RPS 312 - Congenital and acquired paediatric cardiothoracic radiology

Author Block: P. Langguth, M. Huhndorf, H. Seoudy, O. Jansen, A. Uebing, I. Voges; Kiel/DE
Purpose: Patients with Fontan circulation after single ventricle palliation are at increased risk of pathological alterations in the cervicothoracic lymphatic drainage system, including chylothorax and plastic bronchitis. Visualization and classification of these lymphatic pathways may provide prognostic information and help guide clinical management. This study aimed to demonstrate that a 3D mDixon steady-state MR-angiography sequence, commonly used for cardiovascular assessment, can simultaneously visualize lymphatic structures with high diagnostic quality.
Methods or Background: We retrospectively analyzed MR examinations of 88 pediatric and young adult Fontan patients. The 3D mDixon steady-state MR-angiography was assessed for image quality and diagnostic yield regarding cervicothoracic lymphatic pathways. Ventricular volumes, ejection fraction, and mass were quantified from cine imaging. Lymphatic pathways were classified according to an established four-tier system (Types 1–4).
Results or Findings: Image quality of the lymphatic pathways was rated “very good” or “good” in over 90% of cases. Six patients presented with lymphatic complications; five (83.3%) showed higher-grade pathways (Type 3 or 4). Among 88 patients, the distribution was: 31.8% Type 1, 18.2% Type 2, 39.8% Type 3, and 9.1% Type 4. No significant associations were observed between lymphatic type and single ventricle morphology, age at Fontan completion, protein levels, NT-proBNP, or central venous pressure. Likewise, no correlation was found between lymphatic type and ventricular parameters (volumes, ejection fraction, mass).
Conclusion: 3D mDixon steady-state MR-angiography enables high-quality, simultaneous visualization of cardiovascular and cervicothoracic lymphatic anatomy in Fontan patients without additional imaging sequences. Patients with lymphatic complications tended to present with higher lymphatic types, suggesting a role for this technique in risk stratification and follow-up.
Limitations: This was a retrospective study. T2-weighted lymphangiography, the current reference standard for lymphatic imaging, was not systematically performed for comparison. Abdominal lymphatic abnormalities were not evaluated.
Funding for this study: No fundings
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: Christian-Albrechts University

Author Block: S. Azhe, Z. Zhou, Y-K. Guo, L-y. Wen, X. Deng; Chengdu/CN
Purpose: To investigate the distribution patterns of late gadolinium enhancement (LGE) in the chronic phase of Kawasaki disease (KD) and its relationship with coronary artery lesions and myocardial strain.
Methods or Background: In this retrospective, single-center study, 185 KD patients who underwent cardiac magnetic resonance (CMR) during the chronic phase were enrolled. They were divided into an LGE-positive group (n=25) and an LGE-negative group (n=160). CMR image analysis was performed using commercial software.
Results or Findings: Left ventricular (LV) LGE was present in 25 (13.5%) patients, involving 15.07 ± 0.12% of the LV mass. The LGE distribution was as follows: longitudinally, the basal (68%) and mid-ventricular (68%) segments were most frequently affected; circumferentially, the lateral (68%) and anterior (44%) walls were predominant; radially, subendocardial (24%), transmural (32%), and subepicardial (32%) patterns were observed. Coronary artery stenosis (OR: 5.48) and a history of thrombosis (OR: 3.87) were identified as independent risk factors for LGE (all P < 0.05). The LGE location matched the perfusion territory of stenotic or thrombotic coronary arteries in 19 patients, while 6 LGE-positive patients had no coronary artery lesions. Myocardial strain (global and segmental) was significantly impaired in the LGE+ group across all directions (longitudinal, radial, circumferential) compared to the LGE- group (all p < 0.05).
Conclusion: In KD patients, LGE is primarily located in the basal and mid lateral walls of the left ventricle. Coronary artery stenosis and thrombosis history are independent risk factors for LGE. The presence of LGE is associated with subclinical left ventricular dysfunction.
Limitations: This was a single-center, single-vendor, observational study; there was potential for selection bias, and the patient population may not fully represent the global population with KD.
Funding for this study: This study is supported by the National Natural Science Foundation of China (82102020, 82471970) and the Special Project for the Central Government to Guide the Development of Local Science and Technology in Sichuan Province(2023ZYD0100).
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: This study protocol was approved by the Institutional Review Board at our University Hospital (No. 2023095)

