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Background: The three-dimensional balanced-steady-state-free-precession (3D bSSFP) whole-heart (WH) technique has long been used to depict cardiac morphology in congenital heart disease (CHD) but is prone to banding artifacts. The Relaxation Enhanced Angiography without Contrast and Triggering (REACT) sequence is an alternative method that is resistant to off-resonance effects., Objective: To evaluate cardiac structures and great vessels in CHD patients using 3D WH REACT sequence and compare it to 3D WH bSSFP sequence., Materials and Methods: This study was approved by the Institutional Review Board. Thirty CHD patients were prospectively enrolled. Contrast-to-noise ratio (CNR), image quality, and cross-sectional area (CSA) were analyzed. Categorical data were compared with a Wilcoxon signed-rank test and normally distributed variables with a t-test., Results: Thirty patients (16 females) participated in this study (median age 17, range 5 months to 52 years). REACT showed higher CNR in all pulmonary veins (all P<0.05), while 3D bSSFP had higher CNR in the right ventricle (P<0.001) and right pulmonary artery, (P=0.04). Image quality favored 3D bSSFP in the right atrium and ventricle (both P<0.001), main pulmonary artery (P=0.02), and coronary arteries (left: P<0.001, right: P=0.01). REACT outperformed 3D bSSFP for the pulmonary veins (all P<0.05) from image quality perspective. CSA measurements were not significantly different between REACT and 3D bSSFP (all P≥0.05)., Conclusion: The REACT method is associated with improved image quality and CNR for pulmonary veins, with CSA measurements concordant with 3D bSSFP in CHD patients, while bSSFP shows better performance for imaging cardiac chambers and coronary arteries., Competing Interests: Declarations. Conflicts of interest: None, (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

A high temporal resolution, 4-chamber (4CH) cine is the standard method for determining cardiac rest periods during whole heart coronary magnetic resonance angiography (CMRA). We evaluated the image quality and reproducibility between the 4CH cine method and a novel approach using a velocity encoded mitral valve inflow cine (MVI). The goal of this study was to compare the quality of CMRAs utilizing MVI versus 4CH methods. Sharpness and vessel length for the LCA and RCA using each method were determined using Soap Bubble and two blinded observers independently assessed coronary image quality. Offline analysis on a separate, retrospective cohort (n = 25) was used to compare MVI and 4CH reproducibility. In the prospectively evaluated cohort there was no difference in overall vessel sharpness (4CH vs MVI mean ± SD) (31.0 ± 5.5% vs 30.5 ± 5.7%, p  = .63), LCA vessel sharpness (30.0 ± 5.4% vs 31.1 ± 8.2%, p  = .44), LCA length (4.7 ± 1.4 cm vs 4.6 ± 1.6 cm, p  = .66), RCA vessel sharpness (32.1 ± 6.9% vs 31.1 ± 7.7%, p  = .55), RCA length (5.51 ± 2.6 cm vs 5.95 ± 2.4 cm, p  = .38), or image quality rating (2.66 vs 2.62, p  = .80) between methods. In the retrospective cohort, the MVI method had 5.4% lower inter-observer variability (95% CI 3.7,7.2%, p  < .0001) and 3.9% lower intra-observer variability (95% CI 2.4,5.4%, p  < .0001) than the 4CH method. MVI is a technically feasible and more reproducible method to determine cardiac rest periods compared to 4CH while preserving vessel sharpness, vessel length & image quality., Competing Interests: Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2022.)

