Patient-specific closed-loop model of the fontan circulation: Calibration and validation.

The Fontan circulation, designed for managing patients with a single functional ventricle, presents challenges in long-term outcomes. Computational methods offer potential solutions, yet their application in cardiology practice remains largely unexplored. Our aim was to assess the ability of a patient-specific, closed-loop, reduced-order blood flow model to simulate pulsatile blood flow in the Fontan circulation. Using one-dimensional models, we simulated the aorta, superior and inferior venae cavae, and right and left pulmonary arteries, while lumping heart chambers and remaining vessels into zero-dimensional models. The model was calibrated with patient-specific haemodynamic data from combined cardiac catheterisation and magnetic resonance exams, using a novel physics-based stepwise methodology involving simpler open-loop models. Testing on a 10-year-old, anesthetised patient, demonstrated the model's capability to replicate pulsatile pressure and flow in the larger vessels and ventricular pressure. Average relative errors in mean pressure and flow were 2.9 % and 3.6 %, with average relative point-to-point errors (RPPE) in pressure and flow at 5.2 % and 16.0 %. Comparing simulation results to measurements, mean aortic pressure and flow values were 50.7 vs. 50.4 mmHg and 41.6 vs. 41.9 ml/s, respectively, while ventricular pressure values were 28.7 vs. 27.4 mmHg. The model accurately described time-varying ventricular volume with a RPPE of 2.9 %, with mean, minimum, and maximum ventricular volume values for simulation results vs. measurements at 59.2 vs. 58.2 ml, 38.0 vs. 37.6 ml, and 76.0 vs. 74.4 ml, respectively. It provided physiologically realistic predictions of haemodynamic changes from pulmonary vasodilation and atrial fenestration opening. The new model and calibration methodology are freely available, offering a platform to virtually investigate the Fontan circulation's response to clinical interventions and explore potential mechanisms of Fontan failure. Future efforts will concentrate on broadening the model's applicability to a wider range of patient populations and clinical scenarios, as well as testing its effectiveness.

Fully automated planning for anatomical fetal brain MRI on 0.55T.

PURPOSE: Widening the availability of fetal MRI with fully automatic real-time planning of radiological brain planes on 0.55T MRI. METHODS: Deep learning-based detection of key brain landmarks on a whole-uterus echo planar imaging scan enables the subsequent fully automatic planning of the radiological single-shot Turbo Spin Echo acquisitions. The landmark detection pipeline was trained on over 120 datasets from varying field strength, echo times, and resolutions and quantitatively evaluated. The entire automatic planning solution was tested prospectively in nine fetal subjects between 20 and 37 weeks. A comprehensive evaluation of all steps, the distance between manual and automatic landmarks, the planning quality, and the resulting image quality was conducted. RESULTS: Prospective automatic planning was performed in real-time without latency in all subjects. The landmark detection accuracy was 4.2 ± $$ \pm $$ 2.6 mm for the fetal eyes and 6.5 ± $$ \pm $$ 3.2 for the cerebellum, planning quality was 2.4/3 (compared to 2.6/3 for manual planning) and diagnostic image quality was 2.2 compared to 2.1 for manual planning. CONCLUSIONS: Real-time automatic planning of all three key fetal brain planes was successfully achieved and will pave the way toward simplifying the acquisition of fetal MRI thereby widening the availability of this modality in nonspecialist centers.

Design and implementation of multicenter pediatric and congenital studies with cardiovascular magnetic resonance: Big data in smaller bodies.

Cardiovascular magnetic resonance (CMR) has become the reference standard for quantitative and qualitative assessment of ventricular function, blood flow, and myocardial tissue characterization. There is a preponderance of large CMR studies and registries in adults; However, similarly powered studies are lacking for the pediatric and congenital heart disease (PCHD) population. To date, most CMR studies in children are limited to small single or multicenter studies, thereby limiting the conclusions that can be drawn. Within the PCHD CMR community, a collaborative effort has been successfully employed to recognize knowledge gaps with the aim to embolden the development and initiation of high-quality, large-scale multicenter research. In this publication, we highlight the underlying challenges and provide a practical guide toward the development of larger, multicenter initiatives focusing on PCHD populations, which can serve as a model for future multicenter efforts.