Author Block: T. Banaz, F. Köse, M. F. Kazanbaş, B. Tütüncüoğlu, M. Kadıoğlu, S. Ozkök; Istanbul/TR
Purpose: Dilated cardiomyopathy (DCM) is a major cause of heart failure and transplantation in children. While genetic testing can aid diagnosis, it is often costly, slow, and inconclusive. Viral myocarditis is a common cause of pediatric DCM, but distinguishing it from genetic forms is difficult due to overlapping clinical and imaging features. This study aims to investigate whether magnetic resonance imaging (MRI) signal characteristics of lymphoid organs can non-invasively distinguish between viral and genetic DCM.
Methods or Background: This retrospective study included 131 pediatric DCM patients (<18 years) treated between January 2022 and December 2024. Thirty-one patients with confirmed genetic (n=13) or viral (n=18) etiology were analyzed. Exclusion criteria were congenital heart disease, autoimmune disease, malignancy, or missing data. Diagnoses were based on molecular/genetic testing or pathogen detection.
Myocarditis diagnoses were based on clinical, laboratory, ECG, echocardiographic, cardiac MRI findings. No biopsies were performed. Patients underwent cardiac MRI with T1&T2 mapping. Quantitative values were extracted from the myocardium, spleen, and thymus. Median and interquartile ranges were calculated, intergroup comparisons were performed using independent t-tests.
Results or Findings: No significant differences were observed in conventional cardiac MRI parameters, including biventricular volume indices, ejection fractions, native T1&T2 mapping, or myocardial contrast enhancement percentage (p>0.05). Native T1 values were significantly higher in the genetic group in the thymus (p = 0.033) and in the spleen (p = 0.049). Splenic T2 mapping values were higher in the genetic group (p = 0.048).
Conclusion: Conventional MRI may not clearly differentiate genetic and viral DCM. Genetic DCM is linked to prolonged inflammation, with elevated T1 values in thymus and spleen indicating fibrosis or ongoing inflammation, and T2 values in spleen indicating subclinical edema.
Limitations: Retrospective design, small sample size, age/immune/technical variability in lymphoid MRI signals
Funding for this study: No funding was received for this study.
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: This study was approved by the Ethics Committee of Istanbul Medipol University, Istanbul/Turkey.

Author Block: H. Witowicz, W. Grajkowska, E. Jurkiewicz; Warsaw/PL
Purpose: The purpose of our study was to assess very rare genetic disease - Generalised Arterial Calcifications of Infants (GACI) and to find possible differences in management with other rare condition - idiopatic artery calcifications from our Institure.
Methods or Background: We retrospectively assessed four new patients by genetic background, clinical symptoms, diagnostic process and treatment responce. Every patient had CT.

For the purpose of our study paper review was performed. From 1899 were published aproximatelly 256 cases of GACI.
Results or Findings: In the first case the patient was admitted to the hospital at the right time- in the first day after birth, which gave doctors enough time to make correct diagnosis(CT reveled typical arterial calcifications, ENPP1 mutation) and the right treatment (bisphosphonates)was started with almost total regression of the calcifications in 3 months.

In the second case the circumstances of addmission was different-the infant was redirected to our Institute too late,three weeks after birth. In this case CT revealed advanced large and middle-size artierial calcifications. No gene analisis were performed. No bisphosphonate treatment was inclueded. Patient died of sudden cardiac arrest.The right diagnosis (GACI) was made postmortem. This case shoud be an important lesson how fast calcifications could develop without treatment and how crucial is early diagnosis with the the right treatment.

In the third and fourth cases of idiopathic artery calcifications no genetic alterations(typical for GACI) were revealed. Calcifications receded by itself without bisphosphonates treatment.
Conclusion: Our study higlights the importance of early diagnostic of GACI and early start of the treatment with bisphosphonates. Time is crucial in this type of diseases. Every day/week of waiting could worsening the disease significantly with plenty of possible complications. That why time is the most valuable resource.
Limitations: N/A
Funding for this study: N/A
Has your study been approved by an ethics committee? Not applicable
Ethics committee - additional information:

