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Single ventricle (SV) patients undergo multiple surgeries with subsequent changes in anatomy and hemodynamics. There are little cardiac magnetic resonance (CMR) data on serial changes in these patients. This study aimed to assess longitudinal changes of SV anatomy and hemodynamics in a large cohort. Anatomy and flow in SV patients with serial CMRs performed between 2008 and 2019 at a single institution were retrospectively reviewed. Mixed-effects linear regression was used to estimate changes over time at 3 to 9 months, 1 to 5 years, and >5 years after Fontan. A total of 119 patients were included (51% with hypoplastic left heart syndrome; 77% underwent extracardiac Fontan). A total of 88 patients had 3 serial CMRs. Indexed right superior vena cava, inferior vena cava, neoaortic valve, and descending aorta area decreased over time (beta = -0.19, -0.44, and -0.23, respectively; P <.01), as did indexed right superior vena cava, neoaorta and native aorta, and descending aorta flow (beta = -0.49, -0.53, and -0.59, respectively; P <.0001). Inferior vena cava flow and its contribution to total caval flow increased (beta = 0.33; P <.0001). Indexed right and left pulmonary artery flow did not change; however, indexed left pulmonary artery area decreased (beta = -0.16; P =.0014) with time. Systemic-to-pulmonary collateral flow remained unchanged before and early after Fontan (beta = -0.54; P =.42) but decreased with time from Fontan (beta = -0.22; P <.0001). In this cohort of longitudinally followed SV patients, there are significant trends in vascular size and flow over time from Fontan. These findings can be used as a framework to interpret serial CMR data in the SV and noninvasively identify deviations from expected patterns before the development of clinical symptoms. [Display omitted] [ABSTRACT FROM AUTHOR]

After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria. The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy. [Display omitted] [ABSTRACT FROM AUTHOR]

Lymphatic imaging plays a crucial role in novel lymphatic interventions, offering valuable insights into central lymphatic drainage. Lymphatic system abnormalities may appear in various pediatric disorders, and accurate imaging is crucial for effective diagnosis and tailored therapeutic interventions. Traditional imaging modalities have offered valuable insights, but the demand for non-invasive, high-resolution techniques has fueled the development of innovative lymphatic imaging methods. In this review, we explore the state of the art in lymphatic imaging specifically within the context of pediatric surgery. [ABSTRACT FROM AUTHOR]

Pulmonary lymphatic flow disorders involve the abnormal lymphatic flow via lymphatic channels to the lungs and pleural space. Plastic bronchitis and chylothorax are the main complications of this abnormal lymphatic perfusion, which has been termed pulmonary lymphatic perfusion syndrome (PLPS). Following lymphatic access, dynamic contrast MR lymphangiography is the imaging modality of choice to diagnose these disorders. Management includes medical therapy, percutaneous interventions under fluoroscopy, and surgical interventions. [ABSTRACT FROM AUTHOR]

The Fontan operation or the total cavopulmonary connection is a palliative surgery for single ventricle congenital heart disease where the systemic venous return circumvents a pumping chamber and flows directly into the pulmonary circuit. With surgical and medical advances, there has been improvement in life expectancy of these patients, however, it has also resulted in unique complications from the physiology that requires diligent surveillance. A critical component relies on optimal imaging for diagnosis and treatment of these complications. This article describes the normal anatomy of the Fontan circulation, current imaging modalities and techniques, and frequently encountered complications seen when imaging the patients who have undergone Fontan palliation. [ABSTRACT FROM AUTHOR]