Executive Function in Preschool Children with Congenital Heart Disease and Controls: The Role of a Cognitively Stimulating Home Environment.

OBJECTIVE: To assess the relationships between (1) environmental and demographic factors and executive function (EF) in preschool children with congenital heart disease (CHD) and controls and (2) clinical and surgical risk factors and EF in preschool children with CHD. STUDY DESIGN: At 4-6 years of age, parents of children with CHD (n = 51) and controls (n = 124) completed the Behavior Rating Inventory of Executive Function, Preschool Version questionnaire and the Cognitively Stimulating Parenting Scale (CSPS). Multivariable general linear modeling assessed the relationship between Behavior Rating Inventory of Executive Function, Preschool Version composite scores (Inhibitory Self-Control Index [ISCI], Flexibility Index [FI], and Emergent Metacognition Index [EMI]) and group (CHD/control), sex, age at assessment, gestational age, Index of Multiple Deprivation, and CSPS scores. The relationships between CHD type, surgical factors, and brain magnetic resonance imaging injury rating and ISCI, FI, and EMI scores were assessed. RESULTS: The presence of CHD, age at assessment, sex, and Index of Multiple Deprivation were not associated with EF scores. Lower gestational age was associated with greater ISCI and FI scores, and age at assessment was associated with lower FI scores. Group significantly moderated the relationship between CSPS and EF, such that CSPS significantly predicted EF in children with CHD (ISCI: P = .0004; FI: P = .0015; EMI: P = .0004) but not controls (ISCI: P = .2727; FI: P = .6185; EMI: P = .3332). There were no significant relationships between EF scores and surgical factors, CHD type, or brain magnetic resonance imaging injury rating. CONCLUSIONS: Supporting parents to provide a cognitively stimulating home environment may improve EF in children with CHD. The home and parenting environment should be considered when designing intervention studies aimed at improving EF in this patient group.

Perioperative Brain Injury in Relation to Early Neurodevelopment Among Children with Severe Congenital Heart Disease: Results from a European Collaboration.

OBJECTIVE: To examine the relationship between perioperative brain injury and neurodevelopment during early childhood in patients with severe congenital heart disease (CHD). STUDY DESIGN: One hundred and seventy children with CHD and born at term who required cardiopulmonary bypass surgery in the first 6 weeks after birth were recruited from 3 European centers and underwent preoperative and postoperative brain MRIs. Uniform description of imaging findings was performed and an overall brain injury score was created, based on the sum of the worst preoperative or postoperative brain injury subscores. Motor and cognitive outcomes were assessed with the Bayley Scales of Infant and Toddler Development Third Edition at 12 to 30 months of age. The relationship between brain injury score and clinical outcome was assessed using multiple linear regression analysis, adjusting for CHD severity, length of hospital stay (LOS), socioeconomic status (SES), and age at follow-up. RESULTS: Neither the overall brain injury score nor any of the brain injury subscores correlated with motor or cognitive outcome. The number of preoperative white matter lesions was significantly associated with gross motor outcome after correction for multiple testing (P = .013, ß = -0.50). SES was independently associated with cognitive outcome (P < .001, ß = 0.26), and LOS with motor outcome (P < .001, ß = -0.35). CONCLUSION: Preoperative white matter lesions appear to be the most predictive MRI marker for adverse early childhood gross motor outcome in this large European cohort of infants with severe CHD. LOS as a marker of disease severity, and SES influence outcome and future intervention trials need to address these risk factors.

Real-time automatic image-based slice tracking of gadolinium-filled balloon wedge catheter during MR-guided cardiac catheterization: A proof-of-concept study.

