Fast magnetic resonance imaging (MRI) acquisitions with high frame rates can mitigate artefacts caused by physiological motion and bulk patient movement, enabling motion-resolved imaging. This study aims to evaluate the detection performance of triplanar real-time cine fast imaging employing steady-state acquisition (FIESTA), compared to conventional MRI sequences. This retrospective study included 53 children (median age, 1.9 years; range, 1 day–7.1 years) who underwent clinically indicated 1.5-T chest MRI in sedation between January and December 2024. Findings on triplanar real-time cine FIESTA sequences, acquired in up to 1 min, were compared to the findings obtained by dedicated lung sequences (fast spin echo, zero echo time, perfusion imaging) as reference. Detection of extrapulmonary findings (of the chest wall, diaphragm, pleura, mediastinum), lung parenchymal findings (consolidation, atelectasis, bronchial wall thickening, hyperinflation, cysts), and cardiovascular findings by real-time cine FIESTA are presented with descriptive statistics. Real-time MRI provided diagnostic, motion-artefact-free thoracic images in the sedated but freely breathing patients. Diaphragmatic motion abnormalities were detected in one out of one case (100

BACKGROUND:Topsy-turvy heart is an extremely rare congenital heart disease characterized by an axial rotation of the heart owing to a strict supero-inferior orientation of the ventricles and to an elongation of the airways and neck vessels. CASE SUMMARY:We report a case diagnosed prenatally with topsy-turvy heart and aortopulmonary window, with high suspicion of associated airway abnormalities, using combined fetal echocardiography and fetal cardiac magnetic resonance. Immediately after birth, the newborn developed severe respiratory failure requiring mechanical ventilation. DISCUSSION:Multimodality imaging provided comprehensive assessment of the complex intracardiac anatomy and the severe associated vascular and airway anomalies, including severe bronchomalacia and parenchymal lung disease. Despite maximal intensive care support, the clinical condition of the newborn deteriorated rapidly. Considering the clinical course and all imaging findings, the interdisciplinary experts team, based on the available literature, estimated the prognosis as dismal and recommended redirection of care. TAKE HOME MESSAGE:Multimodality imaging used pre- and postnatally in newborns with complex heart disease helps for timely clinical decision making.

The large majority of patients with congenital heart disease (CHD) survive into adulthood in the modern era of surgical advancements. However, patients require life-long follow-up and non-invasive imaging is crucial for diagnostic and therapeutic purposes. Cardiovascular magnetic resonance (CMR) plays a major role in patients with CHD as it is a non-invasive and ionizing radiation free imaging tool which overcomes the technical limitations of echocardiography. 4D flow is an evolving technique for flow quantification allowing improved CMR exam quality and reduced scan time. However, comprehensive postprocessing of hemodynamics in CHD may be demanding. This document addresses the needs for an “ideal” post-processing software for 4D flow in patients with CHD. It provides information on general requirements, velocity encoding, offset and aliasing correction, visualization, segmentation and reconstruction as well as output data. This document aims to support stakeholders in the field to develop solutions for improved 4D flow post-processing and visualization in CHD patients and thus improve the accuracy and efficiency of patient care.

Radial k-space sampling is widely employed in paediatric magnetic resonance imaging (MRI) to mitigate motion and aliasing artefacts. Artificial intelligence (AI)-based image reconstruction has been developed to enhance image quality and accelerate acquisition time. To assess image quality of deep learning (DL)-based denoising image reconstruction of body MRI in children. Children who underwent thoraco-abdominal MRI employing radial k-space filling technique (PROPELLER) with conventional and DL-based image reconstruction between April 2022 and January 2023 were eligible for this retrospective study. Only cases with previous MRI including comparable PROPELLER sequences with conventional image reconstruction were selected. Image quality was compared between DL-reconstructed axial T1-weighted and T2-weighted images and conventionally reconstructed images from the same PROPELLER acquisition. Quantitative image quality was assessed by signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) of the liver and spleen. Qualitative image quality was evaluated by three observers using a 4-point Likert scale and included presence of noise, motion artefact, depiction of peripheral lung vessels and subsegmental bronchi at the lung bases, sharpness of abdominal organ borders, and visibility of liver and spleen vessels. Image quality was compared with the Wilcoxon signed-rank test. Scan time length was compared to prior MRI obtained with conventional image reconstruction. In 21 children (median age 7 years, range 1.5 years to 15.8 years) included, the SNR and CNR of the liver and spleen on T1-weighted and T2-weighted images were significantly higher with DL-reconstruction (P<0.001) than with conventional reconstruction. The DL-reconstructed images showed higher overall image quality, with improved delineation of the peripheral vessels and the subsegmental bronchi in the lung bases, sharper abdominal organ margins and increased visibility of the peripheral vessels in the liver and spleen. Not respiratory-gated DL-reconstructed T1-weighted images demonstrated more pronounced respiratory motion artefacts in comparison to conventional reconstruction (P=0.015), while there was no difference for the respiratory-gated T2-weighted images. The median scan time per slice was reduced from 6.3 s (interquartile range, 4.2 – 7.0 s) to 4.8 s (interquartile range, 4.4 – 4.9 s) for the T1-weighted images and from 5.6 s (interquartile range, 5.4 – 5.9 s) to 4.2 s (interquartile range, 3.9 – 4.8 s) for the T2-weighted images. DL-based denoising image reconstruction of paediatric body MRI sequences employing radial k-space sampling allowed for improved overall image quality at shorter scan times. Respiratory motion artefacts were more pronounced on ungated T1-weighted images.