Dual-energy computer tomography (DECT) technology has experienced rapid growth in recent years, now allowing for the collection of 2 CT data sets and opening the potential for functional data acquisition. Data from a single postcontrast phase are deconstructed and Iodine can be subtracted to create a virtual noncontrast image, or selectively represented as a contrast map that allows for the qualification and quantification of lung perfusion. Virtual monoenergetic images can also be used to reduce beam-hardening artifact from concentrated contrast or metal implants. In children, DECT is of particular interest because it has been shown to be dose neutral in most applications, dose-reducing in multiphase studies, and to increase the contrast to noise ratio in suboptimal studies. We review the basics of acquisition, postprocessing, and thoracic applications of DECT with a focus on pulmonary blood volumes as a surrogate for perfusion imaging. The discussed applications include pulmonary embolism, hypoplastic lung, pulmonary hypertension in bronchopulmonary dysplasia, and pediatric lung masses. [ABSTRACT FROM AUTHOR]

The aim of this study was to determine the feasibility of using contrast-enhanced ultrasound (CEUS) evaluation to determine thoracic duct (TD) outlet patency. Nine patients referred for lymphatic imaging and intervention underwent percutaneous intranodal ultrasound contrast injection and conventional lymphangiography (CL). Eight of 9 patients had a patent TD by CEUS and CL. One patient did not have a patent TD. There was 100% agreement between CEUS and CL. These results suggest that CEUS is an imaging modality that might be as accurate as CL in determining TD patency. [ABSTRACT FROM AUTHOR]

Purpose: Evaluate the feasibility and determinants of image quality of ECG-triggered High-Pitch Dual-Source Computed Tomography Angiography (CTA) for cardiovascular assessment in Children.Material and Methods: All children that underwent ECG-triggered High-Pitch Dual-Source CTA between August 2014 and September 2017 were identified. Scanner parameters and patients' information were retrieved. Objective image quality was evaluated measuring the Hounsfield units (HU) and standard deviation of regions of interests in the left ventricle, ascending and descending aorta. Signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were calculated. Qualitative image quality was recorded independently by two pediatric radiologist blinded using a three-point scale: 1 - good image quality, 2 - mild artifacts, 3 - poor image quality. Continuous variables were presented as mean ± standard deviation. The interobserver agreement and non-parametric test were used.Results: 93 patients (mean age 5.6 ± 7.1 years) were selected. Average cardiovascular attenuation, SNR and CNR were 406.2 ± 146.3 HU, 24.2 ± 16.0 HU and 52.1 ± 38.6 HU, respectively. Average image quality was 1.51 ± 0.48 and the inter-observer agreement was excellent (k = 0.8). Worse subjective quality scores were associated with lower age, height, weight, BSA, lower contrast dose and slower injection rates (p < 0.05). Higher heart rate was associated with high attenuation (p < 0.05), however, SNR and CNR did not show an association with heart rate (p = 0.80).Conclusions: ECG-triggered High-Pitch Dual-Source cardiac CTA is feasible and provides good or excellent image quality for the evaluation of cardiovascular diseases in children. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the safety of power injectors for contrast-enhanced computed tomography (CT) in children, namely: the prevalence and injury severity of contrast extravasations related to power injectors and the factors associated with these events.Methods: The need to obtain informed consent was waived for this HIPAA-compliant and IRB approved retrospective study. Around 2429 contrast-enhanced CT performed with a power injector were identified during a 3-year period. Data collected included patient demographic, power injector, and contrast agent information. The patients' symptoms, severity of injury and treatment with contrast extravasation were recorded. Around 1496 cases (823 boys, 673 girls) were included in the analysis. Independent-sample t test and Chi-square were used. For a sub-analysis using the extravasation cases, nonparametric tests were used.Results: The mean age was 9.5 ± 6.1 years. The most common access site, catheter site, and contrast agent used were the antecubital fossa, 22 gauge and Iohexol. The mean peak pressure was 68.9 ± 62.3 psi and the flow rate was 1.7 ± 0.9 mL/s. Eighteen cases of contrast extravasation were identified with a mean age of 11.2 ± 6.2 years. There were seven mild, six moderate, and five severe. Cases with extravasation had significantly higher peak pressure (p < 0.001) and flow rate (p < 0.001) compared to those without extravasation. Patients who received Iohexol-350 had significantly more contrast extravasation compared to those who used Iohexol-300 (p = 0.03). However, after post-hoc correction, only peak pressure (p < 0.01) and flow rate (p = 0.01) remained significant.Conclusion: The use of power injectors in children undergoing contrast-enhanced CT is associated with a low rate of extravasation and of long-term injury. [ABSTRACT FROM AUTHOR]

