Search

We report an interesting incidental liver finding during ECG-gated cardiac computed tomography (CT) in a newborn with infracardiac total anomalous pulmonary venous connection to the portal vein. This case shows a unique abnormality in hepatic perfusion that was initially mistaken for hepatic vein thrombosis. We review the altered hepatic blood flow distribution in this pathologic anatomy to help explain the observed hepatic perfusion abnormality on CT. This understanding will enable an imager to anticipate hepatic perfusion patterns in similar patients, potentially avoiding misdiagnosis and unnecessary further testing., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: To investigate the ability of a delayed respiratory-navigated, electrocardiographically-gated three-dimensional inversion recovery-prepared flash low angle shot (3D IR FLASH) sequence to evaluate the lower airways in children undergoing routine cardiovascular magnetic resonance (CMR)., Methods: This retrospective study included pediatric patients (0-18 years) who underwent clinical CMR where a delayed 3D IR FLASH sequence was performed between July 2020 and April 2021. The airway image quality and extent of lower airway visibility was graded by two blinded readers using a four-point ordinal scale (0-3). Lower airway anatomical variants and abnormalities were recorded., Results: 180 patients were included with a median age of 11.7 (4.6-15.3) years. 51/180 (28%) were under general anesthesia (GA). Overall, the median grading of airway image quality was 3 (2-3) and extent of lower airway visibility was 3 (3-3). Interrater agreement was almost perfect (κ = 0.867 and κ = 0.956, respectively). Image quality correlated with extent of lower airway visibility (r = 0.62, p < 0.01). Delayed 3D IR FLASH was able to characterize the segmental bronchi in 137/180 (76%) and lobar bronchi in 172/180 (96%) of patients. Lower airway abnormalities were identified in 37/180 (21%) of patients and in 33/129 (26%) with congenital heart disease (CHD). Identified abnormalities included tracheobronchial branching anomalies in 6/180 (3%), abnormal tracheobronchial situs in 6/180 (3%), and extrinsic vascular compression in 25/180 (14%)., Conclusions: Delayed 3D IR FLASH has excellent performance for evaluation of the lower airway anatomy and can simultaneously assess for myocardial late gadolinium enhancement. Lower airway abnormalities are not infrequently seen in children undergoing routine CMR for CHD., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Cardiac magnetic resonance (CMR) offers valuable hemodynamic insights post-Fontan, but is limited by the absence of normative single ventricle data. The Fontan Outcomes Registry using CMR Examinations (FORCE) is a large international Fontan-specific CMR registry. This study used FORCE registry data to evaluate expected CMR ventricular size/function and create Fontan-specific z-scores adjusting for ventricular morphology (VM) in healthier Fontan patients., Methods: "Healthier" Fontan patients were defined as patients free of adverse outcomes, who are New York Heart Association Class I, have mild or less valve disease, and <30% aortopulmonary collateral burden. General linear modeling was performed on 70% of the dataset to create z-scores for volumes and function. Models were tested using the remainder (30%) of the data. The z-scores were compared between children and adults. The z-scores were also compared between "healthier" Fontan and patients with adverse outcomes (death, listing for transplantation or multiorgan disease)., Results: The "healthier" Fontan population included 885 patients (15.0 ± 7.6 years) from 18 institutions with 1,156 CMR examinations. Patients with left ventricle morphology had lower volume, mass and higher ejection fraction (EF) compared to right or mixed (two-ventricles) morphology (p<0.001 for all pairwise comparisons). Gender, BSA and VM were used in z-scores. Of the "healthier" Fontan patients, 647 were children <18 years and 238 were adults. Adults had lower ascending aorta flow (2.9 ± 0.7 vs 3.3 ± 0.8L/min/m2, p<0.001) and ascending aorta flow z-scores (-0.16 ± 1.23 vs 0.05 ± 0.95, 0.02) compared to children. Additionally, there were 1595 patients with adverse outcomes who were older (16.1 ± 9.3 vs 15.0 ± 7.6, p<0.001) and less likely to have LV morphology (35 vs 47%, p<0.001). Patients with adverse outcomes had higher z-scores for ventricular volume and mass and lower z-scores for EF and ascending aorta flow compared to the "healthier" Fontan cohort., Conclusion: This is the first study to generate CMR z-scores post-Fontan. Importantly the z-scores were generated and tested in "healthier" Fontan patients and both pediatric and adult Fontan patients. These equations may improve CMR-based risk stratification after the Fontan operation., Competing Interests: Declaration of Competing Interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests. Tarek Alsaied reports statistical analysis was provided by National Institutes of Health. Rahul Rathod reports a relationship with Additional Ventures LLC that includes: funding grants. Evan’s Heart Foundation also supported the FORCE registry If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Assessment of the right ventricular outflow tract and pulmonary arteries (RVOT) for percutaneous pulmonary valve implantation (PPVI) uses discrete measurements (diameters and lengths) from medical images. This multi-centre study identified the 3D RVOT shape features prevalent in patients late after surgical repair of congenital heart disease (CHD). A 3D RVOT statistical shape model (SSM) was computed from 81 retrospectively selected CHD patients (14.7 ± 6.8 years) who required pulmonary valve replacement late after surgical repair. A principal component analysis identified prevalent shape features (modes) within the population which were compared with standard geometric measurements (diameter, length and surface area) and between sub-groups of diagnosis, RVOT type and dysfunction. Shape mode 1 and 2 represented RVOT size and curvature and tapering and length, respectively. Shape modes 3-5 related to branch pulmonary artery calibre, conical vs. bulbous RVOTs and RVOT curvature, respectively. Tetralogy of Fallot, transannular patch type and regurgitant RVOTs were larger and straighter while conduit and stenotic types were longer and more cylindrical than other subgroups. This SSM analysed the main 3D shape features present in a population of RVOTs, exploiting the wide 3D anatomical information provided by routine imaging. This morphological information may have implications for PPVI patient selection and device design.

