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.

Quantification of lymphatic burden in patients with Fontan circulation by T2 MR lymphangiography and associations with adverse Fontan status.

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.

Innocent until proven guilty? Longstanding atrial ectopy preceding cardiac rhabdomyoma diagnosis in tuberous sclerosis complex: a case report.

BACKGROUND: Cardiac rhabdomyoma are the most common cardiac tumour in childhood and are associated with tuberous sclerosis complex (TSC) up to 96% of infant cases. They classically manifest in the foetal and neonatal period, undergo spontaneous regression in the first years of life and are associated with arrhythmia in part due to interruption of normal conduction pathways by the tumour. CASE SUMMARY: We present a case of a 3-year-old boy with a long-standing history of atrial ectopy who was incidentally found to be in atrial flutter due to a new, rapidly growing cardiac rhabdomyoma impacting ventricular function. The boy was later confirmed with further investigation and TSC1 gene test to have TSC. DISCUSSION: Cardiac Rhabdomyoma does not always present in the infantile period. Any ongoing or new cardiac concern in patient with TSC, even if seemingly minor, should warrant more frequent cardiac evaluation and investigation.

Semiquantitative characterization of dynamic magnetic resonance perfusion of the liver in pediatric Fontan patients.

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.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

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.

Anterior Translocation of the Right Pulmonary Artery to Relieve Bronchial Compression in Truncus Arteriosus With Interrupted Aortic Arch.

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.

Adverse fibrosis remodeling and aortopulmonary collateral flow are associated with poor Fontan outcomes.

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/m2 vs 93 ml/m2, 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/m2 vs 120 ml/m2, 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/m2 vs 118 ml/m2, 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.

Abdominal Imaging of Children and Young Adults With Fontan Circulation: Pathophysiology and Surveillance.

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.

3D Modeling and Printing in Congenital Heart Surgery: Entering the Stage of Maturation.

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.

Diagnostic Performance of a Sonographic Volume and Solid Vascular Tissue Score (VSVTS) for Preoperative Risk Assessment of Pediatric and Adolescent Adnexal Masses.

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.

Preoperative Risk Stratification of Adnexal Masses in the Pediatric and Adolescent Population: Evaluating the Decision Tree System.

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.

Magnetic resonance imaging of pediatric adnexal masses and mimics.

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.

Diagnosis of secondary pulmonary lymphangiectasia in congenital heart disease: a novel role for chest ultrasound and prognostic implications.

BACKGROUND: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction. OBJECTIVES: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia. MATERIALS AND METHODS: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Thirteen children had pulmonary venous obstruction (62% male; median age: 17 days old, range: 1-430 days old) and 13 children did not have obstruction (62% male; median age: 72 days old, range: 4-333 days old). US features of secondary pulmonary lymphangiectasia were documented and diagnostic performance was determined. Clinical course of patients with secondary pulmonary lymphangiectasia was reviewed. RESULTS: Eleven of 13 (84.6%) patients in the obstructed group had a clinical and/or biopsy diagnosis of secondary pulmonary lymphangiectasia. Statistically significant chest US criteria for diagnosis were presence of irregular lung surface (likelihood ratio [LR] 6.8, 95% confidence interval [CI] 1.9-25.1), subpleural cystic appearing structures (LR 3.6, 95% CI 1.2-10.7), and combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9, 95% CI 1.6-75.0). Seven of 11 (63.6%) patients with secondary pulmonary lymphangiectasia died during follow-up, the majority due to cardiopulmonary failure or complications. CONCLUSION: Chest US is an accurate and reproducible bedside method for diagnosing secondary pulmonary lymphangiectasia in patients with pulmonary venous obstruction. These patients may have worse prognoses.

Incidental splenic nodules found on MR imaging done for assessment of iron overload in children.

BACKGROUND: MR imaging is used to assess iron overload in patients with hemoglobinopathies and in those who have undergone multiple blood transfusions. Sometimes splenic nodules are found incidentally on these examinations and this may cause diagnostic uncertainty. OBJECTIVE: To determine the prevalence, imaging characteristics and evolution of splenic nodules found on MR imaging for iron overload evaluation. MATERIALS AND METHODS: Retrospective review of all MR imaging examinations performed for iron overload assessment from 2005 to 2015 in a tertiary pediatric hospital. The presence of focal splenic nodules including number, size, signal characteristics and changes on follow-up MR imaging were recorded. Relevant patient clinical information including underlying hematological disease was also documented. RESULTS: A total of 318 patients had MR imaging for iron overload assessment. Of these, 25 (8%) had at least one incidental splenic nodule. Sickle cell disease was present in 22 patients (88%) and thalassemia in 3 (12%). On intermediate-weighted spin-echo images, the nodules had high signal intensity compared to the remainder of the spleen in 23 patients (92%) and low signal intensity in the remaining 2 (8%). In all patients (100%) the nodules showed progressive loss of signal intensity with increasing echo time values. Follow-up MR imaging was performed in 20 (80%) patients, which showed an increase in the size of the splenic nodules in 7 patients (35%) stability in 11 (55%) and a decrease in size in 2 (10%). CONCLUSION: It is not uncommon to find splenic nodules during MR evaluation of iron overload. In patients with sickle cell disease, most of these nodules are thought to represent preserved splenic tissue and appear hyperintense compared to the remainder of the spleen. They frequently remain stable on follow-up imaging, although about a third of them may show growth. Awareness of these nodules is important to avoid concern for potential malignancy and unnecessary investigations.