Ventricular contouring of cardiac magnetic resonance imaging is the gold standard for volumetric analysis for repaired tetralogy of Fallot (rTOF), but can be time-consuming and subject to variability. A convolutional neural network (CNN) ventricular contouring algorithm was developed to generate contours for mostly structural normal hearts. We aimed to improve this algorithm for use in rTOF and propose a more comprehensive method of evaluating algorithm performance. We evaluated the performance of a ventricular contouring CNN, that was trained on mostly structurally normal hearts, on rTOF patients. We then created an updated CNN by adding rTOF training cases and evaluated the new algorithm's performance generating contours for both the left and right ventricles (LV and RV) on new testing data. Algorithm performance was evaluated with spatial metrics (Dice Similarity Coefficient (DSC), Hausdorff distance, and average Hausdorff distance) and volumetric comparisons (e.g., differences in RV volumes). The original Mostly Structurally Normal (MSN) algorithm was better at contouring the LV than the RV in patients with rTOF. After retraining the algorithm, the new MSN + rTOF algorithm showed improvements for LV epicardial and RV endocardial contours on testing data to which it was naïve (N = 30; e.g., DSC 0.883 vs. 0.905 for LV epicardium at end diastole, p < 0.0001) and improvements in RV end-diastolic volumetrics (median %error 8.1 vs 11.4, p = 0.0022). Even with a small number of cases, CNN-based contouring for rTOF can be improved. This work should be extended to other forms of congenital heart disease with more extreme structural abnormalities. Aspects of this work have already been implemented in clinical practice, representing rapid clinical translation. The combined use of both spatial and volumetric comparisons yielded insights into algorithm errors.

Background: Due to passive blood flow in palliated single ventricle, central venous pressure increases chronically, ultimately impeding lymphatic drainage. Early visualization and treatment of these malformations is essential to reduce morbidity and mortality. Cardiovascular magnetic resonance (CMR) T2-weighted lymphangiography (T2w) is used for lymphatic assessment, but its low signal-to-noise ratio may result in incomplete visualization of thoracic duct pathway. 3D-balanced steady state free precession (3D-bSSFP) is commonly used to assess congenital cardiac disease anatomy. Here, we aimed to improve diagnostic imaging of thoracic duct pathway using 3D-bSSFP., Methods: Patients underwent CMR during single ventricle or central lymphatic system assessment using T2w and 3D-bSSFP. T2w parameters included 3D-turbo spin echo (TSE), TE/TR = 600/2500 ms, resolution = 1 × 1 × 1.8 mm, respiratory triggering with bellows. 3D-bSSFP parameters included electrocardiogram triggering and diaphragm navigator, 1.6 mm isotropic resolution, TE/TR = 1.8/3.6 ms. Thoracic duct was identified independently in T2w and 3D-bSSFP images, tracked completely from cisterna chyli to its drainage site, and classified based on severity of lymphatic abnormalities., Results: Forty-eight patients underwent CMR, 46 of whom were included in the study. Forty-five had congenital heart disease with single ventricle physiology. Median age at CMR was 4.3 year (range 0.9-35.1 year, IQR 2.4 year), and median weight was 14.4 kg (range, 7.9-112.9 kg, IQR 5.2 kg). Single ventricle with right dominant ventricle was noted in 31 patients. Thirty-eight patients (84%) were status post bidirectional Glenn and 7 (16%) were status post Fontan anastomosis. Thoracic duct visualization was achieved in 45 patients by T2w and 3D-bSSFP. Complete tracking to drainage site was attained in 11 patients (24%) by T2w vs 25 (54%) by 3D-bSSFP and in 28 (61%) by both. Classification of lymphatics was performed in 31 patients., Conclusion: Thoracic duct pathway can be visualized by 3D-bSSFP combined with T2w lymphangiography. Cardiac triggering and respiratory navigation likely help retain lymphatic signal in the retrocardiac area by 3D-bSSFP. Visualizing lymphatic system leaks is challenging on 3D-bSSFP images alone, but 3D-bSSFP offers good visualization of duct anatomy and landmark structures to help plan interventions. Together, these sequences can define abnormal lymphatic pathway following single ventricle palliative surgery, thus guiding lymphatic interventional procedures.