Chest imaging in children presents unique challenges due to varying requirements across age groups. For chest radiographs, achieving optimal images often involves careful positioning and immobilisation techniques. Antero-posterior projections are easier to obtain in younger children, while lateral decubitus radiographs are sometimes used when expiratory images are difficult to obtain and for free air exclusion. Chest CT protocols should be age-dependent to minimise radiation exposure and motion artefacts. MRI is primarily used in specialised centres to reduce radiation exposure, requiring specific expertise and sedation in younger children. Respiratory distress syndrome is a leading cause of morbidity in preterm neonates, diagnosed through characteristic radiographic findings and a history of prematurity. Bronchopulmonary dysplasia is the most common complication of extreme preterm birth and chronic oxygen therapy; imaging is used for predicting outcomes for the assessment of severe cases. Transient tachypnoea of the newborn and meconium aspiration syndrome are common in term infants, with specific imaging characteristics aiding in their differentiation. Congenital lung malformations present diagnostic and management challenges, with imaging playing a crucial role in diagnosis and surgical planning. Finally, imaging is essential in detecting complications from pneumonia in children, such as empyema and necrotic pneumonia, or in identifying foreign object aspiration. This review summarises current radiology practice of paediatric chest pathologies, aiding in the accurate diagnosis and management of neonatal and congenital pulmonary conditions and pneumonia complications, ultimately improving patient outcomes through precise imaging interpretation and targeted clinical intervention.

Cardiomyopathies are rare diseases in children but are the primary indication for heart transplantation in this age group. Various causes of paediatric cardiomyopathies, ranging from gene-mediated to underlying infection or systemic disease, result in a wide spectrum of clinical presentations and imaging manifestations. Over the years, the classification and terminology of cardiomyopathy have evolved in children and are currently primarily based on the imaging phenotype (dilated, hypertrophic, and restrictive) and then subdivided based on pathogenesis, organ involvement, genetic or familial inheritance pattern, and aetiology. Dilated and hypertrophic cardiomyopathies are more common than non-compaction, restrictive, and arrhythmogenic cardiomyopathies. Echocardiography remains the first-line modality for functional and structural cardiac assessment. However, cardiac magnetic resonance imaging enhances diagnostic accuracy, provides serial cardiac functional evaluation and tissue characterization, and facilitates individual risk stratification and management in patients with heterogeneous phenotypes. This review provides an overview of paediatric cardiomyopathies with a focus on magnetic resonance imaging indications, technique, and key imaging findings that influence management decision-making.

Fetal MRI is increasingly used to investigate fetal lung pathologies, and super-resolution (SR) algorithms could be a powerful clinical tool for this assessment. Our goal was to investigate whether SR reconstructions result in an improved agreement in lung volume measurements determined by different raters, also known as inter-rater reliability. In this single-center retrospective study, fetal lung volumes calculated from both SR reconstructions and the original images were analyzed. Three radiologists manually segmented the fetal lungs and rated the image quality of all images and reconstructions. Fetal lung volumes were calculated, and the coefficient of variation (CV) was determined for each set of images. Bland–Altman plots were generated, and intraclass correlation coefficients (ICCs) were calculated. A one-sided paired Wilcoxon test was used to compare the fetal lung volume CVs, and a two-sided paired t-test was used to compare the lung volumes. The quality ratings were compared using a two-sided paired Wilcoxon test. A total of 98 fetal scans with gestational ages from 19 to 37 weeks were evaluated. There was a significantly lower CV in the lung volumes segmented from the SR reconstructions (p < 0.001), and the ICCs of the reconstructions were higher than those determined from the original images. Bland–Altman plots demonstrated better agreement in the SR reconstruction lung volumes. No significant differences in quality ratings or lung volumes were found. SR reconstructions of the fetal lungs in MRI enabled better inter-rater reliability of fetal lung volume assessment. SR reconstructions of the fetal body, obtained through fetal MRI, can be a valuable tool for improving the inter-rater reliability of fetal lung volume measurements, a crucial clinical biomarker for assessing fetal development and predicting pregnancy outcomes.