Background: Among patients with congenital heart disease, those with single ventricles have the highest risk of early mortality. Sudden cardiac death is an important cause of death in this population. Understanding the risk factors for sudden cardiac events (SCE) in Fontan patients could improve prediction and prevention., Objectives: The goal of this study was to determine the prevalence of SCE and risk factors for SCE in the Fontan population., Methods: The Fontan Outcomes Registry Using CMR Examinations (FORCE) is an international registry collecting clinical and imaging data on Fontan patients. SCE was defined as: 1) cardiac arrest from a shockable rhythm; 2) need for emergent cardioversion/defibrillation; or 3) documented sustained ventricular tachycardia. Univariate and multivariate Cox proportional hazards regression models estimated hazard ratios for predictors of SCE., Results: Our sample included 3,132 patients (41% female). The median age at first cardiac magnetic resonance was 14.6 years. SCE was experienced by 3.5% (n = 109) over a median follow-up time of 4.00 years. Of the 109 patients with SCE, 39 (36%) died. On multivariable analysis, NYHA functional class >II (HR: 4.91; P < 0.0001), history of protein-losing enteropathy/plastic bronchitis (HR: 2.37; P = 0.0082), single-ventricle end-diastolic volume index >104 mL/m 2 (HR: 3.15; P < 0.0001), and ejection fraction <50% (HR: 1.73; P = 0.0437) were associated with SCE. Kaplan-Meier analysis demonstrated that in patients with none of the above risk factors, the 4-year freedom from SCE was 99.5%., Conclusions: SCE occurred in 3.5% of the study population, and one-third of patients who experienced SCE died. Mild ventricular dysfunction and dilatation by cardiac magnetic resonance, NYHA functional class, and history of protein-losing enteropathy/plastic bronchitis were associated with SCE., Competing Interests: Funding Support and Author Disclosures The FORCE registry is funded through a grant from Additional Ventures and Evan’s Heart. The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Long-term enlargement of the aortic arch after aortic arch reconstruction in hypoplastic left heart syndrome is not well described., Methods: Aortic arch measurements for 50 patients with hypoplastic left heart syndrome who achieved Fontan completion were converted to Pediatric Heart Network z-scores. Dimensions were assessed using linear mixed models, and differences among time points were evaluated with F-tests. Sub-analysis was conducted comparing Norwood (n = 36) with hybrid (n = 14) strategies., Results: Median time to last imaging was 6.4 (interquartile range, 3.5-11.3) years. Before intervention, the main pulmonary artery was dilated, whereas the ascending aorta, transverse arch, and isthmus were hypoplastic. With aortic arch reconstruction, there were expected increases in all arch z-scores. The aortic arch continued to dilate after aortic arch reconstruction, reaching peak values at 7 months (neo-aortic complex: z = 6.9 [5.6-8.0]) or 12 months after stage I (ascending aorta: z = 6.1 [2.9-8.3]; transverse arch: z = 4.7 [3.0-5.9]). After peak values, there was a gradual decline in z-scores with most components still at least mildly dilated at 16 years (neo-aortic complex: z = 3.2 [3.1-3.9], ascending aorta: z = 3.9 [3.3-4.2]; transverse arch: z = 3.1 [2.5-3.7]) with abrupt caliber change at the isthmus: z = -0.8 (-1.1 to -0.3). Norwood and hybrid strategies showed similar enlargement profiles after 7 months of age., Conclusions: Neo-aortic root and aortic arch in hypoplastic left heart syndrome are enlarged early after aortic arch reconstruction and continue to enlarge out of proportion to normal controls until 12 months of age, with gradual decline in enlargement up to adolescence. Further work should focus on modifiable surgical factors that may prove important to optimize arch growth and geometry., Competing Interests: Conflict of Interest Statement The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest., (Copyright © 2024 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Fontan patients exhibit decreased exercise capacity. However, there is a subset of high-performing Fontan (HPF) patients with excellent exercise capacity., Objectives: This study aims to: 1) create a Fontan-specific percent predicted peak VO 2 tool using exercise data; 2) examine clinical factors associated with HPF patients; and 3) examine late outcomes in HPF patients., Methods: Patients in the multi-institutional Fontan Outcomes Registry Using CMR Examination above the age of 8 years who had a maximal exercise test were included. An HPF patient was defined as a patient in the upper Fontan-specific percent predicted peak VO 2 quartile. Multivariable logistic regression was employed to investigate factors associated with the HPF and Cox regression was used to examine the association between HPF patients and late outcomes (composite of death or listing for cardiac transplant)., Results: The study included 813 patients (mean age: 20.2 ± 8.7 years). An HPF patient was associated with left ventricular morphology (OR: 1.50, P  = 0.04), mixed morphology (OR: 2.23, P  < 0.001), and a higher ejection fraction (OR: 1.31 for 10% increase, P  = 0.01). Patients with at least moderate atrioventricular valve regurgitation, protein-losing enteropathy, or who were using psychiatric medications, were less likely to be an HPF patient. After a mean follow-up of 3.7 years, 46 (5.7%) patients developed a composite endpoint. HPF had a lower risk of death or listing for cardiac transplant (HR: 0.06 [95% CI: 0.01-0.25])., Conclusions: Patients with HPF have more favorable outcomes when compared to patients with lower exercise capacity. This large registry data highlights the role of exercise testing in providing personalized care and surveillance post-Fontan., Competing Interests: The FORCE registry is funded through a grant from 10.13039/100020415Additional Ventures and Evan’s Heart. The project described was supported by the 10.13039/100000002National Institutes of Health through Grant Number UL1 TR001857, KL2 TR001856, and/or TL1 TR001858. Dr Rathod received research grant support from Mezzion Pharmaceuticals as the Global PI for the FUEL-2 trial which is a drug RCT in Fontan patients. Dr Alsaied is a center PI for the same trial and also receives similar grant support. The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

The need for urgent or emergent cardiovascular imaging in children is rare when compared to adults. Patients may present from the neonatal period up to adolescence, and may require imaging for both traumatic and non-traumatic causes. In children, coronary pathology is rarely the cause of an emergency unlike in adults where it is the main cause. Radiology, including chest radiography and computed tomography in conjunction with echocardiography, often plays the most important role in the acute management of these patients. Magnetic resonance imaging can occasionally be useful and may be suitable in more subacute cases. Radiologists' knowledge of how to manage and interpret these acute conditions including knowing which imaging technique to use is fundamental to appropriate care. In this review, we will concentrate on the most common cardiovascular emergencies in the thoracic region, including thoracic traumatic and non-traumatic emergencies and pulmonary vascular emergencies, as well as acute clinical disorders as a consequence of primary and postoperative congenital heart disease. This review will cover situations where cardiovascular imaging may be acutely needed, and not strictly emergencies only. Imaging recommendations will be discussed according to the different clinical presentations and underlying pathology., (© 2024. The Author(s).)