Background: Volumetric black-blood cardiovascular magnetic resonance (CMR) has been hampered by long scan times and flow sensitivity. The purpose of this study was to assess the feasibility of black-blood, electrocardiogram (ECG)-triggered and respiratory-navigated 3D fast spin echo (3D FSE) for the visualization of the whole heart and great vessels., Methods: The implemented 3D FSE technique used slice-selective excitation and non-selective refocusing pulses with variable flip angles to achieve constant echo signal for tissue with T1 (880 ms) and T2 (40 ms) similar to the vessel wall. Ten healthy subjects and 21 patients with congenital heart disease (CHD) underwent 3D FSE and conventional 3D balanced steady-state free precession (bSSFP). The sequences were compared in terms of ability to perform segmental assessment, local signal-to-noise ratio (SNR l ) and local contrast-to-noise ratio (CNR l )., Results: In both healthy subjects and patients with CHD, 3D FSE showed superior pulmonary vein but inferior coronary artery origin visualisation compared to 3D bSFFP. However, in patients with CHD the combination of 3D bSSFP and 3D FSE whole-heart imaging improves the success rate of cardiac morphological diagnosis to 100% compared to either technique in isolation (3D FSE, 23.8% success rate, 3D bSSFP, 5% success rate). In the healthy subjects SNR l for 3D bSSFP was greater than for 3D FSE (30.1 ± 7.3 vs 20.9 ± 5.3; P = 0.002) whereas the CNR l was comparable (17.3 ± 5.6 vs 17.4 ± 4.9; P = 0.91) between the two scans., Conclusions: The feasibility of 3D FSE for whole-heart black-blood CMR imaging has been demonstrated. Due to their high success rate for segmental assessment, the combination of 3D bSSFP and 3D FSE may be an attractive alternative to gadolinium contrast enhanced morphological CMR in patients with CHD.

Background: Dual-phase 3-dimensional whole-heart acquisition allows simultaneous imaging during systole and diastole. Respiratory navigator gating and tracking of the diaphragm is used with limited accuracy. Prolonged scan time is common, and navigation often fails in patients with erratic breathing. Image-navigation (iNAV) tracks movement of the heart itself and is feasible in single phase whole heart imaging. To evaluate its diagnostic ability in congenital heart disease, we sought to apply iNAV to dual-phase sequencing., Methods: Healthy volunteers and patients with congenital heart disease underwent dual-phase imaging using the conventional diaphragmatic-navigation (dNAV) and iNAV. Acquisition time was recorded and image quality assessed. Sharpness and length of the right coronary (RCA), left anterior descending (LAD), and circumflex (LCx) arteries were measured in both cardiac phases for both approaches. Qualitative and quantitative analyses were performed in a blinded and randomized fashion., Results: In volunteers, there was no significant difference in vessel sharpness between approaches (p > 0.05). In patients, analysis showed equal vessel sharpness for LAD and RCA (p > 0.05). LCx sharpness was greater with dNAV (p < 0.05). Visualized length with iNAV was 0.5 ± 0.4 cm greater than that with dNAV for LCx in diastole (p < 0.05), 1.0 ± 0.3 cm greater than dNAV for LAD in diastole (p < 0.05), and 0.8 ± 0.7 cm greater than dNAV for RCA in systole (p < 0.05). Qualitative scores were similar between modalities (p = 0.71). Mean iNAV scan time was 5:18 ± 2:12 min shorter than mean dNAV scan time in volunteers (p = 0.0001) and 3:16 ± 1:12 min shorter in patients (p = 0.0001)., Conclusions: Image quality of iNAV and dNAV was similar with better distal vessel visualization with iNAV. iNAV acquisition time was significantly shorter. Complete cardiac diagnosis was achieved. Shortened acquisition time will improve clinical applicability and patient comfort.

Ventricular volumetric ejection fraction (VV EF) is often normal in patients with single ventricle circulations despite them experiencing symptoms related to circulatory failure. We sought to determine if kinetic energy (KE) could be a better marker of ventricular performance. KE was prospectively quantified using four-dimensional flow MRI in 41 patients with a single ventricle circulation (aged 0.5-28 yr) and compared with 43 healthy volunteers (aged 1.5-62 yr) and 14 patients with left ventricular (LV) dysfunction (aged 28-79 yr). Intraventricular end-diastolic blood was tracked through systole and divided into ejected and residual blood components. Two ejection fraction (EF) metrics were devised based on the KE of the ejected component over the total of both the ejected and residual components using 1) instantaneous peak KE to assess KE EF or 2) summating individual peak particle energy (PE) to assess PE EF. KE EF and PE EF had a smaller range than VV EF in healthy subjects (97.9 ± 0.8 vs. 97.3 ± 0.8 vs. 60.1 ± 5.2%). LV dysfunction caused a fall in KE EF ( P = 0.01) and PE EF ( P = 0.0001). VV EF in healthy LVs and single ventricle hearts was equivalent; however, KE EF and PE EF were lower ( P < 0.001) with a wider range indicating a spectrum of severity. Those reporting the greatest symptomatic impairment (New York Heart Association II) had lower PE EF than asymptomatic subjects ( P = 0.0067). KE metrics are markers of healthy cardiac function. PE EF may be useful in grading dysfunction. NEW & NOTEWORTHY Kinetic energy (KE) represents the useful work of the heart in ejecting blood. This article details the utilization of KE indexes to assess cardiac function in health and a variety of pathophysiological conditions. KE ejection fraction and particle energy ejection fraction (PE EF) showed a narrow range in health and a lower wider range in disease representing a spectrum of severity. PE EF was altered by functional status potentially offering the opportunity to grade dysfunction.

Background: CMRA in pediatrics remains challenging due to the smaller vessel size, high heart rates (HR), potential image degradation caused by limited patient cooperation and long acquisition times. High-relaxivity contrast agents have been shown to improve coronary imaging in adults, but limited data is available in children. We sought to investigate whether gadobenate dimeglumine (Gd-BOPTA) together with self-navigated inversion-prepared coronary magnetic resonance angiography (CMRA) sequence design improves coronary image quality in pediatric patients., Methods: Forty consecutive patients (mean age 6±2.8years; 73% males) were prospectively recruited for a 1.5-T MRI study under general anesthesia. Two electrocardiographic-triggered free breathing steady-state free precession (SSFP) angiography sequences (A and B) with isotropic spatial resolution (1.3mm 3 ) were acquired using a recently developed image-based self-navigation technique. Sequence A was acquired prior to contrast administration using T2 magnetization preparation (T2prep). Sequence B was acquired 5-8min after a bolus of Gd-BOPTA with the T2prep replaced by an inversion recovery (IR) pulse to null the signal from the myocardium. Scan time, signal-to noise and contrast-to-noise ratios (SNR and CNR), vessel wall sharpness (VWS) and qualitative visual score for each sequence were compared., Results: Scan time was similar for both sequences (5.3±1.8 vs 5.2±1.5min, p=.532) and average heart rate (78±14.7 vs 78±14.5bpm, p=.443) remained constant throughout both acquisitions. Sequence B resulted in higher SNR (12.6±4.4 vs 31.1±7.4, p<.001) and CNR (9.0±1.8 vs 13.5±3.7, p<.001) and provided improved coronary visualization in all coronary territories (VWS A=0.53±0.07 vs B=0.56±0.07, p=.001; and visual scoring A=3.8±0.59 vs B=4.1±0.53, p<.001). The number of non-diagnostic coronary segments was lower for sequence B [A=42 (13.1%) segments vs B=33 (10.3%) segments; p=.002], and contrary to the pre-contrast sequence, never involved a proximal segment. These results were independent of the patients' age, body surface area and HR., Conclusions: The use of Gd-BOPTA with a 3D IR SSFP CMRA sequence results in improved coronary visualization in small infants and young children with high HR within a clinically acceptable scan time., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: X-ray coronary angiography (XCA) is the current gold standard for the assessment of lumen encroaching coronary stenosis but XCA does not allow for early detection of rupture-prone vulnerable plaques, which are thought to be the precursor lesions of most acute myocardial infarctions (AMI) and sudden death. The aim of this study was to investigate the potential of delayed contrast-enhanced magnetic resonance coronary vessel wall imaging (CE-MRCVI) for the detection of culprit lesions in the coronary arteries., Methods: 16 patients (13 male, age 61.9±8.6 years) presenting with sub-acute MI underwent CE-MRCVI within 24-72h prior to invasive XCA. CE-MRCVI was performed using a T1-weighted 3D gradient echo inversion recovery sequence (3D IR TFE) 40±4 minutes following the administration of 0.2 mmol/kg gadolinium-diethylenetriamine-pentaacetic acid (DTPA) on a 3T MRI scanner equipped with a 32-channel cardiac coil., Results: 14 patients were found to have culprit lesions (7x LAD, 1xLCX, 6xRCA) as identified by XCA. Quantitative CE-MRCVI correctly identified the culprit lesion location with a sensitivity of 79% and excluded culprit lesion formation with a specificity of 99%. The contrast to noise ratio (CNR) of culprit lesions (9.7±4.1) significantly exceeded CNR values of segments without culprit lesions (2.9±1.9, p<0.001)., Conclusion: CE-MRCVI allows the selective visualization of culprit lesions in patients immediately after myocardial infarction (MI). The pronounced contrast uptake in ruptured plaques may represent a surrogate biomarker of plaque activity and/or vulnerability.