Pulmonary complications are known to occur in patients after Fontan palliation. Cardiac MRI is performed in the follow-up of Fontan patients to assess single ventricular function, hemodynamics and potential collateral flow. To date, pulmonary function tests have been used to detect functional lung impairment, but lung MRI has not been integrated into imaging follow-up. In this study, we measured lung-to-background ratio (LBR) on zero echo time (ZTE) MRI sequences in 32 children with Fontan palliation. Patients were divided into 2 groups: LBR > 1.5 and < 1.5 and assessed for associations between LBR and ventricular function, fibrosis, hemodynamics as well as biomarkers, spirometry and previous catheter results. We observed significantly increased extracellular volume (ECV) and decreased tissue inhibitor of metalloproteinase 1 (TIMP-1), soluble suppression of tumorigenicity 2 (sST-2) and a trend towards decreased growth differentiation factor 15 (GDF-15) as well as decreased albumin and prothrombin time values in patients with elevated LBR, whereas the other cardiovascular and pulmonary parameters did not differ significantly. Moreover, patients with LBR > 1.5 had a history of increased pulmonary arterial pressure prior to the Fontan and Glenn procedures and interventional veno-venous collateral embolization. Monitoring lung density with ZTE MRI sequences may be helpful in the diagnostic workup to assess the outcome of patients after Fontan procedure.

Ventricular volumetry using a short-axis stack of two-dimensional (D) cine balanced steady-state free precession (bSSFP) sequences is crucial in any cardiac magnetic resonance imaging (MRI) examination. This task becomes particularly challenging in children due to multiple breath-holds. To assess the diagnostic performance of accelerated 3-RR cine MRI sequences using deep learning reconstruction compared with standard 2-D cine bSSFP sequences. Twenty-nine consecutive patients (mean age 11 ± 5, median 12, range 1–17 years) undergoing cardiac MRI were scanned with a conventional segmented 2-D cine and a deep learning accelerated cine (three heartbeats) acquisition on a 1.5-tesla scanner. Short-axis volumetrics were performed (semi-)automatically in both datasets retrospectively by two experienced readers who visually assessed image quality employing a 4-point grading scale. Scan times and image quality were compared using the Wilcoxon rank-sum test. Volumetrics were assessed with linear regression and Bland–Altman analyses, and measurement agreement with intraclass correlation coefficient (ICC). Mean acquisition time was significantly reduced with the 3-RR deep learning cine compared to the standard cine sequence (45.5 ± 13.8 s vs. 218.3 ± 44.8 s; P < 0.001). No significant differences in biventricular volumetrics were found. Left ventricular (LV) mass was increased in the deep learning cine compared with the standard cine sequence (71.4 ± 33.1 g vs. 69.9 ± 32.5 g; P < 0.05). All volumetric measurements had an excellent agreement with ICC > 0.9 except for ejection fraction (EF) (LVEF 0.81, RVEF 0.73). The image quality of deep learning cine images was decreased for end-diastolic and end-systolic contours, papillary muscles, and valve depiction (2.9 ± 0.5 vs. 3.5 ± 0.4; P < 0.05). Deep learning cine volumetrics did not differ significantly from standard cine results except for LV mass, which was slightly overestimated with deep learning cine. Deep learning cine sequences result in a significant reduction in scan time with only slightly lower image quality.