PURPOSE: MR-guided cardiac catheterization procedures currently use passive tracking approaches to follow a gadolinium-filled catheter balloon during catheter navigation. This requires frequent manual tracking and repositioning of the imaging slice during navigation. In this study, a novel framework for automatic real-time catheter tracking during MR-guided cardiac catheterization is presented. METHODS: The proposed framework includes two imaging modes (Calibration and Runtime). The sequence starts in Calibration mode, in which the 3D catheter coordinates are determined using a stack of 10-20 contiguous saturated slices combined with real-time image processing. The sequence then automatically switches to Runtime mode, where three contiguous slices (acquired with partial saturation), initially centered on the catheter balloon using the Calibration feedback, are acquired continuously. The 3D catheter balloon coordinates are estimated in real time from each Runtime slice stack using image processing. Each Runtime stack is repositioned to maintain the catheter balloon in the central slice based on the prior Runtime feedback. The sequence switches back to Calibration mode if the catheter is not detected. This framework was evaluated in a heart phantom and 3 patients undergoing MR-guided cardiac catheterization. Catheter detection accuracy and rate of catheter visibility were evaluated. RESULTS: The automatic detection accuracy for the catheter balloon during the Calibration/Runtime mode was 100%/95% in phantom and 100%/97 ± 3% in patients. During Runtime, the catheter was visible in 82% and 98 ± 2% of the real-time measurements in the phantom and patients, respectively. CONCLUSION: The proposed framework enabled real-time continuous automatic tracking of a gadolinium-filled catheter balloon during MR-guided cardiac catheterization.

Total and Regional Brain Volumes in Fetuses With Congenital Heart Disease.

BACKGROUND: Congenital heart disease (CHD) is common and is associated with impaired early brain development and neurodevelopmental outcomes, yet the exact mechanisms underlying these associations are unclear. PURPOSE: To utilize MRI data from a cohort of fetuses with CHD as well as typically developing fetuses to test the hypothesis that expected cerebral substrate delivery is associated with total and regional fetal brain volumes. STUDY TYPE: Retrospective case-control study. POPULATION: Three hundred eighty fetuses (188 male), comprising 45 healthy controls and 335 with isolated CHD, scanned between 29 and 37 weeks gestation. Fetuses with CHD were assigned into one of four groups based on expected cerebral substrate delivery. FIELD STRENGTH/SEQUENCE: T2-weighted single-shot fast-spin-echo sequences and a balanced steady-state free precession gradient echo sequence were obtained on a 1.5 T scanner. ASSESSMENT: Images were motion-corrected and reconstructed using an automated slice-to-volume registration reconstruction technique, before undergoing segmentation using an automated pipeline and convolutional neural network that had undergone semi-supervised training. Differences in total, regional brain (cortical gray matter, white matter, deep gray matter, cerebellum, and brainstem) and brain:body volumes were compared between groups. STATISTICAL TESTS: ANOVA was used to test for differences in brain volumes between groups, after accounting for sex and gestational age at scan. PFDR -values <0.05 were considered statistically significant. RESULTS: Total and regional brain volumes were smaller in fetuses where cerebral substrate delivery is reduced. No significant differences were observed in total or regional brain volumes between control fetuses and fetuses with CHD but normal cerebral substrate delivery (all PFDR > 0.12). Severely reduced cerebral substrate delivery is associated with lower brain:body volume ratios. DATA CONCLUSION: Total and regional brain volumes are smaller in fetuses with CHD where there is a reduction in cerebral substrate delivery, but not in those where cerebral substrate delivery is expected to be normal. EVIDENCE LEVEL: 3 TECHNICAL EFFICACY: Stage 3.

Automatic image-based tracking of gadolinium-filled balloon wedge catheters for MRI-guided cardiac catheterization using deep learning.

Introduction: Magnetic Resonance Imaging (MRI) is a promising alternative to standard x-ray fluoroscopy for the guidance of cardiac catheterization procedures as it enables soft tissue visualization, avoids ionizing radiation and provides improved hemodynamic data. MRI-guided cardiac catheterization procedures currently require frequent manual tracking of the imaging plane during navigation to follow the tip of a gadolinium-filled balloon wedge catheter, which unnecessarily prolongs and complicates the procedures. Therefore, real-time automatic image-based detection of the catheter balloon has the potential to improve catheter visualization and navigation through automatic slice tracking. Methods: In this study, an automatic, parameter-free, deep-learning-based post-processing pipeline was developed for real-time detection of the catheter balloon. A U-Net architecture with a ResNet-34 encoder was trained on semi-artificial images for the segmentation of the catheter balloon. Post-processing steps were implemented to guarantee a unique estimate of the catheter tip coordinates. This approach was evaluated retrospectively in 7 patients (6M and 1F, age = 7 ± 5 year) who underwent an MRI-guided right heart catheterization procedure with all images acquired in an orientation unseen during training. Results: The overall accuracy, specificity and sensitivity of the proposed catheter tracking strategy over all 7 patients were 98.4 ± 2.0%, 99.9 ± 0.2% and 95.4 ± 5.5%, respectively. The computation time of the deep-learning-based segmentation step was ∼10 ms/image, indicating its compatibility with real-time constraints. Conclusion: Deep-learning-based catheter balloon tracking is feasible, accurate, parameter-free, and compatible with real-time conditions. Online integration of the technique and its evaluation in a larger patient cohort are now warranted to determine its benefit during MRI-guided cardiac catheterization.

Individual Assessment of Perioperative Brain Growth Trajectories in Infants With Congenital Heart Disease: Correlation With Clinical and Surgical Risk Factors.

Background Infants with congenital heart disease (CHD) are at risk of neurodevelopmental impairments, which may be associated with impaired brain growth. We characterized how perioperative brain growth in infants with CHD deviates from typical trajectories and assessed the relationship between individualized perioperative brain growth and clinical risk factors. Methods and Results A total of 36 infants with CHD underwent preoperative and postoperative brain magnetic resonance imaging. Regional brain volumes were extracted. Normative volumetric development curves were generated using data from 219 healthy infants. Z-scores, representing the degree of positive or negative deviation from the normative mean for age and sex, were calculated for regional brain volumes from each infant with CHD before and after surgery. The degree of Z-score change was correlated with clinical risk factors. Perioperative growth was impaired across the brain, and it was associated with longer postoperative intensive care stay (false discovery rate P<0.05). Higher preoperative creatinine levels were associated with impaired brainstem, caudate nuclei, and right thalamus growth (all false discovery rate P=0.033). Older postnatal age at surgery was associated with impaired brainstem and right lentiform growth (both false discovery rate P=0.042). Longer cardiopulmonary bypass duration was associated with impaired brainstem and right caudate growth (false discovery rate P<0.027). Conclusions Infants with CHD can have impaired brain growth in the immediate postoperative period, the degree of which associates with postoperative intensive care duration. Brainstem growth appears particularly vulnerable to perioperative clinical course, whereas impaired deep gray matter growth was associated with multiple clinical risk factors, possibly reflecting vulnerability of these regions to short- and long-term hypoxic injury.

Real-time fetal brain tracking for functional fetal MRI.

PURPOSE: To improve motion robustness of functional fetal MRI scans by developing an intrinsic real-time motion correction method. MRI provides an ideal tool to characterize fetal brain development and growth. It is, however, a relatively slow imaging technique and therefore extremely susceptible to subject motion, particularly in functional MRI experiments acquiring multiple Echo-Planar-Imaging-based repetitions, for example, diffusion MRI or blood-oxygen-level-dependency MRI. METHODS: A 3D UNet was trained on 125 fetal datasets to track the fetal brain position in each repetition of the scan in real time. This tracking, inserted into a Gadgetron pipeline on a clinical scanner, allows updating the position of the field of view in a modified echo-planar imaging sequence. The method was evaluated in real-time in controlled-motion phantom experiments and ten fetal MR studies (17 + 4-34 + 3 gestational weeks) at 3T. The localization network was additionally tested retrospectively on 29 low-field (0.55T) datasets. RESULTS: Our method achieved real-time fetal head tracking and prospective correction of the acquisition geometry. Localization performance achieved Dice scores of 84.4% and 82.3%, respectively for both the unseen 1.5T/3T and 0.55T fetal data, with values higher for cephalic fetuses and increasing with gestational age. CONCLUSIONS: Our technique was able to follow the fetal brain even for fetuses under 18 weeks GA in real-time at 3T and was successfully applied "offline" to new cohorts on 0.55T. Next, it will be deployed to other modalities such as fetal diffusion MRI and to cohorts of pregnant participants diagnosed with pregnancy complications, for example, pre-eclampsia and congenital heart disease.

BOUNTI: Brain vOlumetry and aUtomated parcellatioN for 3D feTal MRI.

Fetal MRI is widely used for quantitative brain volumetry studies. However, currently, there is a lack of universally accepted protocols for fetal brain parcellation and segmentation. Published clinical studies tend to use different segmentation approaches that also reportedly require significant amounts of time-consuming manual refinement. In this work, we propose to address this challenge by developing a new robust deep learning-based fetal brain segmentation pipeline for 3D T2w motion corrected brain images. At first, we defined a new refined brain tissue parcellation protocol with 19 regions-of-interest using the new fetal brain MRI atlas from the Developing Human Connectome Project. This protocol design was based on evidence from histological brain atlases, clear visibility of the structures in individual subject 3D T2w images and the clinical relevance to quantitative studies. It was then used as a basis for developing an automated deep learning brain tissue parcellation pipeline trained on 360 fetal MRI datasets with different acquisition parameters using semi-supervised approach with manually refined labels propagated from the atlas. The pipeline demonstrated robust performance for different acquisition protocols and GA ranges. Analysis of tissue volumetry for 390 normal participants (21-38 weeks gestational age range), scanned with three different acquisition protocols, did not reveal significant differences for major structures in the growth charts. Only minor errors were present in < 15% of cases thus significantly reducing the need for manual refinement. In addition, quantitative comparison between 65 fetuses with ventriculomegaly and 60 normal control cases were in agreement with the findings reported in our earlier work based on manual segmentations. These preliminary results support the feasibility of the proposed atlas-based deep learning approach for large-scale volumetric analysis. The created fetal brain volumetry centiles and a docker with the proposed pipeline are publicly available online at https://hub.docker.com/r/fetalsvrtk/segmentation (tag brain_bounti_tissue).

Extended reality for procedural planning and guidance in structural heart disease - a review of the state-of-the-art.

Extended reality (XR), which encompasses virtual, augmented and mixed reality, is an emerging medical imaging display platform which enables intuitive and immersive interaction in a three-dimensional space. This technology holds the potential to enhance understanding of complex spatial relationships when planning and guiding cardiac procedures in congenital and structural heart disease moving beyond conventional 2D and 3D image displays. A systematic review of the literature demonstrates a rapid increase in publications describing adoption of this technology. At least 33 XR systems have been described, with many demonstrating proof of concept, but with no specific mention of regulatory approval including some prospective studies. Validation remains limited, and true clinical benefit difficult to measure. This review describes and critically appraises the range of XR technologies and its applications for procedural planning and guidance in structural heart disease while discussing the challenges that need to be overcome in future studies to achieve safe and effective clinical adoption.

Free-breathing, Contrast Agent-free Whole-Heart MTC-BOOST Imaging: Single-Center Validation Study in Adult Congenital Heart Disease.

Purpose: To assess the clinical performance of the three-dimensional, free-breathing, Magnetization Transfer Contrast Bright-and-black blOOd phase-SensiTive (MTC-BOOST) sequence in adult congenital heart disease (ACHD). Materials and Methods: In this prospective study, participants with ACHD undergoing cardiac MRI between July 2020 and March 2021 were scanned with the clinical T2-prepared balanced steady-state free precession sequence and proposed MTC-BOOST sequence. Four cardiologists scored their diagnostic confidence on a four-point Likert scale for sequential segmental analysis on images acquired with each sequence. Scan times and diagnostic confidence were compared using the Mann-Whitney test. Coaxial vascular dimensions at three anatomic landmarks were measured, and agreement between the research sequence and the corresponding clinical sequence was assessed with Bland-Altman analysis. Results: The study included 120 participants (mean age, 33 years ± 13 [SD]; 65 men). The mean acquisition time of the MTC-BOOST sequence was significantly lower compared with that of the conventional clinical sequence (9 minutes ± 2 vs 14 minutes ± 5; P < .001). Diagnostic confidence was higher for the MTC-BOOST sequence compared with the clinical sequence (mean, 3.9 ± 0.3 vs 3.4 ± 0.7; P < .001). Narrow limits of agreement and mean bias less than 0.08 cm were found between the research and clinical vascular measurements. Conclusion: The MTC-BOOST sequence provided efficient, high-quality, and contrast agent-free three-dimensional whole-heart imaging in ACHD, with shorter, more predictable acquisition time and improved diagnostic confidence compared with the reference standard clinical sequence.Keywords: MR Angiography, Cardiac Supplemental material is available for this article. Published under a CC BY 4.0 license.

Retrospective motion correction through multi-average k-space data elimination (REMAKE) for free-breathing cardiac cine imaging.

PURPOSE: To develop a motion-robust reconstruction technique for free-breathing cine imaging with multiple averages. METHOD: Retrospective motion correction through multiple average k-space data elimination (REMAKE) was developed using iterative removal of k-space segments (from individual k-space samples) that contribute most to motion corruption while combining any remaining segments across multiple signal averages. A variant of REMAKE, termed REMAKE+, was developed to address any losses in SNR due to k-space information removal. With REMAKE+, multiple reconstructions using different initial conditions were performed, co-registered, and averaged. Both techniques were validated against clinical "standard" signal averaging reconstruction in a static phantom (with simulated motion) and 15 patients undergoing free-breathing cine imaging with multiple averages. Quantitative analysis of myocardial sharpness, blood/myocardial SNR, myocardial-blood contrast-to-noise ratio (CNR), as well as subjective assessment of image quality and rate of diagnostic quality images were performed. RESULTS: In phantom, motion artifacts using "standard" (RMS error [RMSE]: 2.2 ± 0.5) were substantially reduced using REMAKE/REMAKE+ (RMSE: 1.5 ± 0.4/1.0 ± 0.4, p < 0.01). In patients, REMAKE/REMAKE+ led to higher myocardial sharpness (0.79 ± 0.09/0.79 ± 0.1 vs. 0.74 ± 0.12 for "standard", p = 0.004/0.04), higher image quality (1.8 ± 0.2/1.9 ± 0.2 vs. 1.6 ± 0.4 for "standard", p = 0.02/0.008), and a higher rate of diagnostic quality images (99%/100% vs. 94% for "standard"). Blood/myocardial SNR for "standard" (94 ± 30/33 ± 10) was higher vs. REMAKE (80 ± 25/28 ± 8, p = 0.002/0.005) and tended to be lower vs. REMAKE+ (105 ± 33/36 ± 12, p = 0.02/0.06). Myocardial-blood CNR for "standard" (61 ± 22) was higher vs. REMAKE (53 ± 19, p = 0.003) and lower vs. REMAKE+ (69 ± 24, p = 0.007). CONCLUSIONS: Compared to "standard" signal averaging reconstruction, REMAKE and REMAKE+ provide improved myocardial sharpness, image quality, and rate of diagnostic quality images.

A deep learning approach for fully automated cardiac shape modeling in tetralogy of Fallot.

BACKGROUND: Cardiac shape modeling is a useful computational tool that has provided quantitative insights into the mechanisms underlying dysfunction in heart disease. The manual input and time required to make cardiac shape models, however, limits their clinical utility. Here we present an end-to-end pipeline that uses deep learning for automated view classification, slice selection, phase selection, anatomical landmark localization, and myocardial image segmentation for the automated generation of three-dimensional, biventricular shape models. With this approach, we aim to make cardiac shape modeling a more robust and broadly applicable tool that has processing times consistent with clinical workflows. METHODS: Cardiovascular magnetic resonance (CMR) images from a cohort of 123 patients with repaired tetralogy of Fallot (rTOF) from two internal sites were used to train and validate each step in the automated pipeline. The complete automated pipeline was tested using CMR images from a cohort of 12 rTOF patients from an internal site and 18 rTOF patients from an external site. Manually and automatically generated shape models from the test set were compared using Euclidean projection distances, global ventricular measurements, and atlas-based shape mode scores. RESULTS: The mean absolute error (MAE) between manually and automatically generated shape models in the test set was similar to the voxel resolution of the original CMR images for end-diastolic models (MAE = 1.9 ± 0.5 mm) and end-systolic models (MAE = 2.1 ± 0.7 mm). Global ventricular measurements computed from automated models were in good agreement with those computed from manual models. The average mean absolute difference in shape mode Z-score between manually and automatically generated models was 0.5 standard deviations for the first 20 modes of a reference statistical shape atlas. CONCLUSIONS: Using deep learning, accurate three-dimensional, biventricular shape models can be reliably created. This fully automated end-to-end approach dramatically reduces the manual input required to create shape models, thereby enabling the rapid analysis of large-scale datasets and the potential to deploy statistical atlas-based analyses in point-of-care clinical settings. Training data and networks are available from cardiacatlas.org.

Simultaneous Highly Efficient Contrast-Free Lumen and Vessel Wall MR Imaging for Anatomical Assessment of Aortic Disease.

BACKGROUND: Bright-blood lumen and black-blood vessel wall imaging are required for the comprehensive assessment of aortic disease. These images are usually acquired separately, resulting in long examinations and potential misregistration between images. PURPOSE: To characterize the performance of an accelerated and respiratory motion-compensated three-dimensional (3D) cardiac MRI technique for simultaneous contrast-free aortic lumen and vessel wall imaging with an interleaved T2 and inversion recovery prepared sequence (iT2Prep-BOOST). STUDY TYPE: Prospective. POPULATION: A total of 30 consecutive patients with aortopathy referred for a clinically indicated cardiac MRI examination (9 females, mean age ± standard deviation: 32 ± 12 years). FIELD STRENGTH/SEQUENCE: 1.5-T; bright-blood MR angiography (diaphragmatic navigator-gated T2-prepared 3D balanced steady-state free precession [bSSFP], T2Prep-bSSFP), breath-held black-blood two-dimensional (2D) half acquisition single-shot turbo spin echo (HASTE), and 3D bSSFP iT2Prep-BOOST. ASSESSMENT: iT2Prep-BOOST bright-blood images were compared to T2prep-bSSFP images in terms of aortic vessel dimensions, lumen-to-myocardium contrast ratio (CR), and image quality (diagnostic confidence, vessel sharpness and presence of artifacts, assessed by three cardiologists on a 4-point scale, 1: nondiagnostic to 4: excellent). The iT2Prep-BOOST black-blood images were compared to 2D HASTE images for quantification of wall thickness. A visual comparison between computed tomography (CT) and iT2Prep-BOOST was performed in a patient with chronic aortic dissection. STATISTICAL TESTS: Paired t-tests, Wilcoxon signed-rank tests, intraclass correlation coefficient (ICC), Bland-Altman analysis. A P value < 0.05 was considered statistically significant. RESULTS: Bright-blood iT2Prep-BOOST resulted in significantly improved image quality (mean ± standard deviation 3.8 ± 0.5 vs. 3.3 ± 0.8) and CR (2.9 ± 0.8 vs. 1.8 ± 0.5) compared with T2Prep-bSSFP, with a shorter scan time (7.8 ± 1.7 minutes vs. 12.9 ± 3.4 minutes) while providing a complementary 3D black-blood image. Aortic lumen diameter and vessel wall thickness measurements in bright-blood and black-blood images were in good agreement with T2Prep-bSSFP and HASTE images (<0.02 cm and <0.005 cm bias, respectively) and good intrareader (ICC > 0.96) and interreader (ICC > 0.94) agreement was observed for all measurements. DATA CONCLUSION: iT2Prep-BOOST might enable time-efficient simultaneous bright- and black-blood aortic imaging, with improved image quality compared to T2Prep-bSSFP and HASTE imaging, and comparable measurements for aortic wall and lumen dimensions. EVIDENCE LEVEL: 2. TECHNICAL EFFICACY: Stage 2.

Learning the Hidden Signature of Fetal Arch Anatomy: a Three-Dimensional Shape Analysis in Suspected Coarctation of the Aorta.

Neonatal coarctation of the aorta (CoA) is a common congenital heart defect. Its antenatal diagnosis remains challenging, and its pathophysiology is poorly understood. We present a novel statistical shape modeling (SSM) pipeline to study the role and predictive value of arch shape in CoA in utero. Cardiac magnetic resonance imaging (CMR) data of 112 fetuses with suspected CoA was acquired and motion-corrected to three-dimensional volumes. Centerlines from fetal arches were extracted and used to build a statistical shape model capturing relevant anatomical variations. A linear discriminant analysis was used to find the optimal axis between CoA and false positive cases. The CoA shape risk score classified cases with an area under the curve of 0.907. We demonstrate the feasibility of applying a SSM pipeline to three-dimensional fetal CMR data while providing novel insights into the anatomical determinants of CoA and the relevance of in utero arch anatomy for antenatal diagnosis of CoA.

Neuromonitoring, neuroimaging, and neurodevelopmental follow-up practices in neonatal congenital heart disease: a European survey.

BACKGROUND: Brain injury and neurodevelopmental impairment remain a concern in children with complex congenital heart disease (CHD). A practice guideline on neuromonitoring, neuroimaging, and neurodevelopmental follow-up in CHD patients undergoing cardiopulmonary bypass surgery is lacking. The aim of this survey was to systematically evaluate the current practice in centers across Europe. METHODS: An online-based structured survey was sent to pediatric cardiac surgical centers across Europe between April 2019 and June 2020. Results were summarized by descriptive statistics. RESULTS: Valid responses were received by 25 European centers, of which 23 completed the questionnaire to the last page. Near-infrared spectroscopy was the most commonly used neuromonitoring modality used in 64, 80, and 72% preoperatively, intraoperatively, and postoperatively, respectively. Neuroimaging was most commonly performed by means of cranial ultrasound in 96 and 84% preoperatively and postoperatively, respectively. Magnetic resonance imaging was obtained in 72 and 44% preoperatively and postoperatively, respectively, but was predominantly reserved for clinically symptomatic patients (preoperatively 67%, postoperatively 64%). Neurodevelopmental follow-up was implemented in 40% of centers and planned in 24%. CONCLUSIONS: Heterogeneity in perioperative neuromonitoring and neuroimaging practice in CHD in centers across Europe is large. The need for neurodevelopmental follow-up has been recognized. A clear practice guideline is urgently needed. IMPACT: There is large heterogeneity in neuromonitoring, neuroimaging, and neurodevelopmental follow-up practices among European centers caring for neonates with complex congenital heart disease. This study provides a systematic evaluation of the current neuromonitoring, neuroimaging, and neurodevelopmental follow-up practice in Europe. The results of this survey may serve as the basis for developing a clear practice guideline that could help to early detect and prevent neurological and neurodevelopmental sequelae in neonates with complex congenital heart disease.

Magnetization Transfer BOOST Noncontrast Angiography Improves Pulmonary Vein Imaging in Adults With Congenital Heart Disease.

BACKGROUND: Cardiac MRI plays an important role in the diagnosis and follow-up of patients with congenital heart disease (CHD). Gadolinium-based contrast agents are often needed to overcome flow-related and off-resonance artifacts that can impair the quality of conventional noncontrast 3D imaging. As serial imaging is often required in CHD, the development of robust noncontrast 3D MRI techniques is desirable. PURPOSE: To assess the clinical utility of noncontrast enhanced magnetization transfer and inversion recovery prepared 3D free-breathing sequence (MTC-BOOST) compared to conventional 3D whole heart imaging in patients with CHD. STUDY TYPE: Prospective, image quality. POPULATION: A total of 27 adult patients (44% female, mean age 30.9 ± 14.8 years) with CHD. FIELD STRENGTH/SEQUENCE: A 1.5 T; free-breathing 3D MTC-BOOST sequence. ASSESSMENT: MTC-BOOST was compared to diaphragmatic navigator-gated, noncontrast T2 prepared 3D whole-heart imaging sequence (T2prep-3DWH) for comparison of vessel dimensions, lumen-to-myocardium contrast ratio (CR), and image quality (vessel wall sharpness and presence and type of artifacts) assessed by two experienced cardiologists on a 5-point scale. STATISTICAL TESTS: Mann-Whitney test, paired Wilcoxon signed-rank test, Bland-Altman plots. P < 0.05 was considered statistically significant. RESULTS: MTC-BOOST significantly improved image quality and CR of the right-sided pulmonary veins (PV): (CR: right upper PV 1.06 ± 0.50 vs. 0.58 ± 0.74; right lower PV 1.32 ± 0.38 vs. 0.81 ± 0.73) compared to conventional T2prep-3DWH imaging where the PVs were not visualized in some cases due to off-resonance effects. MTC-BOOST demonstrated resistance to degradation of luminal signal (assessed by CR) secondary to accelerated or turbulent flow conditions. T2prep-3DWH had higher image quality scores than MTC-BOOST for the aorta and coronary arteries; however, great vessel dimensions derived from MTC-BOOST showed excellent agreement with standard T2prep-3DWH imaging. DATA CONCLUSION: MTC-BOOST allows for improved contrast-free imaging of pulmonary veins and regions characterized by accelerated or turbulent blood flow compared to standard T2prep-3DWH imaging, with excellent agreement of great vessel dimensions. EVIDENCE LEVEL: 1 TECHNICAL EFFICACY: Stage 2.