Objectives: The aim of this study was to investigate the impact of BMI on late gadolinium enhancement (LGE) of the coronary artery wall in identical monozygous twins discordant for BMI. Coronary LGE represents a useful parameter for the detection and quantification of atherosclerotic coronary vessel wall disease., Methods: Thirteen monozygote female twin pairs (n = 26) with significantly different BMIs (>1.6 kg/m2) were recruited out of >10,000 twin pairs (TwinsUK Registry). A coronary 3D-T2prep-TFE MR angiogram and 3D-IR-TFE vessel wall scan were performed prior to and following the administration of 0.2 mmol/kg of Gd-DTPA on a 1.5 T MR scanner. The number of enhancing coronary segments and contrast to noise ratios (CNRs) of the coronary wall were quantified., Results: An increase in BMI was associated with an increased number of enhancing coronary segments (5.3 ± 1.5 vs. 3.5 ± 1.6, p < 0.0001) and increased coronary wall enhancement (6.1 ± 1.1 vs. 4.8 ± 0.9, p = 0.0027) compared to matched twins with lower BMI., Conclusions: This study in monozygous twins indicates that acquired factors predisposing to obesity, including lifestyle and environmental factors, result in increased LGE of the coronary arteries, potentially reflecting an increase in coronary atherosclerosis in this female study population., Key Points: • BMI-discordant twins allow the investigation of the influence of lifestyle factors independent from genetic confounders. • Only thirteen obesity-discordant twins were identified underlining the strong genetic component of BMI. • In female twins, a BMI increase is associated with increased coronary late gadolinium enhancement. • Increased late gadolinium enhancement in the coronary vessel wall potentially reflects increased atherosclerosis.

Background: Children with a single systemic right ventricle, such as in hypoplastic left heart syndrome (HLHS), frequently experience reduced exercise capacity. Elucidating the causes could help with optimising treatment strategies., Methods: Prospective data from 10 consecutive symptomatic patients with HLHS undergoing clinical cardiac magnetic resonance with catheterisation (XMR) were analysed. Mean age 8.6years (range 3.5-11.6years), mean time since Fontan completion 5.5years. MR-compatible catheters were placed in the systemic right ventricle and branch pulmonary arteries to record pressures at rest, with dobutamine infusion at 10mcg/kg/min and at 20mcg/kg/min. Cine short-axis stacks of the ventricle were performed at each condition and used to construct pressure-volume loops., Results: Compared to rest, cardiac index increased with low-dose dobutamine (p<0.01) with no further rise at peak stress despite a further, albeit, blunted rise in heart rate (p=0.002). A fall in stroke volume occurred (p=0.014) despite good contractility (74% increase, p=0.045) and a well-coupled ventriculo-arterial ratio. End-diastolic pressure and early active relaxation, markers of diastolic function, were normal at rest. However, preload fell at peak stress (p<0.008) while pulmonary vascular resistance (PVR) was low throughout. This group of HLHS patients demonstrated a fall in SV at peak stress, coinciding with a fall in preload., Conclusions: Markers of systolic and diastolic function remained normal. Failure to adequately fill the ventricle implies a ceiling of maximal flow through the Fontan circuit despite low PVR., (Copyright © 2016. Published by Elsevier B.V.)