BackgroundLeft pulmonary artery (LPA) stenting is often required in single ventricle (SV) patients. Due to their close anatomical relationship an LPA stent could potentially compress the left main bronchus (LMB). We assessed the impact of LPA stenting on bronchial size, pulmonary volumes, and lung function in a cohort of SV patients.MethodsForty-nine patients underwent cardiovascular magnetic resonance (CMR) and 36 spirometry 11 (8–15) years after Fontan. All patients were free of respiratory symptoms. LPA stents were inserted in 17 (35%) patients at 8.8 (3.4–12.6) years. Area/shape of the main bronchi (n = 46) and lung volumes (n = 47) were calculated from CMR-ZTE images for each lung and transformed in right-to-left (r/l) ratio and indexed for BSA. The effect of early stent insertion (prior to stage III) was analyzed.ResultsPatients with LPA stent had larger r/l ratio for main bronchus area (p < 0.001) and r/l ratio difference for lung volumes was slightly larger in patients with early stenting. A trend toward a deformation of LMB shape in patients with LPA stent and toward a higher prevalence of abnormal spirometry in patients with early stent implantation was observed.ConclusionsIn this cohort of patients, early insertion of LPA stents seems to relate with smaller LMB sizes and a trend toward smaller left lung volume and higher prevalence of impaired lung function. Whether these findings are caused by the stent or, at least to a certain degree, present prior to the implantation needs to be verified.

In many cardiac diseases, right and left ventricular volumes in systole and diastole are diagnostically and prognostically relevant. Measurements are made by segmentation of the myocardial borders on cardiac magnetic resonance (CMR) images. Automatic detection of myocardial contours is possible by signal thresholding techniques, but must be validated before use in clinical settings. Biventricular volumes were measured in end-diastole (EDVi) and in end-systole (ESVi) both manually and with the MassK application, with signal thresholds at 30%, 50%, and 70%. Stroke volumes (SV) and cardiac indices (CI) were calculated from volumetric measurements and from flow measured in the ascending aorta and the main pulmonary artery, and both methods were compared. Reproducibility of volumetric measurements was tested in 20 patients. Measurements were acquired in 94 patients aged 15 ± 9 years referred for various conditions. EDVi and ESVi of both ventricles were largest with manual segmentation and inversely proportional to the MassK threshold. Manual and k30 SV and CI corresponded best to flow measurements. Interobserver variability was low for all volumes manually and with MassK. In conclusion, manual and 30% threshold-based biventricular volume segmentation agree best with two-dimensional, phantom-corrected phase contrast flow measurements in a young cardiac referral population and are well reproducible.

Childhood onset Takayasu’s arteritis (TA) is a rare entity of large-vessel vasculitis that typically affects the aorta and its main branches and is otherwise observed in women between 20 and 30 years of age [Aeschlimann FA et al. Presentation and Disease Course of Childhood-Onset Versus Adult-Onset Takayasu Arteritis. Arthritis Rheumatol. 2019; 71 (2): 315–323]. The etiology of the pathological inflammatory process that may lead to severe morbidity as a result of organ ischemia and chronic disease course is not well understood so far even though a genetic contribution to disease susceptibility is being increasingly recognized [Di Santo M, et al. Takayasu arteritis in paediatrics. Cardiol Young. 2018; 28 (3): 354–361]. In the early stage, diagnosis is frequently difficult and delayed because of nonspecific symptoms. The classification criteria of the updated EULAR recommendations for childhood vasculitides that include pathological imaging findings of the aorta and main branches achieve a high diagnostic accuracy, see table 1 [Ozen et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides, Ann Rheum Dis 2006; 65: 936–941]. Cardiovascular imaging is essential for diagnosis and disease monitoring with MRI including MR angiography (MRA) as the most relevant imaging modality.

Hemodynamic assessment is an integral part of the diagnosis and management of cardiovascular disease. Four-dimensional cardiovascular magnetic resonance flow imaging (4D Flow CMR) allows comprehensive and accurate assessment of flow in a single acquisition. This consensus paper is an update from the 2015 '4D Flow CMR Consensus Statement'. We elaborate on 4D Flow CMR sequence options and imaging considerations. The document aims to assist centers starting out with 4D Flow CMR of the heart and great vessels with advice on acquisition parameters, post-processing workflows and integration into clinical practice. Furthermore, we define minimum quality assurance and validation standards for clinical centers. We also address the challenges faced in quality assurance and validation in the research setting. We also include a checklist for recommended publication standards, specifically for 4D Flow CMR. Finally, we discuss the current limitations and the future of 4D Flow CMR. This updated consensus paper will further facilitate widespread adoption of 4D Flow CMR in the clinical workflow across the globe and aid consistently high-quality publication standards.