Purpose of Review: In the ever-evolving field of medical imaging, this review highlights significant advancements in preoperative and postoperative imaging for Tetralogy of Fallot (TOF) and double outlet right ventricle (DORV) over the past 18 months., Recent Findings: This review showcases innovations in echocardiography such as 3D speckle tracking echocardiography (3DSTE) for assessing right ventricle-pulmonary artery coupling (RVPAC) and Doppler velocity reconstruction (DoVeR) for intracardiac flow fields evaluation. Furthermore, advances in assessment of cardiovascular anatomy using computed tomography (CT) improve the integration of imaging in ablation procedures. Additionally, the inclusion of cardiac magnetic resonance (CMR) parameters as risk score predictors for morbidity, and mortality and for timing of pulmonary valve replacement (PVR) indicates its significance in clinical management. The utilization of 4D flow techniques for postoperative hemodynamic assessment promises new insights into pressure mapping. Lastly, emerging technologies such as 3D printing and 3D virtual reality are expected to improve image quality and surgical confidence in preoperative planning., Summary: Developments in multimodality imaging in TOF and DORV are poised to shape the future of clinical practice in this field., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: Evaluate implementation of an institutional protocol to reduce the time to removal of esophageal button battery (BB) and increase use of mitigation strategies., Methods: We developed a protocol for esophageal BB management [Zakai's Protocol (ZP)]. All cases of esophageal BB impaction managed at a tertiary care center before and after implementation from 2011 to 2023 were reviewed. Time to BB removal, adherence to critical steps, and use of mitigation strategies (honey/sucralfate, acetic acid) were evaluated., Results: Fifty-one patients (38 pre-ZP, 13 post-ZP) were included. Median age was 2.3 years (IQR 1.3-3.4). After implementation, the time from arrival at the institution to arrival in the operating room (OR) reduced by 4.2 h [4.6 h (IQR 3.9-6.5) to 0.4 h (IQR 0.3-0.6), p < 0.001] and there was improvement in all management steps. The number of referrals direct to otolaryngology increased from 51% to 92%, arrival notification increased from 86% to 100%, avoidance of second x-ray increased from 63% to 100%, and direct transfer to OR increased from 92% to 100%. Adherence to mitigation strategies such as preoperative administration of honey or sucralfate increased from 0% to 38%, intraoperative use of acetic acid from 3% to 77%, and nasogastric tube insertion from 53% to 92%., Conclusion: Implementation of ZP substantially reduced the time to BB removal and the use of mitigation strategies in our tertiary care institution. Additional strategies focused on prevention of BB ingestion, and shortening the transfer time to the tertiary care hospital are required to prevent erosive complications., Level of Evidence: Level 3 Case-series Laryngoscope, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose To develop an end-to-end deep learning (DL) pipeline for automated ventricular segmentation of cardiac MRI data from a multicenter registry of patients with Fontan circulation (Fontan Outcomes Registry Using CMR Examinations [FORCE]). Materials and Methods This retrospective study used 250 cardiac MRI examinations (November 2007-December 2022) from 13 institutions for training, validation, and testing. The pipeline contained three DL models: a classifier to identify short-axis cine stacks and two U-Net 3+ models for image cropping and segmentation. The automated segmentations were evaluated on the test set ( n = 50) by using the Dice score. Volumetric and functional metrics derived from DL and ground truth manual segmentations were compared using Bland-Altman and intraclass correlation analysis. The pipeline was further qualitatively evaluated on 475 unseen examinations. Results There were acceptable limits of agreement (LOA) and minimal biases between the ground truth and DL end-diastolic volume (EDV) (bias: -0.6 mL/m 2 , LOA: -20.6 to 19.5 mL/m 2 ) and end-systolic volume (ESV) (bias: -1.1 mL/m 2 , LOA: -18.1 to 15.9 mL/m 2 ), with high intraclass correlation coefficients (ICCs > 0.97) and Dice scores (EDV, 0.91 and ESV, 0.86). There was moderate agreement for ventricular mass (bias: -1.9 g/m 2 , LOA: -17.3 to 13.5 g/m 2 ) and an ICC of 0.94. There was also acceptable agreement for stroke volume (bias: 0.6 mL/m 2 , LOA: -17.2 to 18.3 mL/m 2 ) and ejection fraction (bias: 0.6%, LOA: -12.2% to 13.4%), with high ICCs (>0.81). The pipeline achieved satisfactory segmentation in 68% of the 475 unseen examinations, while 26% needed minor adjustments, 5% needed major adjustments, and in 0.4%, the cropping model failed. Conclusion The DL pipeline can provide fast standardized segmentation for patients with single ventricle physiology across multiple centers. This pipeline can be applied to all cardiac MRI examinations in the FORCE registry. Keywords: Cardiac, Adults and Pediatrics, MR Imaging, Congenital, Volume Analysis, Segmentation, Quantification Supplemental material is available for this article. © RSNA, 2023.

The Single Ventricle Reconstruction (SVR) Trial was a randomized prospective trial designed to determine survival advantage of the modified Blalock-Taussig-Thomas shunt (BTTS) vs the right ventricle to pulmonary artery conduit (RVPAS) for patients with hypoplastic left heart syndrome. The primary aim of the long-term follow-up (SVRIII) was to determine the impact of shunt type on RV function. In this work, we describe the use of CMR in a large cohort follow up from the SVR Trial as a focused study of single ventricle function. The SVRIII protocol included short axis steady-state free precession imaging to assess single ventricle systolic function and flow quantification. There were 313 eligible SVRIII participants and 237 enrolled, ages ranging from 10 to 12.5 years. 177/237 (75%) participants underwent CMR. The most common reasons for not undergoing CMR exam were requirement for anesthesia (n = 14) or ICD/pacemaker (n = 11). A total of 168/177 (94%) CMR studies were diagnostic for RVEF. Median exam time was 54 [IQR 40-74] minutes, cine function exam time 20 [IQR 14-27] minutes, and flow quantification time 18 [IQR 12-25] minutes. There were 69/177 (39%) studies noted to have intra-thoracic artifacts, most common being susceptibility artifact from intra-thoracic metal. Not all artifacts resulted in non-diagnostic exams. These data describe the use and limitations of CMR for the assessment of cardiac function in a prospective trial setting in a grade-school-aged pediatric population with congenital heart disease. Many of the limitations are expected to decrease with the continued advancement of CMR technology., (© 2023. The Author(s).)

Objectives: To assess abnormal liver enhancement on 15-20 min delayed 3D high-resolution late gadolinium enhancement (3D HR LGE) sequence in patients with Fontan circulation., Methods: Retrospective study of pediatric Fontan patients (< 18 years old) with combined cardiac-liver MRI from January 2018 to August 2021. Abnormal hepatic enhancement was graded (0-3) for each lobe, summed for a total liver enhancement score (0-6), and compared to repaired tetralogy of Fallot (rTOF) patients. Correlations with other hepatic imaging biomarkers were performed. Temporal relationships of enhancement compared to traditional early portal venous and 5-7-min delayed phase liver imaging were analyzed., Results: The Fontan group (n = 35, 13 ± 3.4 years old, median time from Fontan 10 (9-12) years) had 23/35 (66%) with delayed 3D HR LGE total liver enhancement score > 0 (range 0-5), with greater involvement of the right lobe (1 (0-1) vs 0 (0-1), p < 0.01). The rTOF group (n = 35, 14 ± 2.6 years old) had no abnormal enhancement. In the Fontan group, total liver enhancement was 3 (2-4) in the early portal venous phase, lower at 1 (1-2) in the 5-7-min delayed phase (p < 0.01), and lowest at 1 (0-2) in the 15-20-min delayed phase (p = 0.03). 3D HR LGE enhancement correlated inversely with portal vein flow (r s  =  - 0.42, p = 0.01) and positively with left lobe stiffness (r s  = 0.51, p < 0.01). The enhancement score decreased in 13/35 (37%) between the 5-7- and 15-20-min delayed phases., Conclusions: Liver fibrosis can be assessed on 3D HR LGE sequences in patients with Fontan circulation, correlates with other imaging biomarkers of Fontan liver disease, and may add information for hepatic surveillance in this population., Key Points: • Abnormal liver enhancement on 3D HR LGE sequences in Fontan patients likely represents liver fibrosis and is seen in up to 66% of children and adolescents with variable distribution and severity. • The degree of 3D HR LGE liver enhancement correlates with decreased portal vein flow and increased left hepatic lobe stiffness., (© 2023. The Author(s), under exclusive licence to European Society of Radiology.)

Cardiac tumors in children are rare and the majority are benign. The most common cardiac tumor in children is rhabdomyoma, usually associated with tuberous sclerosis complex. Other benign cardiac masses include fibromas, myxomas, hemangiomas, and teratomas. Primary malignant cardiac tumors are exceedingly rare, with the most common pathology being soft tissue sarcomas. This paper provides consensus-based imaging recommendations for the evaluation of patients with cardiac tumors at diagnosis and follow-up, including during and after therapy., (© 2022 Wiley Periodicals LLC.)

Aims: To quantify thoracic lymphatic burden in paediatric Fontan patients using MRI and correlate with clinical status., Methods and Results: Paediatric Fontan patients (<18-years-old) with clinical cardiac MRI that had routine lymphatic 3D T2 fast spin echo (FSE) imaging performed from May 2017 to October 2019 were included. 'Lymphatic burden' was quantified by thresholding-based segmentation of the 3D T2 FSE maximum intensity projection image and indexed to body surface area, performed by two independent readers blinded to patient status. There were 48 patients (27 males) with median age at MRI of 12.9 (9.4-14.7) years, time from Fontan surgery to MRI of 9.1 (5.9-10.4) years, and follow-up time post-Fontan surgery of 9.4 (6.6-11.0) years. Intraclass correlation coefficient between two observers for lymphatic burden was 0.96 (0.94-0.98). Greater lymphatic burden correlated with post-Fontan operation hospital length of stay and duration of chest tube drainage (rs = 0.416, P = 0.004 and rs = 0.439, P = 0.002). Median lymphatic burden was greater in patients with chylous effusions immediately post-Fontan (178 (118-393) vs. 113 (46-190) mL/m2, P = 0.028), and in patients with composite adverse Fontan status (n = 13) defined by heart failure (n = 3), transplant assessment (n = 2), recurrent effusions (n = 6), Fontan thrombus (n = 2), and/or PLE (n = 6) post-Fontan (435 (137-822) vs. 114 (51-178) mL/m2, P = 0.003). Lymphatic burden > 600 mL/m2 was associated with late adverse Fontan status with sensitivity of 57% and specificity of 95%., Conclusion: Quantification of MR lymphatic burden is a reliable tool to assess the lymphatics post-Fontan and is associated with clinical status., Competing Interests: Conflicts of interest: A.I.D. is a board member-at-large of the American Society of Transplantation and the medical director of the David Foster Foundation. E.J.-S.-M. receives grant funding from the Canadian Institute of Health Research. C.Z.L. receives funding as part of FORCE (Fontan Outcomes Registry using CMR Examinations). The other authors have no other relevant disclosures. ., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Background: This study compares three-dimensional (3D) high-resolution (HR) late gadolinium enhancement (LGE; 3D HR-LGE) imaging using a respiratory navigated, electrocardiographically-gated inversion recovery gradient echo sequence with conventional LGE imaging using a single-shot phase-sensitive inversion recovery (PSIR) balanced steady-state free precession (bSSFP; PSIR-bSSFP) sequence for routine clinical use in the pediatric population., Methods: Pediatric patients (0-18 years) who underwent clinical cardiovascular magnetic resonance (CMR) with both 3D HR-LGE and single-shot PSIR-bSSFP LGE between January 2018 and June 2020 were included. Image quality (0-4) and detection of LGE in the left ventricle (LV) (per 17 segments), in the right ventricle (RV) (per 3 segments), as endocardial fibroelastosis (EFE), at the hinge points, and at the papillary muscles was analyzed by two blinded readers for each sequence. Ratios of the mean signal intensity of LGE to normal myocardium (LGE:Myo) and to LV blood pool (LGE:Blood) were recorded. Data is presented as median (1st-3rd quartiles). Wilcoxon signed rank test and chi-square analyses were used as appropriate. Inter-rater agreement was analyzed using weighted κ-statistics., Results: 102 patients were included with median age at CMR of 8 (1-13) years-old and 44% of exams performed under general anesthesia. LGE was detected in 55% of cases. 3D HR LGE compared to single-shot PSIR-bSSFP had longer scan time [4:30 (3:35-5:34) vs 1:11 (0:47-1:32) minutes, p < 0.001], higher image quality ratings [3 (3-4) vs 2 (2-3), p < 0.001], higher LGE:Myo [23.7 (16.9-31.2) vs 5.0 (2.9-9.0), p < 0.001], detected more segments of LGE in both the LV [4 (2-8) vs 3 (1-7), p = 0.045] and RV [1 (1-1) vs 1 (0-1), p < 0.001], and also detected more cases of LGE with 13/56 (23%) of patients with LGE only detectable by 3D HR LGE (p < 0.001). 3D HR LGE specifically detected a greater proportion of RV LGE (27/27 vs 17/27, p < 0.001), EFE (11/11 vs 5/11, p = 0.004), and papillary muscle LGE (14/15 vs 4/15, p < 0.001). Inter-rater agreement for the recorded variables ranged from 0.42 to 1.00., Conclusions: 3D HR LGE achieves greater image quality and detects more LGE than conventional single-shot PSIR-bSSFP LGE imaging, and should be considered an alternative to conventional LGE sequences for routine clinical use in the pediatric population., (© 2023. The Author(s).)

The pulmonary circulation after one and one-half ventricle repair is complex because of the direct connection of two sources of blood flow. Associated pulmonary regurgitation further complicates the pulmonary circulation. We report the complex hemodynamics depicted by phase-contrast magnetic resonance imaging in a patient with one and one-half ventricle circuit and severe pulmonary regurgitation. Antegrade superior vena caval flow occurred almost exclusively during diastole and regurgitated into the right ventricle. Consequently, the entire systemic venous return reached the right ventricle during diastole and is pumped back into the pulmonary arteries in systole. The case highlights the importance of analyzing flow patterns phase-by phase throughout the cardiac cycle instead of relying on the net flow volumes through each source of pulmonary blood flow., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: 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., Objectives: 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., Methods: 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., Results: 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., Conclusions: CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Crown Copyright © 2022. Published by Elsevier Inc. All rights reserved.)

Cardiovascular magnetic resonance (CMR) and cardiac computed tomography (CCT) are robust cross-sectional imaging modalities that are increasingly being used to guide the diagnosis and management of those born with single-ventricle physiology. The purpose of this review is to acquaint the reader with the wide range of cross-sectional imaging applications that can be applied in this population. Illustrative examples of information provided by CMR and CCT are included, such as delineation of cardiac anatomy, measurement of ventricular volumetry, interrogation of vascular anatomy and flows, evaluation of myocardial viability, and exclusion of thromboembolic disease. Focus is placed on the appropriate selection and timing of advanced cardiac imaging modalities, and differentiations are made between established indications for imaging and emerging applications (such as 4-dimensional [4D] flow assessment and parametric imaging for identification of diffuse fibrosis using CMR). Potential CMR and CCT contributions in the selection of interventional strategies in the child and for surveillance of postoperative complications in the adult are highlighted. Furthermore, the importance of extracardiac cross-sectional imaging in the single-ventricle population is emphasized given the recognition that complications post-Fontan palliation are not limited to the cardiovascular system but will often include extracardiac manifestations of disease (such as hepatic disease or lymphatic abnormalities), While the value of CMR and CCT for contemporary clinical care of individuals born with single-ventricle physiology appears to be well-established, further research will be necessary to explore the impact of risk stratification using imaging biomarkers on clinical outcomes following Fontan palliation., (Copyright © 2022 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Purpose: To assess regional blood flow in fasting pediatric patients with Fontan circulation by using MRI and to explore associations with clinical parameters., Materials and Methods: In this retrospective study, pediatric patients who had undergone the Fontan procedure (<18 years of age) and had undergone clinical cardiac MRI, performed after at least 4 hours of fasting, between 2018 and 2021 were included. Regional blood flow was compared with published healthy volunteer data ( n = 19) and assessed in relation to hemodynamic parameters and clinical status. Data are presented as medians, with first to third quartiles in parentheses. Mann-Whitney U , Kruskal-Wallis, χ 2 , and Spearman rank correlation tests were used., Results: Fifty-five patients (38 boys) with median age at MRI of 14 years (IQR, 11-16 years) and median time from Fontan procedure to MRI of 10 years (IQR, 8-12 years) were included. Patients after Fontan procedure had lower ascending aortic, inferior vena cava, and total systemic blood flow compared with healthy volunteers (3.00 L/min/m 2 [IQR, 2.75-3.30 L/min/m 2 ] vs 3.61 L/min/m 2 [IQR, 3.29-4.07 L/min/m 2 ]; 1.73 L/min/m 2 [IQR, 1.40-1.94 L/min/m 2 ] vs 2.24 L/min/m 2 [IQR, 2.06-2.75 L/min/m 2 ]; 2.78 L/min/m 2 [IQR, 2.45-3.10 L/min/m 2 ] vs 3.95 L/min/m 2 [IQR, 3.20-4.30 L/min/m 2 ], respectively; P < .001). Portal vein flow was greater than hepatic vein flow in 25% of patients. Fontan blood flow was inversely correlated with pre-Fontan mean pulmonary artery pressure (Spearman rank correlation coefficient [ r s ]= -0.42, P = .005) and ventricular end diastolic pressure ( r s = -0.33, P = .04) and positively correlated with post-Fontan percent predicted oxygen consumption at peak workload ( r s = 0.34, P = .02)., Conclusion: Reference ranges are provided for regional systemic blood flow derived by using MRI in fasting pediatric patients with Fontan circulation, who had lower systemic blood flow compared with healthy volunteers. Lower fasting Fontan blood flow correlated with lower exercise capacity. Keywords : Pediatrics, Heart, Congenital, MR Imaging, Hemodynamics/Flow Dynamics, Cardiac Supplemental material is available for this article. © RSNA, 2022., Competing Interests: Disclosures of conflicts of interest: C.Z.L. Grant-funded reimbursement from Additional Ventures as the site primary investigator at the Hospital for Sick Children for participation in the Fontan Outcomes Registry using CMR Extractions (FORCE); member of the executive leadership team for FORCE. D.D. No relevant relationships. L.A.I. No relevant relationships. P.P. No relevant relationships. A.I.D. No relevant relationships. E.J.S.M. No relevant relationships. R.R.C. No relevant relationships. S.C.L. No relevant relationships. R.M.W. No relevant relationships. G.B.C. Service on the Society for Pediatric Radiology Board. M.S. No relevant relationships. S.J.Y. No relevant relationships., (© 2022 by the Radiological Society of North America, Inc.)

Pediatric hypertrophic cardiomyopathy (HCM) is the most common form of cardiomyopathy in children and a leading cause of sudden cardiac death. Yet, the association between genotype variation, phenotype expression, and adverse events in pediatric HCM has not been fully elucidated. Although the literature on this topic is evolving in adult HCM, the evidence in children is lacking. Solidifying our understanding of this relationship could improve risk stratification as well as improve our comprehension of the underlying pathophysiological characteristics of pediatric HCM. In this state-of-the-art review, we examine the current literature on genetic variations in HCM and their association with outcomes in children, discuss the current approaches to identifying cardiovascular phenotypes in pediatric HCM, and explore possible avenues that could improve sudden cardiac death risk assessment.

Background: Dynamic contrast-enhanced magnetic resonance imaging (MRI) of the liver in pediatric Fontan patients often shows peripheral reticular areas of hypoenhancement, which has not been studied in detail., Objective: To semiquantitatively score the hepatic MR perfusion abnormality seen in pediatric Fontan patients, and to correlate the perfusion abnormality with functional clinical and hemodynamic parameters., Materials and Methods: All children (< 18 years old) after Fontan palliation with combined clinical cardiac and liver MRI performed between May 2017 and April 2019 were considered for inclusion. A semiquantitative perfusion score was used to assess the severity of the hepatic reticular pattern seen on dynamic contrast-enhanced liver imaging. The liver was divided into four sections: right posterior, right anterior, left medial and left lateral. Each liver section was assigned a score from 0 to 4 depending on the amount of abnormal reticular hypoenhancement. Scoring was assigned for each section of the liver across eight successive dynamic contrast-enhanced modified spoiled gradient echo runs. Scores were correlated with clinical and hemodynamic parameters., Results: All Fontan children showed hepatic reticular hypoenhancement by MRI, most severe in the early portal venous phase with a median maximum total perfusion abnormality score of 12 (range: 9-14). All perfusion abnormalities progressively resolved during the hepatic venous phase. Perfusion abnormality scores were greatest in the right compared to left hepatic lobes (7 range: [6-8] vs. 5 [range: 3-6], P < 0.01). The maximum left hepatic lobe perfusion abnormality scores were greatest in children with versus without imaging signs of portal hypertension (8 [range: 7-8] vs. 4 [range: 3-5], P < 0.01). High unconjugated bilirubin and low platelets correlated with greater perfusion abnormality (R = 0.450, P = 0.024, and R =  - 0.458, P < 0.01, respectively). Age at MRI, time from Fontan, focal liver lesions and cardiac MRI hemodynamic parameters did not show significant correlations with the severity of the liver perfusion abnormality., Conclusion: All Fontan children have hepatic reticular hypoenhancement abnormalities seen with MRI that are most severe in the right hepatic lobe and universally show gradual resolution through the hepatic venous phase. Perfusion abnormality in the left hepatic lobe is worse in children with portal hypertension., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

This review introduces the basic principles of contrast-enhanced magnetic resonance (MR) angiography and details four contrast-enhanced MR angiography sequences for body imaging with extracellular gadolinium-based contrast agents in pediatric patients. Specifically, this review covers (1) respiratory-navigated, cardiac-gated MR angiography; (2) time-resolved MR angiography; (3) conventional MR angiography; and (4) modified spoiled gradient echo variants. We present and discuss indications, technical considerations, sequence optimization, advantages and disadvantages, along with practical tips and illustrative case examples for each sequence., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature.)

We report an anterior translocation of the right pulmonary artery procedure to relieve severe left bronchial obstruction that was caused by right pulmonary artery stent placement in a 1-year-old patient with truncus arteriosus and interrupted aortic arch. After neonatal repair, the patient re-presented with severe truncal valve regurgitation, right pulmonary artery stenosis, and severe biventricular dysfunction, which was treated with truncal valve repair and right pulmonary artery plasty. The patient suffered from left bronchial compression from right pulmonary artery stent placement, which was successfully treated by the translocation procedure. Bronchial stenosis was successfully relieved by the translocation procedure. Indications, advantages, and disadvantages of this procedure are discussed.

Background: The extent and significance in of cardiac remodeling in Fontan patients are unclear and were the subject of this study., Methods: This retrospective cohort study compared cardiovascular magnetic resonance (CMR) imaging markers of cardiac function, myocardial fibrosis, and hemodynamics in young Fontan patients to controls., Results: Fifty-five Fontan patients and 44 healthy controls were included (median age 14 years (range 7-17 years) vs 13 years (range 4-14 years), p = 0.057). Fontan patients had a higher indexed end-diastolic ventricular volume (EDVI 129 ml/m 2 vs 93 ml/m 2 , p < 0.001), and lower ejection fraction (EF 45% vs 58%, p < 0.001), circumferential (CS - 23.5% vs - 30.8%, p < 0.001), radial (6.4% vs 8.2%, p < 0.001), and longitudinal strain (- 13.3% vs - 24.8%, p < 0.001). Compared to healthy controls, Fontan patients had higher extracellular volume fraction (ECV) (26.3% vs 20.6%, p < 0.001) and native T1 (1041 ms vs 986 ms, p < 0.001). Patients with a dominant right ventricle demonstrated larger ventricles (EDVI 146 ml/m 2 vs 120 ml/m 2 , p = 0.03), lower EF (41% vs 47%, p = 0.008), worse CS (- 20.1% vs - 25.6%, p = 0.003), and a trend towards higher ECV (28.3% versus 24.1%, p = 0.09). Worse EF and CS correlated with longer cumulative bypass (R = - 0.36, p = 0.003 and R = 0.46, p < 0.001), cross-clamp (R = - 0.41, p = 0.001 and R = 0.40, p = 0.003) and circulatory arrest times (R = - 0.42, p < 0.001 and R = 0.27, p = 0.03). T1 correlated with aortopulmonary collateral (APC) flow (R = 0.36, p = 0.009) which, in the linear regression model, was independent of ventricular morphology (p = 0.9) and EDVI (p = 0.2). The composite outcome (cardiac readmission, cardiac reintervention, Fontan failure or any clinically significant arrhythmia) was associated with increased native T1 (1063 ms vs 1026 ms, p = 0.029) and EDVI (146 ml/m 2 vs 118 ml/m 2 , p = 0.013), as well as decreased EF (42% vs 46%, p = 0.045) and worse CS (- 22% vs - 25%, p = 0.029). APC flow (HR 5.5 CI 1.9-16.2, p = 0.002) was independently associated with the composite outcome, independent of ventricular morphology (HR 0.71 CI 0.30-1.69 p = 0.44) and T1 (HR1.006 CI 1.0-1.13, p = 0.07)., Conclusions: Pediatric Fontan patients have ventricular dysfunction, altered myocardial mechanics and increased fibrotic remodeling. Cumulative exposure to cardiopulmonary bypass and increased aortopulmonary collateral flow are associated with myocardial dysfunction and fibrosis. Cardiac dysfunction, fibrosis, and collateral flow are associated with adverse outcomes., (© 2021. The Author(s).)

OBJECTIVE. The Fontan procedure has significantly improved the survival in children with a functional single ventricle, but it is associated with chronically elevated systemic venous pressure that leads to multisystemic complications. Imaging plays an important role in assessing these complications and guiding management. The pathophysiology, imaging modalities, and current surveillance recommendations are discussed and illustrated. CONCLUSION. Significant improvement in survival of patients with Fontan circulation is associated with ongoing cardiac and extracardiac comorbidities and multisystemic complications. The liver and intestines are particularly vulnerable to damage. In addition, this patient population has been shown to be at increased risk of certain malignancies such as hepatocellular carcinoma and neuroendocrine tumors. Familiarity with imaging findings of Fontan-associated liver disease and other abdominal complications of the Fontan circulation is essential for radiologists because we are likely to encounter these patients in our general practice.

Neonatal ductus arteriosus aneurysm (DAA) is a rare abnormality that is typically diagnosed at fetal third trimester or early postnatal echocardiography. While echocardiography is usually adequate for diagnosis and clinical decision-making, cross-sectional imaging, including CT or cardiac MRI, may be necessary to clarify the diagnosis or delineate associated complications. Severe complications include thromboembolism, infection, compression of adjacent structures, airway erosion, and aneurysm rupture. This imaging essay reviews the pathophysiology and depicts the spectrum of cross-sectional imaging appearances of neonatal DAAs. Most neonatal DAAs will spontaneously regress and can be managed conservatively. Keywords: CT, MRI, Cardiac, Aneurysms, Congenital Supplemental material is available for this article. ©RSNA, 2021., Competing Interests: Disclosures of Conflicts of Interest: B.B. disclosed no relevant relationships. S.A. disclosed no relevant relationships. S.J.Y. disclosed no relevant relationships. M.S. disclosed no relevant relationships. C.Z.L. disclosed no relevant relationships., (2021 by the Radiological Society of North America, Inc.)

Study Objective: To evaluate the diagnostic performance of a Volume and Solid Vascular Tissue Score (VSVTS) for preoperative risk assessment of pediatric and adolescent adnexal masses., Design: A retrospective cohort study comprised of all female individuals who presented with an adnexal mass that was managed surgically between April 2011 and March 2016., Setting: The Hospital for Sick Children (Toronto, Ontario, Canada)., Participants: Female individuals 1-18 years of age who presented to a large tertiary pediatric hospital with an adnexal mass that was managed surgically., Main Outcome Measures: Main outcome measures included diagnostic performance of the VSVTS for malignancy via sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), positive likelihood ratio (LR+), negative likelihood ratio (LR-), and receiver operating characteristic area-under-the-curve (AUC) analysis., Results: A total of 179 masses in 169 subjects were included. The malignancy rate was 10.6%. The AUC for the VSTVS was 0.919. A VSTVS cut-off value of 4 achieved a sensitivity of 79% (95% CI 0.54-0.93), specificity of 88% (95% CI 0.82-0.93), PPV of 0.44 (95% CI 0.33-0.56), NPV of 0.97 (95% CI 0.94-0.99), LR+ of 6.77 (95% CI 4.18-10.97), and LR- of 0.24 (95% CI 0.10-0.57)., Conclusions: A sonographic scoring system based on the volume and presence of solid vascular tissue improves PPV for preoperative risk stratification of adnexal masses in the pediatric and adolescent population compared to existing ultrasound-only approaches. Further prospective research is needed to determine how best to incorporate components of such scoring systems into clinical management algorithms., (Copyright © 2020 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Background: Cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) requires identification of the normal myocardial nulling time using inversion time (TI)-scout imaging sequence. Although TI-scout images are not primarily used for myocardial assessment, they provide information regarding different signal recovery patterns of normal and abnormal myocardium facilitating identification of LGE in instances where standard LGE images alone are not diagnostic. We aimed to assess the diagnostic performance of TI-scout as compared to that of standard LGE images., Methods: CMR studies with LGE imaging in 519 patients (345 males, 1-17 years) were reviewed to assess the diagnostic performance of LGE imaging in terms of the location of LGE and the pathologic entities. The diagnostic performance of the TI-scout and standard LGE imaging was classified into four categories: (1) equally diagnostic, (2) TI-scout superior to standard LGE, (3) standard LGE superior to TI-scout, and (4) complementary, by the consensus of the two observers., Results: The study cohort consisted of 440 patients with negative LGE and 79 with evidence for LGE. For a negative diagnosis of LGE, TI-scout and standard LGE images were equally diagnostic in 75% of the cases and were complementary in 12%. For patients with LGE, TI-scout images were superior to standard LGE images in 52% of the cases and were complementary in 19%. The diagnostic performance of TI-scout images was superior to that of standard LGE images in all locations. TI-scout images were superior to standard LGE images in 11 of 12 (92%) cases with LGE involving the papillary muscles, in 7 /12 (58%) cases with subendocardial LGE, and in 4/7 (57%) cases with transmural LGE. TI-scout images were particularly useful assessing the presence and extent of LGE in hypertrophic cardiomyopathy (HCM). TI-scout was superior to standard LGE in 6/10 (60%) and was complementary in 3/10 (30%) of the positive cases with HCM., Conclusions: TI-scout images enhance the diagnostic performance of LGE imaging in children.

3D printing allows the most realistic perception of the surgical anatomy of congenital heart diseases without the requirement of physical devices such as a computer screen or virtual headset. It is useful for surgical decision making and simulation, hands-on surgical training (HOST) and cardiovascular morphology teaching. 3D-printed models allow easy understanding of surgical morphology and preoperative surgical simulation. The most common indications for its clinical use include complex forms of double outlet right ventricle and transposition of the great arteries, anomalous systemic and pulmonary venous connections, and heterotaxy. Its utility in congenital heart surgery is indisputable, although it is hard to "scientifically" prove the impact of its use in surgery because of many confounding factors that contribute to the surgical outcome. 3D-printed models are valuable resources for morphology teaching. Educational models can be produced for almost all different variations of congenital heart diseases, and replicated in any number. HOST using 3D-printed models enables efficient education of surgeons in-training. Implementation of the HOST courses in congenital heart surgical training programs is not an option but an absolute necessity. In conclusion, 3D printing is entering the stage of maturation in its use for congenital heart surgery. It is now time for imagers and surgeons to find how to effectively utilize 3D printing and how to improve the quality of the products for improved patient outcomes and impact of education and training., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Yoo, Hussein, Peel, Coles, Arsdell, Honjo, Haller, Lam, Seed and Barron.)

Purpose: (1) To assess the visibility and diameters of the thoracic duct (TD) and cisterna chyli (CC) on MR cholangiopancreatography (MRCP) in children. (2) To evaluate for the presence of any lymphatic abnormalities and assess their association with diseases in which the immune system is implicated in etiopathogenesis., Methods: This retrospective study included 142 MRCPs performed in children 8-17 years old and without prior surgeries. Two radiologists reviewed all exams for visibility and diameters of the TD and CC, and presence of abnormal lymphatic collaterals. TD and CC diameters in various disease processes were compared using Student's t tests. The association of collaterals with immune-mediated diseases was assessed using Fisher's exact tests., Results: The TD and CC were seen in 134/142 (93.7%) cases with mean diameter of 3.25 ± 1.07 mm and 126/142 (88.7%) cases with mean diameter of 4.55 ± 1.37 mm respectively. The mean diameter of CC was larger in patients with portal hypertension (p = 0.021). There were no significant differences in the TD and CC diameters between immune-mediated and non-immune-mediated diseases. Retroperitoneal collaterals were seen in 41/142 (28.8%) of cases and were associated with both portal hypertension (p = 0.0019) and immune-mediated diseases (p = 0.0083)., Conclusion: The TD and CC can be visualized in the majority of children on routine MRCP images, and CC has larger diameter in patients with portal hypertension. The association of collaterals with immune-mediated diseases supports a potential role of the lymphatic system in the etiopathogenesis of immune-mediated diseases., Key Points: • The lymphatic system has been increasingly implicated in a number of inflammatory and immune-mediated diseases. • The abdominal lymphatic system can be visualized in the majority of children above 8 years on routine MRCP images. Similar to adult studies, the cisterna chyli is significantly larger in children with portal hypertension. • Retroperitoneal lymphatic collaterals, seen in 29% children, are associated with immune-mediated diseases, which supports the potential role of the lymphatic system in the pathogenesis of immune-mediated diseases.

Various congenital heart diseases are associated with malalignment of a part of the ventricular septum. Most commonly, the outlet septum is malaligned toward the right or left ventricle. Less commonly, the whole or a major part of the ventricular septum is malaligned in relation to the atrial septal plane. Although the pathological conditions associated with ventricular septal malalignment have been well recognized, the descriptions are often confusing and sometimes incorrect. In this pictorial essay, we introduce our systematic approach to the assessment of malalignment type ventricular septal defects with typical case examples. The systematic approach comprises description of the essential features of malalignment, including the following: (1) the malaligned part of the ventricular septum, (2) the reference structure, (3) the mechanism of malalignment, (4) the direction of malalignment, and (5) the severity of malalignment.

Purpose: To establish normative data for myocardial T1, including extracellular volume (ECV) fraction, in healthy children., Materials and Methods: In this retrospective, single-center study, T1 mapping data were collected from 48 healthy pediatric patients (14 years ± 3 [standard deviation]; range, 9-18 years; 27 of 48 [56%] male) referred for cardiac screening 1.5-T MRI between 2014 and 2017. T1 relaxometry was performed using a 5(number of heartbeats [nHB])3 modified Look-Locker inversion recovery (MOLLI) sequence, where nHB was three to five heartbeats depending on the heart rate, and was repeated 15 minutes following the administration of 0.2 mmol per kilogram of body weight of gadobenate dimeglumine, with 19 patients receiving contrast material. T1 values were calculated using a curve-fitting algorithm on average region-of-interest signal and corrected for imperfect inversion pulse efficiency. Comparisons within patients were performed with paired Student t test, between groups with unpaired Student t test or Mann-Whitney U test, and linear regression was performed to examine for associations with other variables., Results: Average native T1 was 1008 msec ± 31, with a nonsignificant increase in females (1017 msec ± 27 vs 1001 msec ± 33, P = .066). Average ECV was 20.8% ± 2.4, with a nonsignificant increase in values in females (21.7% ± 1.9 vs 20.0% ± 2.6, P = .123). T1 and ECV values were increased in the septum versus the free wall., Conclusion: Normative data are presented for myocardial native T1 and ECV using the MOLLI T1 mapping sequence at 1.5 T. Supplemental material is available for this article. © RSNA, 2020., Competing Interests: Disclosures of Conflicts of Interest: J.J.P.. disclosed no relevant relationships. D.Y. disclosed no relevant relationships. C.Z.L. disclosed no relevant relationships. S.J.Y. disclosed no relevant relationships. M.S. disclosed no relevant relationships. L.G.W. disclosed no relevant relationships., (2020 by the Radiological Society of North America, Inc.)