Author Block: L. Deng, Z. Jia; Yulin/CN
Purpose: Delayed diagnosis of paediatric refractory Mycoplasma pneumoniae pneumonia (RMPP) leads to severe sequelae, as current single-modality predictors lack accuracy. We aimed to develop and validate a synergistic, multi-modal AI framework, integrating heterogeneous clinical data with Swin Transformer-based CT analysis, for high-fidelity and clinically trustworthy early risk stratification
Methods or Background: This retrospective study of 466 children with MPP fused four data pillars: clinical/laboratory data, quantified radiologist knowledge, automated objective CT biomarkers, and deep sub-visual features from a Swin Transformer. An XGBoost model integrated these features, with SHAP analysis ensuring decision transparency. The framework was validated on an independent test set.
Results or Findings: On an independent test set, the fusion model showed excellent discrimination (AUC: 0.92; 95% CI: 0.88-0.96) with high sensitivity (0.85) and specificity (0.88), enabling robust identification of high-risk patients. Ablation studies confirmed the multi-modal approach significantly outperformed single-modality baselines. SHAP analysis revealed a synergy between known clinical factors (e.g., LDH, CRP) and novel, influential sub-visual CT patterns from the Swin Transformer, confirming the data fusion's synergistic gain.
Conclusion: This study’s core contribution is quantitatively confirming a significant synergistic effect among four heterogeneous data dimensions via ablation study, establishing the necessity of multi-modal fusion for RMPP prediction. The proposed explainability framework transforms opaque model decision-making into a transparent, auditable evidence pathway, providing a reliable basis for clinical decisions. This work offers an empirical foundation for a safer, more precise human-machine collaborative model, aiming to improve outcomes through timely intervention.
Limitations: Not applicable
Funding for this study: Yulin City Youth Talent Support Program
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: Yulin Hospital,The First Affiliated Hospital of Xi'an Jiaotong University:2025048

Author Block: D. Velan, F. Abubacker Sulaiman, R. Praveenkumar, J. Lydia, M. Nivitha, K. Saravanan; Chennai/IN
Purpose: To explore the feasibility and diagnostic potential of 4D Flow MRI in evaluating dynamic hemodynamics of fetal and neonatal circulation, including intracardiac flow patterns, shunt physiology, and cerebroplacental perfusion.

The study aims to establish normative flow parameters and identify early markers of circulatory compromise in high-risk pregnancies.
Methods or Background: Conventional Doppler ultrasound provides limited two-dimensional assessment of blood flow velocity and direction, lacking volumetric and temporal resolution. 4D Flow MRI, with its time-resolved three-dimensional velocity encoding, enables comprehensive quantification of flow vectors, wall shear stress, and vorticity throughout the cardiac cycle. In this prospective study, 40 fetuses (28–38 weeks) and 20 neonates underwent 4D Flow MRI using a prototype highly accelerated phase-contrast sequence with retrospective gating.


Post-processing included vessel segmentation, streamline visualization, and quantitative analysis of flow through the foramen ovale, ductus arteriosus, and umbilical vessels.
Results or Findings: 4D Flow MRI successfully delineated intracardiac and extracardiac flow pathways in 95% of cases. Quantitative analysis revealed consistent flow patterns correlating with gestational age. In growth-restricted fetuses, altered shunt flow ratios and reduced cerebral perfusion indices were observed.

Neonatal 4D Flow data demonstrated transitional circulatory changes post-delivery, validating physiologic closure of fetal shunts
Conclusion: 4D Flow MRI provides unparalleled insights into fetal and neonatal hemodynamics, overcoming limitations of Doppler ultrasound. It holds transformative potential for early detection of circulatory dysfunction, particularly in placental insufficiency and congenital heart disease.
Limitations: Motion artifacts, fetal heart rate variability, and prolonged acquisition times currently restrict routine clinical application. Absence of real-time gating and small sample size limit statistical generalizability. Further optimization of faster, motion-robust sequences and standardized fetal cardiac gating are required for broader clinical translation.
Funding for this study: None
Has your study been approved by an ethics committee? Not applicable
Ethics committee - additional information:

Author Block: Y. Jia; Sichuan/CN
Purpose: To evaluate the characteristics of fetal congenital cystic lung diseases using MRI and to investigate lung development and prognosis.
Methods or Background: A retrospective analysis was conducted on 230 normal fetuses and 150 fetuses with congenital cystic lung diseases . The normal group underwent measurements of total lung volume and bilateral lung-to-liver signal intensity ratio (LLSIR). The case group was evaluated for lesion signal, volume, cystic volume ratio, bilateral LLSIR, fetal lung volume, and observed/expected fetal lung volume. Postnatal outcomes were obtained via medical record review and telephone follow-up. Cases were classified into a good prognosis group and a poor prognosis group.
Results or Findings: In the normal group, both total lung volume and bilateral LLSIR increased progressively with gestational age. Multivariate logistic regression analysis revealed that the following factors were associated with poor prognosis: lesion volume/affected lung volume (OR = 1.07), absolute difference in bilateral LLSIR (OR = 1.08), and affected lung volume/expected unilateral lung volume (OR = 1.03). ROC curve analysis demonstrated that the combination of lesion volume/affected lung volume and bilateral LLSIR difference showing the highest predictive value. During follow-up, increases in CVR and bilateral LLSIR differences were associated with worse outcomes, while reduction in mass effect indicated better prognosis.
Conclusion: In healthy fetuses, lung volume, bilateral LLSIR, and their differences increase with gestational age. MRI allows precise evaluation of fetal diseases, and its derived indicators hold significant value for predicting poor prognosis. The combination of lesion volume/affected lung volume and bilateral LLSIR difference demonstrates superior predictive performance.
Limitations: Future validation and optimization of these prediction models will require prospective, multicenter studies with larger sample sizes to enhance generalizability and robustness, alongside exploring advanced techniques like radiomics and deep learning for improved prognostic accuracy.
Funding for this study: None.
Has your study been approved by an ethics committee? Not applicable
Ethics committee - additional information:

