Imaging of pediatric cardiac tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

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.

The ductus arteriosus: a review of embryology to intervention.

This article reviews the physiology of the ductus arteriosus, the pathophysiology of the patent ductus arteriosus (PDA), and the role advanced imaging such as computed tomography (CT) and magnetic resonance imaging (MRI) can play in guiding diagnosis and percutaneous or surgical intervention. A PDA can have variable clinical and radiologic presentations and can be important to characterize in patients with vascular rings, aortic maldevelopment and congenital heart disease. An understanding of the PDA and the application of CT and MRI can allow the radiologist to provide key information to physicians who plan to close a PDA or maintain PDA patency in the setting of ductal-dependent congenital heart disease.

Cardiac Computed Tomographic Angiography Evaluation of Right Ventricle-Pulmonary Artery Conduits with Surgical Correlation.

Right ventricle-pulmonary artery (RV-PA) conduits are used in the treatment of certain congenital heart disease (CHD). RV-PA conduit complications might develop over time and require intervention. To evaluate how well cardiac computed tomographic angiography (CCTA) performs compared to transthoracic echocardiography (TTE) in evaluating RV-PA conduit complications by using surgical findings as the reference standard. A retrospective chart review of all patients over a 5-year period who underwent CCTA for RV-PA conduit evaluation was performed. Patient demographics and clinical data were recorded. Preoperative CCTA and TTE findings were compared to the operative findings for concordance or discordance. Forty-one patients were included, 51% females. The complications were conduit stenosis (28.68%), infection (7.17%) and aneurysm/pseudoaneurysm (6.15%). TTE and CCTA were consistently able to visualize focal conduit stenosis (96%). The greatest discrepancy between TTE and CCTA was in evaluating for aneurysm/pseudoaneurysm, where TTE detected only 2/6 (33%) compared to CCTA which detected 6/6 (100%) of the cases. However, TTE was slightly better at detecting conduit infection (3/7, 43%) compared to CCTA (2/7, 29%). Note that 5 out of 7 patients with endocarditis had bovine jugular graft. CCTA and TTE provide similar diagnostic accuracy evaluating certain types of RV-PA conduit complications. However, certain complications were only visualized on CCTA or TTE making both modalities complementary to each other during diagnostic evaluation.

How we approach the diagnosis of a vascular anomaly.

Vascular anomalies represent a diverse group of complex disorders that can cause significant complications, including coagulopathies, pain, and decreased function. The diagnosis of vascular anomalies is often challenging due to heterogeneity of presenting phenotypes and overlapping clinical features with other pediatric conditions. Pediatric hematologists/oncologists (PHO) are uniquely positioned for an essential role in diagnosing, managing, and coordinating the multidisciplinary care required to maximize the quality of life of these patients. Here, we review the diagnostic approach involved in patients with vascular anomalies and utilize cases to highlight the challenges involved, and how PHOs can play a vital part in the care of these patients.

Role of gated cardiac computed tomographic angiography in the evaluation of postsurgical complications after stage I Norwood procedure and its implications on management: a comparative study with two-dimensional echocardiography.

BACKGROUND: The Norwood procedure is the first part of a three-stage surgical palliation for patients with functionally single ventricle anatomy. Complications after the stage I operation are not uncommon. Transthoracic echocardiography (TTE) is traditionally the mainstay for evaluation. OBJECTIVE: The purpose of our study is to compare gated cardiac computed tomographic angiography (CCTA) with TTE when evaluating for postoperative complications after stage I Norwood procedure and to describe management implications. MATERIALS AND METHODS: A retrospective chart review of all patients over a 4-year period who underwent nonelective urgent CCTA for suspected complications related to stage I Norwood procedure was performed. Elective CCTA studies before stage II palliation were excluded. Patient demographics, CCTA and TTE findings, as well as interventions performed, were recorded. RESULTS: Thirty-four patients were included. The mean age at CCTA was 63 days (range: 4-210 days). All patients had a recent TTE with a mean time interval between TTE and CCTA of 2 days. CCTA detected 56 abnormalities in 30 patients, with 23 directly related to postsurgical complications, including shunt-related complications (10/23, 43%), Damus-Kaye-Stansel anastomotic narrowing (2/23, 9%) and neo-aortic arch/branch vessel abnormalities (11/23, 48%). These complications were managed as follows: surgery (9, 39%), catheter-based intervention (7, 30%), medical (4, 17%) and no change in management (3, 13%). TTE did not detect 8/23 (35%) findings found on CCTA, of which 75% were either managed with surgery (4/8, 50%) or catheter-based intervention (2/8, 25%). CONCLUSION: CCTA plays an important role in detecting surgical complications after stage I Norwood procedure and demonstrates additional findings that have direct management implications.

Accuracy of computed tomography angiography and structured reporting of high-risk morphology in anomalous aortic origin of coronary artery: comparison with surgery.

BACKGROUND: Morphological features including interarterial course, intramural course, high ostial location and slit-like ostium are presumed risk factors for sudden cardiac death in children with anomalous aortic origin of the coronary artery (AAOCA). To facilitate clinical risk stratification, the diagnostic accuracy of CT angiography for individual risk factors in the setting of AAOCA must be established. OBJECTIVE: We assessed diagnostic accuracy of standardized CT angiography interpretation for morphological characteristics that might determine risk in children with AAOCA by comparing them to surgical findings. MATERIALS AND METHODS: We created a standardized protocol for CT angiography of AAOCA and retrospectively evaluated diagnostic performance in 25 consecutive surgical patients. Relevant morphological variables in AAOCA were assessed by three independent blinded readers, with surgery as the reference standard. We used Cohen kappa coefficients and accuracies to assess agreement between readers and surgical findings, and we calculated intraclass correlation coefficients to compare length of the intramural course. RESULTS: CT angiography correctly identified AAOCA in all patients. For the three readers, accuracies for detecting ostial stenosis were 84%, 94% and 96%; for high ostial origin, accuracies were 76%, 78% 82%; for intramurality using the peri-coronary fat sign, accuracies were 98%, 96% and 92%; and for intramurality using oval shape of coronary artery, accuracies were 98%, 94% and 92%. The intraclass correlation coefficients (ICCs) for predicting intramural length among the three readers were 0.67, 0.75 and 0.81 using peri-coronary fat, and 0.69, 0.50 and 0.81 using oval shape, respectively. CONCLUSION: CT angiography reliably identified AAOCA in all children and detected the presence of intramurality with high accuracy.

Diagnostic sensitivity and specificity of CT angiography for renal artery stenosis in children.

BACKGROUND: Children with suspected renal artery stenosis (RAS) are screened with renal Doppler ultrasonography or computed tomography (CT) angiography/magnetic resonance (MR) angiography depending on institutional preference. CT angiography produces images with superior resolution, allowing higher quality multiplanar two-dimensional reformats and three-dimensional reconstructions. However, there is a paucity of data in the literature regarding the utility and diagnostic performance of renal CT angiography in pediatric RAS. OBJECTIVE: The objective of this study is to retrospectively review our experience with renal CT angiography in the diagnosis of pediatric RAS relative to digital subtraction angiography (DSA) as the reference standard. MATERIALS AND METHODS: All patients 0-18 years of age who underwent CT angiography for evaluation of RAS as a cause of hypertension between January 2012 and May 2019 were identified for the study. A total of 131 patients were identified, 23 of whom had DSA correlation. RESULTS: Twenty-three patients (17 boys, 6 girls) with a mean age of 6 years 3 months (range: 3 months to 14 years 7 months) were included in this study. Of the 59 renal arteries studied by DSA, 22 were abnormal on CT angiography and 20 were abnormal on DSA. Of the 59 renal arteries, CT angiography was true positive in 18 and true negative in 35. The sensitivity and specificity of CT angiography for RAS diagnosis were 90.0% and 89.7%, respectively. CT angiography identified all cases of main RAS. CONCLUSION: Renal CT angiography has a high sensitivity and specificity for pediatric RAS diagnosis in patients referred for DSA.

Pediatric Hepatoblastoma, Hepatocellular Carcinoma, and Other Hepatic Neoplasms: Consensus Imaging Recommendations from American College of Radiology Pediatric Liver Reporting and Data System (LI-RADS) Working Group.

Appropriate imaging is imperative in evaluating children with a primary hepatic malignancy such as hepatoblastoma or hepatocellular carcinoma. For use in the adult patient population, the American College of Radiology created the Liver Imaging Reporting and Data System (LI-RADS) to provide consistent terminology and to improve imaging interpretation. At present, no similar consensus exists to guide imaging and interpretation of pediatric patients at risk for developing a liver neoplasm or how best to evaluate a pediatric patient with a known liver neoplasm. Therefore, a new Pediatric Working Group within American College of Radiology LI-RADS was created to provide consensus for imaging recommendations and interpretation of pediatric liver neoplasms. The article was drafted based on the most up-to-date existing information as interpreted by imaging experts comprising the Pediatric LI-RADS Working Group. Guidance is provided regarding appropriate imaging modalities and protocols, as well as imaging interpretation and reporting, with the goals to improve imaging quality, to decrease image interpretation errors, to enhance communication with referrers, and to advance patient care. An expanded version of this document that includes broader background information on pediatric hepatocellular carcinoma and rationale for recommendations can be found in Appendix E1 (online).

Correlation of ductus arteriosus length and morphology between computed tomographic angiography and catheter angiography and their relation to ductal stent length.

BACKGROUND: Patent ductus arteriosus (PDA) stent placement in infants with ductal-dependent pulmonary blood flow is being increasingly used in clinical practice. OBJECTIVE: To correlate computed tomographic (CT) angiography morphology and length of the PDA with catheter angiography and its relation to eventual PDA stent length. MATERIALS AND METHODS: We retrospectively identified all pediatric patients who underwent PDA stenting at our institute from 2004 to 2018. We included children who had CT angiography prior to stenting. PDA length was measured by a radiologist blinded to the catheter angiography data, using Syngo-via post-processing software (Siemens, Erlangen, Germany). Vessel centerline technique was used. We measured the actual length of the duct as well as straight length between aortic and pulmonary ends. PDA morphology tortuosity index was classified as straight (Type I), mildly tortuous with 1 turn (Type II) and tortuous with >1 turn (Type III), and the PDA origin was noted. The PDA was also measured and morphology classified on catheter angiography by an interventional cardiologist blinded to the CT angiography findings. We compared the CT angiography and catheter angiography lengths, straight lengths and stent length using scatter plots and intraclass correlation coefficient (ICC). RESULTS: A total of 83 children who had PDA stenting were identified, of whom 17 had prior CT angiography. Fifteen of these were neonates. There was agreement between CT angiography and catheter angiography regarding the PDA morphology tortuosity index in 94% of cases and PDA origin in 100% of cases. There was moderate agreement between CT angiography and catheter angiography actual and straight PDA lengths, with ICC coefficients of 0.65 and 0.68, respectively. There was moderate agreement between CT angiography actual length, CT angiography straight length, catheter angiography actual length and eventual stented PDA length, with ICCs of 0.57, 0.67 and 0.73, respectively. There was poor agreement between catheter angiography straight length and eventual stented PDA length, with an ICC of 0.39. CONCLUSION: PDA length and morphology description on CT angiography correlates well with catheter angiography and can be a reliable guide for the interventional cardiologist in decision-making regarding appropriate choice of PDA stent length.

Value of emergent pediatric cardiac computed tomographic angiography service: initial experience at a large children's hospital.

BACKGROUND: Demand for pediatric cardiac computed tomography (CT) angiography is increasing due to recent advances that reduce the need for sedation and radiation exposure while enhancing diagnostic accuracy. This has resulted in the increasing use of cardiac CT angiography emergently during weekends and after hours. The unexpected demand for these services can be challenging, as most hospitals are not staffed to provide 24/7 pediatric cardiovascular imaging. OBJECTIVE: To describe a large single-center experience of providing emergent cardiac CT angiography services in children. MATERIALS AND METHODS: We identified all patients who underwent after--hours weekday and weekend emergent cardiac CT angiography between January 2017 and August 2018. Cardiac CT angiography in the settings of congenital heart disease and coronary imaging were included. Data collected included day and time of cardiac CT angiography, patient age, referral unit, indication, surgical history, need for sedation, need for surgery, intervention and/or change in medical management based on the cardiac CT angiography. RESULTS: Forty-seven studies were identified, 26 (55%) of which were performed on a weekend or holiday and 21 (45%) after 5 p.m. on a weekday. Based on cardiac CT angiography findings, 20 (43%) patients underwent either surgery or an interventional procedure, and 9 (19%) had a change in medical management. The time between cardiac CT angiography and the related surgery/intervention ranged from 0 to 29 days with a median of 3.5 days. CONCLUSION: Emergent pediatric cardiac CT angiography is a valuable service. Larger multi-institutional studies with standardized referral and utilization patterns are needed to determine if outcomes are affected by this service, which in turn will influence hospital staffing patterns for emergent imaging.

Guidance Document for Hepatic Hemangioma (Infantile and Congenital) Evaluation and Monitoring.

OBJECTIVE: To define the types of hepatic hemangiomas using the updated International Society for the Study of Vascular Anomalies classification and to create a set of guidelines for their diagnostic evaluation and monitoring. STUDY DESIGN: We used a rigorous, transparent consensus protocol defined by an approved methodology, with input from multiple pediatric experts in vascular anomalies from hematology-oncology, surgery, pathology, radiology, and gastroenterology. RESULTS: In the first section, we define the subtypes of hepatic hemangiomas based on the clinical course, histology, and radiologic characteristics. We recommend against using the term "hemangioma" for any vascular malformations affecting the liver or any hypervascular tumors that are not characterized by the approved definitions. We recommend against using the term "hemangioendothelioma" for infantile or congenital hemangioma. The following 2 sections dedicated to infantile hepatic hemangioma and to congenital hepatic hemangioma individually describe these subtypes in further detail, including complications to be considered during monitoring and respectively recommended screening evaluations. CONCLUSIONS: Although institutional variations may exist for specific clinical details, a clear understanding of the diagnosis of hepatic hemangiomas affecting children and the possible complications that require screening during the monitoring period should be standard. As children with hepatic hemangiomas are managed by different medical and surgical specialties, we offer an expert opinion multidisciplinary consensus based on current literature and on data extracted from the liver hemangioma registry.

Magnetic resonance imaging features of common focal liver lesions in children.

Magnetic resonance imaging (MRI) is commonly used to characterize focal liver masses in the pediatric population. MRI is the preferred modality because of its superior contrast resolution and utility for obtaining functional sequences such as diffusion-weighted imaging (DWI). MR exams performed with a hepatocyte-specific gadolinium-based contrast agent can characterize focal liver lesions, which helps in differentiating a common benign entity such as focal nodular hyperplasia from other liver pathology when the background liver is normal. The most common benign focal lesion is a hemangioma, and metastases followed by hepatoblastoma are the most common malignant lesions. This article can help radiologists become familiar with the pre- and post-contrast imaging features of common pediatric liver masses.

Undifferentiated Embryonal Sarcoma of the Liver (UESL): A Single-Center Experience and Review of the Literature.

Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive pediatric malignancy. The purpose of this study was to review the clinical, radiologic, and pathologic features and outcome of children with UESL at our institution, in the United Network of Organ Sharing database and to review the existing literature to define the state of the art for children with UESL. Six children were diagnosed with UESL at the Texas Children's Cancer Center between 1993 and 2014, 12 children underwent liver transplantation registered in the United Network of Organ Sharing database, and 198 children with UESL were described in 23 case series during 1978 to 2014. Patients were treated with multimodal treatment approaches including primary surgical resection, neoadjuvant and/or adjuvant chemotherapy, and liver transplantation resulting in overall survival reported between 20% and 100% with significant improvement over the recent years. We show that complete tumor removal remains the key element of treatment and our single-institutional experience and data in the published literature suggest that combination chemotherapy with ifosfamide and doxorubicin to facilitate complete surgical resection is an effective approach to cure children with UESL.

Intraoperative ultrasound for liver tumor resection in children.

BACKGROUND: Primary hepatic neoplasms in children are rare tumors. All malignant and medically refractive benign primary pediatric liver tumors ultimately require surgical resection for cure. Accurate preoperative imaging including multidetector helical computerized tomography or magnetic resonance imaging (MRI) is necessary to determine resectability. In the literature intraoperative ultrasound (IOUS) has proven to be a vital adjunct to liver surgery in adults, but this is not well established in children. MATERIALS AND METHODS: Between April 2003 and November 2014, children (<18-y-old) with a primary liver neoplasm, preoperatively evaluated with multidetector helical computerized tomography or MRI, who had IOUS used at the time of surgery were retrospectively reviewed. RESULTS: Preoperative evaluation with high-resolution MRI and IOUS were discordant in 4 of 19 patients (21%). In one case, right hepatic vein involvement was not accurately assessed with MRI. Two cases showed tumor involvement in segment IV by MRI; however, IOUS revealed no medial segment involvement. The final patient had a large (>5 cm), solitary hepatic adenoma on MRI, but IOUS in this case revealed diffuse adenomatosis. The operative management was altered in three of these cases. CONCLUSIONS: Although MRI can provide a detailed view of the hepatic anatomy and is an invaluable tool for preoperative planning for the pediatric patient with a primary liver neoplasm, IOUS may provide further and more up to date delineation of tumor extent and should be considered a crucial element in operative planning for hepatectomy in children.

Myocardial bridges in a pediatric population: Outcomes following a standardized approach.

OBJECTIVE: To describe clinical, functional, surgical, and outcomes data in pediatric patients with a myocardial bridge (MB) evaluated and managed following a standardized approach. METHODS: Prospective observational study included patients evaluated in the Coronary Artery Anomalies Program. Anatomy was determined by computed tomography angiography, myocardial perfusion by stress perfusion imaging, and coronary hemodynamic assessment by cardiac catheterization. RESULTS: In total, 39 of 42 patients with a complete evaluation for MB were included (December 2012 to June 2022) at a median age of 14.1 years (interquartile range, 12.2-16.4). Sudden cardiac arrest occurred in 3 of 39 (8%), exertional symptoms in 14 (36%), and no/nonspecific symptoms in 7 (18%) patients. Exercise stress test was abnormal in 3 of 34 (9%), stress perfusion imaging in 8 of 34 (24%), and resting instantaneous wave-free ratio ≤0.89 or diastolic dobutamine fractional flow reserve ≤0.80 in 11 of 21 (52%) patients. As a result, 15 of 39 (38%) patients were determined to have hemodynamically significant MB, 1 of 15 patients started beta-blocker, and 14 of 15 were referred for surgery. Myotomy (n = 11) and coronary bypass (n = 1) were performed successfully, resulting in improved symptoms and stress testing results. One patient required pericardiocentesis postoperatively, and all were discharged without other complications. At median follow-up time of 2.9 (1.8-5.8) years, all (except 2 pending surgery) were doing well without exercise restriction. CONCLUSIONS: Pediatric patients with MB can present with myocardial ischemia and sudden cardiac arrest. Provocative stress test and intracoronary hemodynamic tests helped risk-stratify symptomatic patients with MB and concern for ischemia. Surgical repair was safe and effective in mitigating exertional symptoms and stress test results, allowing patients to return to exercise without restriction.

Pediatric Hepatocellular Adenomas: What Is Known and What Is New?

Current understanding and classification of pediatric hepatocellular adenomas (HCA) are largely based on adult data. HCAs are rare in children and, unlike in adults, are often seen in the context of syndromes or abnormal background liver. Attempts to apply the adult classification to pediatric tumors have led to several "unclassifiable" lesions. Although typically considered benign, few can show atypical features and those with beta-catenin mutations have a risk for malignant transformation. Small lesions can be monitored while larger (>5.0 cm) lesions are excised due to symptoms or risk of bleeding/rupture, etc. Management depends on gender, age, underlying liver disease, multifocality, size of lesion, histologic subtype and presence of mutation, if any. In this review, we summarize the data on pediatric HCAs and highlight our experience with their diagnosis and management.

Investigating the effectiveness of a smart mental health intervention (inMind) for stress reduction during pharmacological treatment for mild to moderate major depressive disorders: Study protocol for a randomized control trial.

Background: Although psychological interventions for stress relief, such as cognitive behavioral therapy (CBT) and mindfulness-based stress reduction (MBSR), have been developed, they have not been widely used in treating depression. The use of mobile devices can increase the possibility of actual use by integrating interventions and reducing the difficulty and cost burden of treatment application. This study aims to determine whether "inMind," an integrated mobile application for stress reduction, developed for the general population, decreases stress for patients with mild to moderate major depressive disorder during the pharmacological treatment period. Methods: This study is a single-blind, multicenter, randomized, controlled crossover trial. The App, developed in Republic of Korea, provides integrated interventions for stress reduction for the general population through three modules based on mindfulness-based stress reduction, cognitive behavior therapy, and relaxation sounds that are known to be effective in stress reduction ("meditation," "cognitive approach," and "relaxation sounds," respectively). Participants (n = 215) recruited via medical practitioner referral will be randomized to an App first group (fAPP) or a wait list crossover group (dAPP). The study will be conducted over 8 weeks; the fAPP group will use the App for the first 4 weeks and the dAPP group for the next 4 weeks. During all study periods, participants will receive their usual pharmacological treatment. The Depression Anxiety Stress Scale-21 is the primary outcome measure. The analysis will employ repeated measurements using a mixed-model approach. Discussion: The App can potentially be an important addition to depression treatment because of its applicability and the comprehensive nature of the interventions that covers diverse stress-relieving models. Clinical trial registration: https://clinicaltrials.gov/ct2/show/NCT05312203, identifier 2021GR0585.

Detection of anomalous aortic origin of a coronary artery (AAOCA) by echocardiogram: When does computed tomographic angiography add value?

BACKGROUND AND OBJECTIVE: There is limited literature comparing TTE and CCTA in children with suspected AAOCA. To determine the distribution of various coronary anomalies comparing TTE and CCTA data, and define the added value advanced imaging brings in clinical decision-making. MATERIALS AND METHODS: Retrospective review of data was obtained in patients aged 0-18 years who underwent TTE and CCTA for suspected AAOCA. Patient demographics, CCTA and TTE findings, and interventions performed were recorded. RESULTS: 100 consecutive patients were included (60% male), mean age 11 years (7 days-18 years old). In 93 patients, CCTA detected 94 anomalous coronaries. Definitive coronary abnormality was reported on TTE in 77 patients; 76 of which were confirmed by CCTA, 1 patient was found to have a normal variant. Suspected anomalous origin was reported in 16 patients on TTE, 13 of which were abnormal on CCTA. The coronary origin was not seen on TTE in 6 patients; of these, 3 had AAOCA on CCTA and 3 had hypoplastic RCA with left dominant system. Only 1 patient who had a normal TTE was found to have AAOCA on CCTA. CCTA was better than TTE in defining ostial characteristics and the course of the anomalous coronary artery, and detecting myocardial bridge. CONCLUSIONS: CCTA adds value in diagnosing AAOCA when the coronary origins are not well assessed or suspected anomalous origin is suggested on TTE. In addition, when a confident definitive diagnosis of AAOCA is reported on TTE, CCTA demonstrates better performance in determining additional features of AAOCA.