Four-dimensional time-resolved phase-contrast cardiovascular magnetic resonance imaging (4D flow MRI) enables blood flow quantification in multiple vessels, which is crucial for patients with congenital heart disease (CHD). We investigated net flow volumes in the ascending aorta and pulmonary arteries by four different postprocessing software packages for 4D flow MRI in comparison with 2D cine phase-contrast measurements (2D PC). 4D flow and 2D PC datasets of 47 patients with biventricular CHD (median age 16, range 0.6–52 years) were acquired at 1.5 T. Net flow volumes in the ascending aorta, the main, right, and left pulmonary arteries were measured using four different postprocessing software applications and compared to offset-corrected 2D PC data. Reliability of 4D flow postprocessing software was assessed by Bland–Altman analysis and intraclass correlation coefficient (ICC). Linear regression of internal flow controls was calculated. Interobserver reproducibility was evaluated in 25 patients. Correlation and agreement of flow volumes were very good for all software compared to 2D PC (ICC ≥ 0.94; bias ≤ 5

We assessed the impact of bicuspid aortic valve (BAV), aortic stenosis (AS), and regurgitation (AR) on the metrics of left ventricular (LV) remodeling, as measured by electrocardiogram (ECG), transthoracic echocardiography (TTE), and cardiac magnetic resonance (CMR). This retrospective CMR study included 11 patients with both AS and AR (BAV-ASR), 30 with AS (BAV-AS), 28 with AR (BAV-AR), 47 with neither AS nor AR (BAV-no_AS/AR), and 40 with trileaflet aortic valve (TAV-no_AS/AR). CMR analysis included the LV end-diastolic volume index (LVEDVi), mass index (LVMi), and extracellular volume fraction (ECV). The Sokolow-Lyon and Cornell products by ECG and TTE-derived E/e’ were measured. There were no differences in the ECG, TTE, and CMR parameters between BAV-no_AS/AR and TAV-no_AS/AR. However, the presence of aortic valve dysfunction resulted in an elevated Sokolow-Lyon product for BAV-ASR (p = 0.017) and BAV-AR (p = 0.001), as well as increased Cornell product (p = 0.04) and E/e’ (p < 0.001) for BAV-AS compared with BAV-no_AS/AR. LVEDVi and LVMi were elevated in patients with BAV-ASR and BAV-AR compared with those with BAV-no_AS/AR (LVEDVi: 101 ± 29 ml/m2 and 112 ± 32 ml/m2 vs. 74 ± 15 ml/m2, p = 0.005 and p < 0.001, LVMi: 75 ± 7 g/m2 and 64 ± 14 g/m2 vs. 47 ± 9 g/m2, respectively; p < 0.001). There was no difference in ECV between the BAV and TAV-no_AS/AR subgroups. Normally functioning BAV did not result in LV remodeling. However, concomitant AV dysfunction was associated with statistically significant morphological remodeling.

Lung magnetic resonance imaging (MRI) using conventional sequences is limited due to strong signal loss by susceptibility effects of aerated lung. Our aim is to assess lung signal intensity in children on ultrashort echo-time (UTE) and zero echo-time (ZTE) sequences. We hypothesize that lung signal intensity can be correlated to lung physical density. Lung MRI was performed in 17 children with morphologically normal lungs (median age: 4.7 years, range 15 days to 17 years). Both lungs were manually segmented in UTE and ZTE images and the average signal intensities were extracted. Lung-to-background signal ratios (LBR) were compared for both sequences and between both patient groups using non-parametric tests and correlation analysis. Anatomical region-of-interest (ROI) analysis was performed for the normal cohort for assessment of the anteroposterior lung gradient. There was no significant difference between LBR of normal lungs using UTE and ZTE (p < 0.05). Both sequences revealed a LBR age-dependency with a high negative correlation for UTE (Rs =  – 0.77; range 2.98–1.41) and ZTE (Rs =  – 0.82; range 2.66–1.38)). Signal-to-noise (SNR) and contrast-to-noise ratios (CNR) were age-dependent for both sequences. SNR was higher for children up to 2 years old with 3D UTE Cones while for the rest it was higher with 4D ZTE. CNR was similar for both sequences. Posterior lung areas exhibited higher signal intensity compared to anterior ones (UTE 9.4% and ZTE 12% higher), both with high correlation coefficients (R2UTE = 0.94, R2ZTE = 0.97). The ZTE sequence can measure signal intensity similarly to UTE in pediatric patients. Both sequences reveal an age- and gravity-dependency of LBR.

GCA is the most common large vessel vasculitis in the elderly population. In recent years, advanced imaging has changed the way GCA can be diagnosed in many locations. The GCA fast-track clinic approach combined with US examination allows prompt treatment and diagnosis with high certainty. Fast-track clinics have been shown to improve prognosis while being cost effective. However, all diagnostic modalities are highly operator dependent, and in many locations expertise in advanced imaging may not be available. In this paper, we review the current evidence on GCA diagnostics and propose a simple algorithm for diagnosing GCA for use by rheumatologists not working in specialist centres.