Author Block: S-Z. Dong; Shanghai/CN
Purpose: To show the types of ductus arteriosus (DA) position and course, and further measure the diameters of DA in foetal different branching patterns of the right aortic arch (RAA) using prenatal cardiac magnetic resonance
Methods or Background: 88 foetuses with RAA were analysed retrospectively. RAA mirror-image branching with left anterior DA (n=16), RAA with aberrant left subclavian artery (ALSCA) and left posterior DA (n=50), RAA mirror-image branching with left retroesophageal DA (n=9), RAA mirror-image branching with right DA (n=11), RAA with ALSCA and bilateral DA (n=1) and RAA mirror-image branching with bilateral DA (n=1). Two radiologists measured the diameters of DA respectively, and then statistically analysed the data of the DA diameters obtained from all foetuses. Statistical comparisons between the z score of DA diameter were analysed using the non-parametric Mann-Whitney U test.
Results or Findings: Of the 88 foetuses, the mean z score of the DA diameter with 16 foetuses with RAA mirror-image branching with left anterior DA was significantly less than that of the 11 cases with RAA with right DA (P < 0.05); the DA of 50 cases with RAA with ALSCA were larger than that of 9 cases with RAA with left retroesophageal DA (P < 0.05). Eleven neonates (12.5%) had respiratory symptoms, nine with RAA with ALSCA and left posterior DA, one with RAA and left anterior DA, and one with RAA and left retroesophageal DA.
Conclusion: Foetal CMR can describe the position and course of DA, and further measure the diameters of DA in different branching patterns of RAA.
Limitations: All cases were followed up only during the neonatal period, therefore, only a few cases have clinical symptoms in this study.
Funding for this study: National Natural Science Foundation of China (81971582, 81571628), Shanghai Pujiang Programme (2019PJD030)
Has your study been approved by an ethics committee? Yes
Ethics committee - additional information: This study was authorized by the ethics commission of our hospital.

Author Block: G. Gourincour¹, H. Boeth², F. Kording², D. Grevent³, R. Chaoui⁴, L. J. Salomon³; ¹Marseille/FR, ²Hamburg/DE, ³Paris/FR, ⁴Berlin/DE
Purpose: To study the clinical and research use of fetal smart-sync cardiovascular magnetic resonance imaging (F-ss-CV-MRI) worldwide
Methods or Background: Extensive survey sent to 70 centers equipped with the device. Questions concerned project-leading specialty, country, setting, quantitative and qualitative user analysis, degree of satisfaction and remaining needs
Results or Findings: 30 centers answered (43 %). In 19 (63 %) team was lead by radiologists, in 8 (27 %) by cardiologists, and in 3 (10 %) by researchers. Thirteen centers were in the US (43 %), 4 (13 %) in Germany, 3 (10 %) in France, 2 (6.7 %) in the Netherlands, Sweden and the UK, and 1 (3.3 %) in Italy, Switzerland and Israel. Setting was academic in 23 (77 %), hospital-based in 16 (54 %) and private in 2 (6.7 %).
Centers have been users for a mean of 21.6 months (Min 1, max 48), with a mean number of exams of 33.2 (Min 2, Max 200). Twenty-eight centers perform MRI between 30 and 35 weeks’ gestation (WG), 8 after 35 WG and 4 before 30 WG. In 19 centers it is performed for clinics and research, in 6 only for clinics and in 6 only research.
Twenty-four centers (80 %) rate as high/very high the improvement and would recommend it.
Clinical situations when it would be recommended were mostly unclear complex congenital heart disease with need for improved delineation
Conclusion: F-ss-CV-MRI is a promising technique which needs shorter sequences, better consistency and wider diffusion
Limitations: Image quality for 10 centers (33 %), convincing their referrals for 8 (27 %), and MR accessibility for 8 (27).
Fourteen centers (47 %) consider that it has not yet an impact on diagnosis but 24 (80 %) believe it will have
Funding for this study: None
Has your study been approved by an ethics committee? Not applicable
Ethics committee - additional information: