rCBF < 20-38%. VITREA defined the infarct core as cerebral blood volume (CBV) < 26-56%. Olea Sphere was used to measure infarct core volume on DWI. The CTP-infarct core volume with different thresholds of perfusion parameters (CBF threshold vs CBV threshold) were compared with DWI-infarct core volumes. RESULTS: The median time between CTP and DWI was 37.5min. The commonly used threshold of CBV< 41% (4.3 mL) resulted in lower median infarct core volume difference compared to the commonly used thresholds of rCBF < 30% (8.2mL). On the other hand, the optimal thresholds of CBV < 26% (-1.0mL; 95% CI, -53.9 to 58.1 mL; 0.945) resulted in the lowest median infarct core volume difference, narrowest limits of agreement, and largest interclass correlation coefficient compared with the optimal thresholds of rCBF < 38% (4.9 mL; 95% CI, -36.4 to 62.9 mL; 0.939). CONCLUSION: Our study found that the both optimal and commonly used thresholds of CBV provided a more accurate prediction of the infarct core volume in patients with AIS than rCBF.

Outcomes of Arterial Switch Operation With Aortic Arch Reconstruction.

BACKGROUND: This study investigated the outcomes and factors associated with reintervention or development of significant pulmonary stenosis (PS) after repair of transposition of the great arteries (TGA) or Taussig-Bing anomaly (TBA) with aortic arch obstruction. METHODS: A total of 51 patients with TGA or TBA who underwent an arterial switch operation and aortic arch reconstruction between 2004 and 2020 were included. The outcomes of interest were all-cause death, including heart transplantation, all-cause reintervention, right-sided reintervention, and development of significant PS. RESULTS: The median age and body weight at repair were 9 days and 3.2 kg, respectively. Forty-nine patients (96.1%) underwent 1-stage repair. A total of 28 patients (54.9%) had TBA, and 8 patients (15.7%) had interrupted aortic arch. There were 5 early deaths (9.8%) and 2 late deaths during a median follow-up duration of 59 months. The transplant-free survival rate 10 years after repair was 82.6%. A total of 21 reinterventions were required in 10 patients. The significant PS-free survival rate 10 years after repair was 68.8%. In univariable analysis, a higher ratio of the diameter of the main pulmonary artery to the ascending aorta was associated with all-cause reintervention (P = .007) and right-sided reintervention (P = .002). A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS (P = .049). CONCLUSIONS: The rates of overall mortality and reintervention after repair were not negligible. A higher degree of size discrepancy between the 2 great arteries was associated with all-cause or right-sided reintervention. A smaller aortic annulus z-score at the pulmonary position was associated with the development of significant PS.

Outcomes of Surgical Repair for Truncus Arteriosus: A 30-Year Single-Center Experience.

Background: We investigated the long-term outcomes of truncus arteriosus repair at a single institution with a 30-year study period. Methods: Patients who underwent repair of truncus arteriosus between 1993 and 2022 were reviewed retrospectively. Factors associated with early mortality, overall attrition, and reintervention were identified using appropriate statistical methods. Results: In total, 42 patients were enrolled in this study. The median age and weight at repair were 26 days and 3.5 kg, respectively. Thirty patients (71.4%) underwent 1-stage repair. There were 8 early deaths (19%). In the univariable analysis, undergoing surgery before 2011 was associated with early mortality (p=0.031). The overall survival rate at 10 years was 73.8%. In the multivariable analysis, significant truncal valve (TrV) dysfunction (p=0.010), longer cardiopulmonary bypass time (p=0.018), and the earlier era of surgery (p=0.004) were identified as risk factors for overall mortality. During follow-up, 47 reinterventions were required in 27 patients (64.3%). The freedom from all-cause reintervention rate at 10 years was 23.6%. In the multivariable analysis, associated arch obstruction (p<0.001) and significant TrV dysfunction (p=0.011) were identified as risk factors for all-cause reintervention. Arch obstruction (p=0.027) and a number of TrV cusps other than 3 (p=0.014) were identified as risk factors for right ventricle to pulmonary artery (RV-PA) reintervention, and significant TrV dysfunction was identified as a risk factor for TrV reintervention (p=0.002). Conclusion: Despite recent improvements in survival outcomes after repair of truncus arteriosus, RV-PA or TrV reinterventions were required in a significant number of patients during follow-up.

Outcomes of pulmonary artery sling repair without tracheoplasty.

OBJECTIVE: This study aimed to investigate surgical outcomes of pulmonary artery (PA) sling without tracheoplasty. METHODS: From 2001 through 2020, among 22 patients who underwent PA sling repair, all but 1 patient who underwent concomitant tracheal surgery were analyzed. The outcomes of interest were all-cause death, PA reintervention, tracheal intervention, and readmission for respiratory symptoms. Computed tomography was used to measure the narrowest tracheal diameter. RESULTS: The median age and weight at repair were 7.6 months and 7.7 kg, respectively. Most patients (20 out of 21, 95.2%) had preoperative respiratory symptoms. Associated airway anomalies included tracheal ring in 12 (57.1%), bridging bronchus in 8 (38.1%), and tracheal bronchus in 2 patients (9.5%). There was 1 in-hospital death (4.8%). The median ventilator time and intensive care unit stay were 23 hours and 3 days, respectively. There was neither late death nor tracheal intervention during follow-up. Five patients (25.0%) underwent reintervention for left PA stenosis. Hospital readmission for respiratory symptom was required in 7 patients and was associated with the narrowest preoperative tracheal diameter (P = .025) and cardiopulmonary bypass time (P = .040) in univariable analysis. The narrowest tracheal diameter of 3.4 mm was identified as a cutoff value for readmission for respiratory symptom. Freedom from readmission for respiratory symptom was 63.3% at 10 years. CONCLUSIONS: PA sling repair without tracheal surgery might be a reasonable surgical option with rare need for tracheal intervention. Hospital readmissions for respiratory symptoms are more frequently required in patients with smaller tracheal diameter and all readmissions were limited to within 2 years after repair.

Sialylated IVIg binding to DC-SIGN+ Hofbauer cells induces immune tolerance through the caveolin-1/NF-kB pathway and IL-10 secretion.

Although the use of IVIg has increased in various immune-driven diseases and even in pregnancy, the exact action mechanisms of IVIg are not fully understood. Dendritic cell-specific intercellular adhesion molecule-3 grabbing non-integrin (DC-SIGN) is a known receptor for α-2,6-sialylated IgG (sIVIg), which is responsible for the anti-inflammatory effect of IVIg. DC-SIGN is expressed on Hofbauer cells (HBCs) of the fetal villi of the placenta which act as an innate immune modulator at the maternal-fetal interface. Preeclampsia is a major complication in pregnancy and is related to IL-10, a cytokine with an important role in immune tolerance. DC-SIGN interaction with sIVIg in HBCs promoted IL-10 secretion through the activation of the caveolin-1/NF-κB pathway, especially in plasma lipid rafts. Consistent results were obtained for HBCs from patients with preeclampsia. Collectively, the stimulation of DC-SIGN+ HBCs with sIVIg enhanced immune tolerance in the feto-maternal environment, suggesting the therapeutic application of sIVIg to prevent preeclampsia.

The Usefulness of Computed Tomography in Predicting Left Ventricular Outflow Tract Obstruction After Neonatal Arch Repair.

This study investigated the outcome after neonatal arch repair, and the usefulness of computed tomography (CT) in predicting the development of left ventricular outflow tract (LVOT) obstruction (LVOTO). A total of 150 neonates who underwent arch repair between 2008 and 2019 were included. The diameters of the aortic valve annulus (AVA) and LVOT in millimeters were measured with transthoracic echocardiography (TTE) or CT and indexed by subtracting body weight in kilograms. The outcomes of interest were the development of LVOTO (peak flow velocity > 3 m/s on TTE) and reintervention or reoperation for LVOTO. The median follow-up duration was 3.6 years. The rates of overall survival, freedom from reintervention for LVOTO, and freedom from the LVOTO development at 7 years were 93.7%, 88.2%, and 81.0%, respectively. In univariable Cox regression analysis, weight-indexed CT-measured LVOT diameter (concordance index [C-index] = 0.73, P = 0.002) and weight-indexed TTE-measured AVA diameter (C-index = 0.69, P = 0.001) were significant predictors of LVOTO. The maximal chi-square test identified the following cutoff values for predicting LVOTO: 1.4 for weight-indexed CT-measured LVOT diameter and 1.6 for weight-indexed TTE-measured AVA diameter. The high-risk group (both measures lower than the cutoff values) had a significantly lower rate of freedom from LVOTO development than the low-risk group (both measures higher than the cutoff values) (P < 0.001). The weight-indexed CT-measured LVOT diameter could be used to predict LVOTO development after neonatal arch repair, as an independent measure or complementary to traditional measures.