Objectives: The Norwood procedure for hypoplastic left heart syndrome (HLHS) is performed either via a right ventricle-to-pulmonary artery (RVPA) conduit or a modified Blalock-Taussig (MBT) shunt. Cardiac magnetic resonance (CMR) data was used to assess the effects of the RVPA conduit on ventricular shape and function through a computational analysis of anatomy and assessment of indices of strain., Methods: A retrospective analysis of 93 CMR scans of subjects with HLHS was performed (59 with MBT shunt, 34 with RVPA conduit), incorporating data at varying stages of surgery from two congenital centres. Longitudinal and short-axis cine images were used to create a computational cardiac atlas and assess global strain., Results: Those receiving an RVPA conduit had significant differences (P< 0.0001) in the shape of the RV corresponding to increased ventricular dilatation (P = 0.001) and increased sphericity (P = 0.006). Differences were evident only following completion of stage II surgery. Despite preserved ejection fraction in both groups, functional strain in the RVPA conduit group compared with that in the MBT shunt group was reduced across multiple ventricular axes, including a reduced systolic longitudinal strain rate (P< 0.0001), reduced diastolic longitudinal strain rate (P = 0.0001) and reduced midventricular systolic circumferential strain (P < 0.0001)., Conclusions: Computational modelling analysis reveals differences in ventricular remodelling in patients with HLHS undergoing an RVPA conduit insertion with focal scarring and volume loading leading to decreased functional markers of strain. The need for continued surveillance is warranted, as deleterious effects may not become apparent until later years., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Opinion Statement: Cardiac magnetic resonance imaging (CMR) is an important imaging modality in the evaluation of congenital heart diseases (CHD). CMR has several strengths including good spatial and temporal resolutions, wide field-of-view, and multi-planar imaging capabilities. CMR provides significant advantages for imaging in CHD through its ability to measure function, flow and vessel sizes, create three-dimensional reconstructions, and perform tissue characterization, all in a single imaging study. Thus, CMR is the most comprehensive imaging modality available today for the evaluation of CHD. Newer MRI sequences and post-processing tools will allow further development of quantitative methods of analysis, and opens the door for risk stratification in CHD. CMR also can interface with computer modeling, 3D printing, and other methods of understanding the complex anatomic and physiologic relationships in CHD.

Background: The use of gadolinium contrast agents in cardiovascular magnetic resonance is well-established and serves to improve both vascular imaging as well as enable late gadolinium enhancement (LGE) imaging for tissue characterization. Currently, gadofosveset trisodium, an intravascular contrast agent, combined with a three-dimensional inversion recovery balanced steady state free precession (3D IR bSSFP) sequence, is commonly used in pediatric cardiac imaging and yields excellent vascular imaging, but cannot be used for late gadolinium enhancement. Gadofosveset use remains limited in clinical practice, and manufacture was recently halted, thus an alternative is needed to allow 3D IR bSSFP and LGE in the same study., Methods: Here we propose a protocol to give a bolus of 0.1 mL/kg = 0.1 mmol/kg gadobutrol (GADAVIST/GADOVIST) for time-resolved magnetic resonance angiography (MRA). Subsequently, 0.1 mmol/kg is diluted up to 5 or 7.5 mL with saline and then loaded into intravenous tubing connected to the patient. A 0.5 mL short bolus is infused, then a slow infusion is given at 0.02 or 0.03 mL/s. Image navigated (iNAV) 3D IR bSSFP imaging is initiated 45-60 s after the initiation of the infusion, with a total image acquisition time of ~5 min. If necessary, LGE imaging using phase sensitive inversion recovery reconstruction (PSIR) is performed at 10 min after the infusion is initiated., Results: We have successfully performed the above protocol with good image quality on 10 patients with both time-resolved MRA and 3D IR bSSFP iNAV imaging. Our initial attempts to use pencil beam respiratory navigation failed due to signal labeling in the liver by the navigator. We have also performed 2D PSIR LGE successfully, with both LGE positive and LGE negative results., Conclusion: A bolus of gadobutrol, followed later by a slow infusion, allows time-resolved MRA, 3D IR bSSFP using the iNAV navigation technique, and LGE imaging, all in a single study with a single contrast agent.

Three-dimensional printing has an increasing number of clinical applications in pediatric cardiology. Time required for dataset segmentation and conversion to stereolithography (STL) format remains a significant limitation. We investigated the impact of semi-automated cardiovascular-specific segmentation software on time and reproducibility of segmentation. Magnetic resonance angiograms (MRAs) of 19 patients undergoing intervention for right ventricular outflow lesions were segmented to demonstrate the right heart. STLs were created by two independent clinicians using semi-automated cardiovascular segmentation (SAS) and traditional manual segmentation (MS). Time was recorded and geometric STL disagreement was determined (0 % = no disagreement, 100 % = complete disagreement). MRA datasets were categorized as clean when only right heart structures were present in the MRA, or contaminated when left heart structures were also present and required removal. Eighteen (seven clean and 11 contaminated) cases were successfully segmented with both methods. Time to STL for clean datasets was faster with MS than SAS [median 209 s (IQR 192-252) vs. 296 s (272-317), p = 0.018] while contaminated datasets were faster with SAS [455 s (384-561) vs. 866 s (310-1429), p = 0.033]. Interobserver STL geometric disagreement was significantly lower using SAS than MS overall (0.70 ± 1.15 % vs. 1.31 ± 1.52 %, p = 0.030), and for the contaminated subset (0.81 ± 1.08 % vs. 1.75 ± 1.57 %, p = 0.036). Most geometric disagreement occurred at areas where left heart contamination was removed. Semi-automated segmentation was faster and more reproducible for contaminated datasets, while MS was faster but equally reproducible for clean datasets. Semi-automated segmentation methods are preferable for contaminated datasets and continued refinement of these tools should be supported.

Background: The purpose of this study was to evaluate a recently developed two-dimensional (2D) image-based navigation approach (iNAVG+C ) combined with respiratory bellows gating for CMRA in patients with congenital heart disease., Methods: Nine healthy volunteers (mean age 32 ± 6 years [standard deviation]) and 29 patients (28 ± 9 years) were scanned on a 1.5 Tesla clinical scanner using iNAV(G+C) motion compensated T2prepared CMRA, and the conventional 1D NAV approach. Scan time was recorded for each CMRA scan. An image quality score was given to each coronary artery from (0, uninterpretable; to 4, excellent image quality). Additionally, vessel sharpness of each coronary artery was measured., Results: Average scan time was significantly shorter (P < 0.01) using the proposed iNAVC+G approach (7:57 ± 1:34) compared with 1D NAV (9:15 ± 3:02). Improved visual scores of the right coronary artery (iNAV(G+C) : 4,3,4 (median, 25th percentile, 75th percentile) versus 1D NAV: 3,3,4; P < 0.001) and left anterior descending artery (iNAV(G+C) : 3,3,4 versus 1D NAV: 3,2,3; P < 0.001) were obtained using iNAV(G+C) compared with 1D NAV as well as an increased vessel sharpness of the right coronary artery (iNAV(G+C) : 65.3% ± 6.6% (mean ± standard deviation) versus 1D NAV: 60.2% ± 11.4%; P < 0.05) and left anterior descending artery (iNAV(G+C) : 63.2% ± 6.7% versus 1D NAV: 58.3% ± 9.5%; P < 0.05)., Conclusion: Image-based navigation in combination with respiratory bellows gating allows for more robust suppression of respiratory motion artifacts for whole-heart CMRA compared with conventional 1D NAV as images can be acquired in a shorter time and with improved image quality., (© 2015 Wiley Periodicals, Inc.)