Patients with single ventricle (SV) may often undergo aortic reconstruction that creates a stiff large vessel, increasing afterload and affecting exercise performance. The objective of this study was to determine the relationship of pulse wave velocity (PWV) and distensibility in reconstructed and normal aortic arches after Fontan with exercise variables. PWV and distensibility of the descending aorta at the level of the diaphragm (DAo) were calculated with real-time exercise cardiac magnetic resonance in 48 patients with SV after Fontan (18 after aortic reconstruction; 30 without aortic reconstruction) and compared with metabolic exercise stress test variables. PWV was greater in the reconstructed group than in the non-reconstructed group (median 4.4 m/s [range: 2.3 to 9.8 m/s] versus 3.6 [range: 2.6 to 6.3 m/s], respectively, p = 0.003). Statistically significant inverse correlations were found between PWV and end-diastolic, end-systolic, and stroke volumes at rest and at exercise in the reconstructed group. In addition, inverse correlations also existed in the reconstructed group between distensibility of the DAo and the exercise variables such as peak oxygen pulse (R = 0.56, p = 0.02), peak oxygen consumption (R = 0.63, p = 0.008), oxygen consumption at ventilatory anaerobic threshold (R = 0.48, p = 0.04), and peak work (R = 0.54, p = 0.02). Similar correlations were not seen in patients with non-reconstructed aortas. Patients with SV with reconstructed aortas have increased aortic stiffness, increasing afterload on the ventricle. Native DAo stiffness distal to the reconstruction is inversely correlated with exercise performance, presumably to decrease impedance mismatch to maintain homogeneity of the aortic wall. This information suggests a possible mechanism for decreased exercise performance in patients with SV with aortic reconstructions. [ABSTRACT FROM AUTHOR]

Rationale and Objectives: This study aimed to describe prenatal and postnatal imaging features and outcomes of neonates with neonatal lymphatic disorders (NLDs).Materials and Methods: An institutional review board-approved search of the radiology database for patients with NLD identified five patients. Inclusion criteria include prenatal imaging (fetal magnetic resonance [MR] imaging and ultrasound) and postnatal three-dimensional T2 Sampling Perfection with Application optimized Contrasts using different flip angle Evolution (SPACE) and dynamic contrast-enhanced MR lymphangiography within 6 months of life. Chart review was undertaken to evaluate morbidity and mortality.Results: Prenatal finding of "nutmeg lung" or fetal pulmonary lymphatic disorder was identified in all five patients on fetal MR imaging, and in four of five patients on fetal ultrasound. Postnatal dynamic contrast-enhanced MR lymphangiography demonstrated abnormal lymphatic flow to the lungs in four of five patients, but absent in the single patient with coexisting hypoplastic left heart syndrome (HLHS). Dermal backflow was seen in one patient, also the only patient with prenatal body wall edema. Three patients with lymphatic flow to the lungs only were classified as neonatal chylothorax. The patient with dermal backflow and perfusion to the lungs was diagnosed with central lymphatic flow disorder (CLFD). The HLHS patient with normal lymphatic perfusion maintained the HLHS diagnosis. Of the five patients, the patient with CLFD and the one with HLHS expired because of respiratory distress.Conclusions: NLDs can be recognized on prenatal and postnatal imaging and may be primary, as in neonatal chylothorax or CLFD, or secondary. In this small series, "nutmeg lung" was present in all patients. Prenatal imaging demonstrates that body wall edema may correlate with postnatal dermal backflow, which, in our small cohort, carried a poor prognosis. [ABSTRACT FROM AUTHOR]

Objectives Identify MRE features of colonic strictures in children/young adults with Crohn's disease. Methods Included are patients with colonic strictures on colonoscopy imaged with MRE. Bowel wall thickening (BWT) with luminal narrowing, pre-stenotic dilatation (PSD), bowel wall enhancement, and diffusion restriction were evaluated. Results All potential strictures (n = 12) had BWT with luminal narrowing on MRE. 8/12 had PSD, meeting our MRE stricture criteria. MRE diagnosed 8/12 colonic strictures compared to colonoscopy but was concordant with findings in all surgical patients. Conclusion Strictures on MRE did not always agree with colonoscopy, but when correlating with surgery, MRE findings were all concordant. [ABSTRACT FROM AUTHOR]

Purpose: To assess the feasibility of validating the position of the needle in groin lymph nodes using injection of ultrasound (US) contrast prior to magnetic resonance (MR) lymphangiography.Materials and Methods: In 28 patients (average age, 52 y; 15 women, 13 men), 25G needles were placed in bilateral groin lymph nodes using US guidance outside the MR suite. The confirmation of the position of the needles in lymph nodes and efferent lymphatic flow was performed by injecting 1 mL of the US contrast agent (Lumason) mixed with 2 mL of lidocaine and observing contrast enhancement of the efferent lymphatic ducts leading to the pelvis from the lymph node. The patients were then transferred to the MR suite, and MR lymphangiography was performed.Results: In 6 patients, the needle was repositioned due to nonvisualization of the efferent lymphatic flow toward pelvis at the first attempt. MR lymphangiogram was then performed in all patients with good opacification of the central lymphatic system. No extravasation of gadolinium contrast agent was observed.Conclusions: Injection of US contrast to confirm the position of the needles inside the lymph nodes is a safe and effective technique. This technique can serve as a substitute for fluoroscopic confirmation of needle position, allowing performance of MR lymphangiography on any MR machine with a detachable table. [ABSTRACT FROM AUTHOR]

Abstract: Background/purpose: Obstructive symptoms in a patient post-Ladd procedure raise the question of recurrent volvulus. Our objective is to determine the incidence and the radiographical evaluation of recurrent volvulus and abdominal complications after a Ladd procedure. Methods: One hundred ninety-five patients who underwent a Ladd procedure for malrotation for 10 years were retrieved from a database. Forty-eight patients were excluded (false-positive studies, heterotaxy, diaphragmatic hernia, abdominal wall defects). Of the remaining 147, 38 patients presented with abdominal symptoms. Surgical records and imaging were reviewed. Results: Of 38 patients, 33 had imaging studies including abdominal radiographs (AXR) and/or upper gastrointestinal examinations. Of these 33 patients, 17 had normal or expected imaging findings on AXR and/or upper gastrointestinal. Of the 11 patients who had surgery, 8 had an AXR. Of these, only a single patient had a normal AXR. The most common post-Ladd complication found at reoperation was small bowel obstruction secondary to adhesions (5.4%). One patient (0.7%) had midgut volvulus. Conclusion: Although recurrent volvulus is a feared postoperative Ladd complication, it rarely occurs. Adhesive small bowel obstruction is more common, and an AXR with clinical findings is sufficient for diagnosis. [Copyright &y& Elsevier]

We sought to evaluate contractile function in single-ventricle patients before and after imposition of Fontan physiology. Single right ventricle (SRV; n = 38) and single left ventricle (SLV; n = 11) patients underwent cardiac magnetic resonance imaging pre and post Fontan operation. Global radial strain (GRS), global circumferential strain (GCS), and global longitudinal strain were measured along with ejection fraction (EF) and atrioventricular valve regurgitation (AVVR). Age at cardiac magnetic resonance imaging before the Fontan operation was 3.1 ± 1.3 years and after the Fontan procedure was 5.8 ± 2.7 years. There were no significant EF differences between SRV and SLV patients before and after the Fontan procedure, and EF did not deteriorate significantly after the Fontan operation. GRS was significantly lower for SRV patients than for SLV patients before (24.3% vs 32.1%; P =.048) and after (21.8% vs 29.7%; P =.045) the Fontan procedure. GRS and GCS of the SRV patients deteriorated significantly after the Fontan operation (GRS, P =.01; GCS, P =.009). Strains showed positive correlations before and after the Fontan operation with positive correlations among each strain. Within all patients, strains correlated positively with EF. Strains and EF negatively correlated with AVVR (GRS P =.03, r = −0.22; GCS P =.03, r = −0.23; EF P <.001, r = −0.37). Strains were lower for SRV than for SLV patients before and after the Fontan operation and deteriorated after the Fontan operation. Our study suggests that strain measures might detect ventricular deterioration earlier than EF. Because strains before and after the Fontan operation were positively correlated, and negatively correlated with AVVR, the early institution of myocardial protective therapy including AVVR management, especially for SRV patients, might have benefit. [ABSTRACT FROM AUTHOR]

Magnetic resonance imaging (MRI) is a routinely used imaging modality for the diagnosis and treatment planning of many health conditions in children and adults. Yet, its use has been limited in many institutions for patients with cardiac implantable electronic devices (CIEDs) due to safety concerns. Current evidence relates primarily to devices with transvenous leads. However, patients with complex cardiac anatomy and palliative surgery procedures often require epicardial pacemakers. To date, very few studies have addressed MRI safety considerations with non-conditional CIEDs or abandoned epicardial leads in infants, and to our knowledge, this is the first report that shows Fontan palliation patients who underwent Dynamic Contrast enhanced MR Lymphangiography (DCMRL) with these types of devices. We present our institutional experience with five cases where a DCMRL was safely performed in three children and two adults with Fontan palliation to evaluate their lymphatic anatomy and guide interventional procedures. Regarding our brief experience, we concluded that DCMRL may be considered in post-Fontan patients with non-conditional CIEDs, including epicardial leads, seeking the best diagnostic and treatment options available. Institutional protocols must be revised in advance to perform this technique in a controlled setting. • Patients with MR non-conditional cardiac implantable electronic devices (CIEDs) are still restricted from the use of MRI due to safety concerns. • Fontan patients have a complex anatomy that usually comes with abnormal lymphatic drainage. • The recent utilization of Dynamic Contrast enhanced MR Lymphangiography (DCMRL) in Fontan patients has improved their prognosis by allowing the visualization and treatment of their lymphatic leakages. • DCMRL may be considered in post-Fontan patients with non-conditional CIEDs, including epicardial leads, seeking the best diagnostic and treatment options available. [ABSTRACT FROM AUTHOR]

To characterize the normal progression of quantitative CT parameters in normal children from birth to adulthood. Patients aged 0–18 years with non-contrast-enhanced chest CT and evidence of normal lung parenchyma were included. Patients with respiratory symptoms, incomplete anthropometric measurements, or sub-optimal imaging technique were excluded. Segmentation was performed using an open-source software with an automated threshold segmentation. The following parameters were obtained: mean lung density, kurtosis, skewness, lung volume, and mass. Linear and exponential regression models were calculated with age and height as independent variables. A p -value of <0.05 was considered significant. 220 patients (111 females, 109 males) were included. Mean age was 9.6 ± 5.9 years and mean height was 133.9 ± 35.1 cm. Simple linear regression showed a significant relationship between mean lung density with age (R 2 = 0.70) and height (R 2 = 0.73). Kurtosis displayed a significant exponential correlation with age (R 2 = 0.70) and height (R 2 = 0.71). Skewness showed a significant exponential correlation with age (R 2 = 0.71) and height (R 2 = 0.73). Lung mass showed a correlation with age (R 2 = 0.93) and height (R 2 = 0.92). Exponential regression showed a significant relationship between lung volume with age (R 2 = 0.88) and height (R 2 = 0.93). Quantitative CT parameters of the lung parenchyma demonstrate changes from birth to adulthood. As children grow, the mean lung density decreases, and the lung parenchyma becomes more homogenous. • Age, sex, and height are predictors of quantitative CT parameters in children • Lung density decreases linearly, but lung volume and mass increase exponentially • As children grow, density histogram shows a right-skewed distribution [ABSTRACT FROM AUTHOR]