Dynamic contrast-enhanced magnetic resonance lymphangiography is a novel technique to image central conducting lymphatics. It is performed by injecting contrast into groin lymph nodes and following passage of contrast through lymphatic system using T1-weighted MR images. Currently, it has been successfully applied to image and plan treatment of thoracic duct pathologies, lymphatic leaks, and other lymphatic abnormalities such as plastic bronchitis. It is useful in the assessment of chylothorax and chyloperitoneum. Its role in other areas such as intestinal lymphangiectasia and a variety of lymphatic anomalies is likely to increase., Competing Interests: Disclosure All authors have no funding to disclose related to this work., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Study Objective: To evaluate the diagnostic performance of the Decision Tree System (DTS) rules 2 and 3 for surgically managed adnexal masses in the North American population and to compare it with the risk stratification criteria used at The Hospital for Sick Children (≥8 cm and complex/solid)., Design: A retrospective cohort study of patients who presented with adnexal masses and were surgically treated between April 2011 and March 2016., Setting: The Hospital for Sick Children (Toronto, Ontario, Canada)., Participants: Patients 1-18 years of age with adnexal masses who underwent surgical treatment., Interventions and Main Outcome Measures: Main outcome measures included diagnostic performance (preoperative sensitivity, specificity, positive predictive value [PPV], and negative predictive value [NPV] for malignancy) of the DTS rules 2 and 3 and ≥8 cm and complex/solid criteria., Results: The malignancy rate was 10.4%. The DTS rules 2 and 3 had a sensitivity of 84% (95% confidence interval [CI], 79-90), specificity of 77% (95% CI, 71-83), PPV of 30% (95% CI, 17-42), and NPV of 98% (95% CI, 94-100). The 8 cm or larger and complex/solid criteria had a sensitivity of 89% (95% CI, 85-94), specificity of 71% (95% CI, 64-77), PPV of 27% (95% CI, 16-38), and NPV of 98% (95% CI, 96-100)., Conclusion: Our study showed that DTS rules 2 and 3 had similar diagnostic performance as the 8 cm or larger and complex/solid criteria in the same population, with a very high NPV and a low PPV. Future prospective investigations should be conducted to further assess how DTS components can be incorporated into future algorithms for the management of adnexal masses in the pediatric population., (Copyright © 2019 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Background: Myocardial T1 relaxometry can be performed by contouring on individual T1-weighted source images (source method) or on a single T1 map (mapping method)., Objective: This study compares (a) agreement between native T1 and extracellular volume results of the two methods and (b) interobserver reproducibility of the two methods in children without heart disease and those with tetralogy of Fallot (TOF)., Materials and Methods: We retrospectively analyzed pediatric patients (controls and those with repaired TOF) with cardiac magnetic resonance examinations including extracellular volume quantification using the modified Look-Locker inversion recovery (MOLLI) sequence. We compared native T1 and extracellular volume of the entire left ventricle and interventricular septum derived using the source and the mapping approaches., Results: In the control group (n=25, median age 14.0 years, interquartile range [IQR] 11.5-16.5 years), the mapping method produced lower native T1 values than the source method in the interventricular septum (mean difference ± standard deviation [SD] = 12±15 ms, P<0.001). In the TOF group (n=50, median age 13.3 years, IQR 9.9-15.0 years), the mapping method produced lower values for native T1 and extracellular volume in the interventricular septum (mean difference 9±14 ms and 0.6±1.1%, P<0.001). In 6-12% of the children, differences were >3 standard deviations from the mean difference. Interobserver reproducibility between the two methods by intraclass correlation coefficients were clinically equivalent., Conclusion: T1 and extracellular volume values generated by the source and mapping methods show systematic differences and can vary significantly in an individual child, and thus cannot be used interchangeably in clinical practice. The source method might allow for easier detection and, in some cases, mitigation of artifacts that are not infrequent in children and can be difficult to appreciate on the T1 map.

Background: Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion., Methods: Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls., Results: Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan., Conclusion: Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures., Trial Registration: Retrospectively registered data.

Objective: To investigate the regional flow distribution in patients with Fontan circulation by using magnetic resonance imaging (MRI)., Materials and Methods: We identified 39 children (18 females and 21 males; mean age, 9.3 years; age range, 3.3-17.0 years) with Fontan circulation in whom flow volumes across the thoracic and abdominal arteries and veins were measured by using MRI. The patients were divided into three groups: fenestrated Fontan circulation group with MRI performed under general anesthesia (GA) (Group 1, 15 patients; average age, 5.9 years), completed Fontan circulation group with MRI performed under GA (Group 2, 6 patients; average age, 8.7 years), and completed Fontan circulation group with MRI performed without GA (Group 3, 18 patients; average age, 12.5 years). The patient data were compared with the reference ranges in healthy controls., Results: In comparison with the controls, Group 1 showed normal cardiac output (3.92 ± 0.40 vs. 3.72 ± 0.69 L/min/m², p = 0.30), while Group 3 showed decreased cardiac output (3.24 ± 0.71 vs. 3.96 ± 0.64 L/min/m², p = 0.003). Groups 1 and 3 showed reduced abdominal flow (1.21 ± 0.28 vs. 2.37 ± 0.45 L/min/m², p < 0.001 and 1.89 ± 0.39 vs. 2.64 ± 0.38 L/min/m², p < 0.001, respectively), which was mainly due to the diversion of the cardiac output to the aortopulmonary collaterals in Group 1 and the reduced cardiac output in Group 3. Superior mesenteric and portal venous flows were more severely reduced in Group 3 than in Group 1 (ratios between the flow volumes of the patients and healthy controls was 0.26 and 0.37 in Group 3 and 0.63 and 0.53 in Group 1, respectively). Hepatic arterial flow was decreased in Group 1 (0.11 ± 0.22 vs. 0.34 ± 0.38 L/min/m², p = 0.04) and markedly increased in Group 3 (0.38 ± 0.22 vs. -0.08 ± 0.29 L/min/m², p < 0.0001). Group 2 showed a mixture of the patterns seen in Groups 1 and 3., Conclusion: Fontan circulation is associated with reduced abdominal flow, which can be attributed to reduced cardiac output and portal venous return in completed Fontan circulation, and diversion of the cardiac output to the aortopulmonary collaterals in fenestrated Fontan circulation., Competing Interests: The authors have no potential conflicts of interest to disclose., (Copyright © 2019 The Korean Society of Radiology.)

This technical innovation paper describes a technique for performing cardiac-gated, respiratory-navigated cardiovascular magnetic resonance angiography using an extracellular gadolinium-based contrast agent at 1.5 Tesla (T) with a dual phase bolus injection and slow infusion technique.

Evaluation of adnexal masses in children and adolescents relies on imaging for appropriate diagnosis and management. Pelvic MRI is indicated and adds value for all adnexal masses when surgery is considered or when ultrasound findings are indeterminate. Specifically, features on MR imaging can help distinguish between benign and malignant lesions, which not only influences the decision between surgery and conservative treatment, but also the type of surgery to be performed, including potential use of fertility-sparing approaches with minimally invasive techniques. Larger size, younger age, presentation with precocious puberty or virilization, restricted diffusion in a solid mass, and rapid and strong enhancement of solid components are all features concerning for malignancy. In addition, distinctive MR imaging features of adnexal masses, combined with clinical and laboratory biomarkers, might suggest a specific histological diagnosis.

Background: It is advocated that heterotaxy should be segregated into right or left isomerism according to atrial appendage morphology. However, atrial situs determination is often based on the pattern of associated findings rather than on atrial morphology itself, which can be difficult to define. The objective was to assess how often concordant patterns of isomerism classified by atrial appendage morphology, bronchopulmonary pattern, and splenic status are breached using cardiac magnetic resonance or computed tomography. The secondary objective was to determine the feasibility of defining atrial appendage morphology using cardiac magnetic resonance or computed tomography., Methods and Results: Retrospective review of 114 pediatric patients (median, 2.4 years; range, 1 day-17.9 years) with heterotaxy who underwent cardiac magnetic resonance or computed tomography was performed to evaluate atrial appendage, bronchopulmonary, and visceral organ arrangements. Atrial appendage and splenic anatomy were not definable in 17 of 114 (15%) and 4 of 114 (3.5%) patients, respectively. In the remaining 93 patients, 39% had classic right isomerism (bilateral right atrial appendages, right bronchopulmonary pattern, and asplenia) and 40% had classic left isomerism (bilateral left atrial appendages, left bronchopulmonary pattern, and polysplenia). Classic pattern of isomerism was breached in 20 of 93 (21.5%) patients: 13 (65%) displayed incongruent abdominal situs, 5 (25%) incongruent bronchial situs, 1 (5%) had discrepant appendage morphology, and 1 (5%) incongruent situs at all levels., Conclusions: Atrial appendage morphology is difficult to assess and not always indicative of bronchopulmonary or abdominal situs. Discordance between bronchopulmonary branching, atrial appendage arrangement, and splenic status was identified in >20% patients with heterotaxy. Independent description of each organ system is required when arrangements are disharmonious among different organ systems., (© 2018 American Heart Association, Inc.)