To determine the optimal MRI protocol and sequences for liver and cardiac iron estimation in children. We evaluated patients ≤18 years with cardiac and liver MRIs for iron content estimation. Liver T2 was determined by a third-party company. Cardiac and Liver T2* values were measured by an observer. Liver T2* values were calculated using the available liver parenchyma in the cardiac MRI. Linear correlations and Bland-Altman plots were run between liver T2 and T2*, cardiac T2* values; and liver T2* on dedicated cardiac and liver MRIs. 139 patients were included. Mean liver T2 and T2* values were 8.6 ± 5.4 ms and 4.5 ± 4.1 ms, respectively. A strong correlation between liver T2 and T2* values was observed (r = 0.96, p < 0.001) with a bias (+4.1 ms). Mean cardiac bright- and dark-blood T2* values were 26.5 ± 12.9 ms and 27.2 ± 11.9 ms, respectively. Cardiac T2* values showed a strong correlation (r = 0.81, p < 0.001) with a low bias (−1.0 ms). The mean liver T2* on liver and cardiac MRIs were 4.9 ± 4.7 ms and 4.6 ± 3.9 ms, respectively. A strong correlation between T2* values was observed (r = 0.96, p < 0.001) with a small bias (−0.2 ms). MRI protocols for iron concentration in the liver and the heart can be simplified to avoid redundant information and reduce scan time. In most patients, a single breath-hold GRE sequence can be used to evaluate the iron concentration in both the liver and heart. • Liver T2 and T2* have excellent agreement and consistency. • Bright- and dark-blood T2* sequences yield similar values. The later improves visualization of the myocardium and analysis. • Liver T2* estimation using the liver parenchyma available in the cardiac acquisition is feasible and accurate • The decision to use a single sequence for cardiac and liver T2* estimation should be taken according to each patient • A single breath hold GRE sequence for liver and cardiac T2* requires optimization for patients with severe iron overload [ABSTRACT FROM AUTHOR]

Abstract Objective Assess the image quality of ECG-triggered High-Pitch Dual-Source CTA for the evaluation of native coronaries in children. Materials and methods Between August 2014 and September 2017, 45 children with morphologically normal cardiac chambers had cardiac prospective ECG-triggered High-Pitch Dual-Source CTA. Two pediatric radiologists blinded to clinical data, independently reviewed each case. The coronary arteries were evaluated using a four-point scale quality score according to the coronary segment. Attenuation, signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) were measured using values from the air, inter-ventricular septum and ascending aorta at the level of the sinuses of Valsalva. Results 225 coronary segments were assessed showed a mean score of 2.40 ± 0.73, 94.2% had diagnostic image quality. The best and worst average quality were seen in segment 5 and 2, respectively. Inter-observer agreement was moderate for all segments except for segment 1, which was excellent. Worse quality scores were significantly associated with younger patients and low body mass index as well as with higher heart rates in all segments. The mean observed heart rate and BSA in patients with diagnostic image quality were below 77 bpm and over 1.4 m2 respectively. There is no significant association between attenuation, SNR and CNR with image quality. Conclusions Prospective ECG-triggered High-Pitch Dual-Source Computed Tomography Angiography achieves consistent and diagnostic image quality for coronary artery assessment at a low effective dose in pediatric patients. Highlights • ECG-triggered High-Pitch Dual-Source CTA offers good image quality for coronary artery assessment • The left main coronary artery showed the best subjective image quality compared to other coronary segments • Image quality declines in younger and smaller patients as well as those with high heart rate during the CTA • Good image quality can be achieve in patients with a heart rate up to 77 bpm and a body mass index as low as 1.4 m2 • ECG-triggered High-Pitch Dual-Source CTA yields lower effective dose compared to first generation scanners [ABSTRACT FROM AUTHOR]