Cardiovascular magnetic resonance is a non-invasive imaging modality which is emerging as important tool for the investigation and management of pediatric cardiovascular disease. In this review we describe the key technical and practical differences between scanning children and adults, and highlight some important considerations that must be taken into account for this patient population. Using case examples commonly seen in clinical practice, we discuss the important clinical applications of cardiovascular magnetic resonance, and briefly highlight key future developments in this field.

Background: The purpose of this study was to see how coronary magnetic resonance angiography (CMRA) compared to echocardiography for the detection of coronary artery origins and to compare CMRA measurements for coronary dimensions in children with published echocardiographic reference values., Methods: Enrolled patients underwent dual cardiac phase CMRA and echocardiography under the same anesthetic. Echocardiographic measurements of the right coronary artery (RCA), left anterior descending (LAD) and left main (LM) were made. CMRA dimensions were assessed manually at the same points as the echocardiographic measurements. The number of proximal LAD branches imaged was also recorded in order to give an estimate of distal coronary tree visualization., Results: Fifty patients (24 boys, mean age 4.0 years (range 18 days to 18 years)) underwent dual-phase CMRA. Coronary origins were identified in 47/50 cases for CMRA (remaining 3 were infants aged 3, 9 and 11 months). In comparison, origins were identified in 41/50 cases for echo (remaining were all older children). CMRA performed better than echocardiography in terms of distal visualization of the coronary tree (median 1 LAD branch vs. median 0; p = 0.001). Bland-Altman plots show poor agreement between echocardiography and CMRA for coronary measurements. CMRA measurements did vary according to cardiac phase (systolic mean 1.90, s.d. 0.05 mm vs. diastolic mean 1.84, s.d. 0.05 mm; p = 0.002)., Conclusions: Dual-phase CMRA has an excellent (94 %) success rate for the detection of coronary origins in children. Newborn infants remain challenging and echocardiography remains the accepted imaging modality for this age group. Echocardiographic reference ranges are not applicable to CMRA measurements as agreement was poor between modalities. Future coronary reference values, using any imaging modality, should quote the phase in which it was measured.

This article provides expert opinion on the use of cardiovascular magnetic resonance (CMR) in young patients with congenital heart disease (CHD) and in specific clinical situations. As peculiar challenges apply to imaging children, paediatric aspects are repeatedly discussed. The first section of the paper addresses settings and techniques, including the basic sequences used in paediatric CMR, safety, and sedation. In the second section, the indication, application, and clinical relevance of CMR in the most frequent CHD are discussed in detail. In the current era of multimodality imaging, the strengths of CMR are compared with other imaging modalities. At the end of each chapter, a brief summary with expert consensus key points is provided. The recommendations provided are strongly clinically oriented. The paper addresses not only imagers performing CMR, but also clinical cardiologists who want to know which information can be obtained by CMR and how to integrate it in clinical decision-making.

The aim of this prospective study is to compare the performance of 2D time-resolved phase-contrast (PC) MRI prior to and after the administration of an intravascular (gadofosveset-trisodium) and extravascular (gadopentetate-dimeglumine) contrast agent in the same patient in the cardiovascular system. This study was approved by the ethics committee (Study-Number-07/Q0704/2) and registered with the MedicinesAndHealthcareProductsRegulatoryAgency (MHRA-Study-Number-28482/0002/001-0001, EudraCT-Number-2006-007042). All patients signed an informed consent. 20 patients were examined using a 1.5T MR-scanner and 32-channel-coil-technology. Gadopentetate-dimeglumine (GdD) and gadofosveset-trisodium (GdT) were administered in the same patient on consecutive days. Image quality, velocity-to-noise-ratios (VNRs) and standard-deviation of blood-flow-velocities (phase-noise) were compared between GdT, GdD and non-contrast-enhanced imaging. On both days pre- and post-contrast-scans were performed. The administration of GdT significantly improved the delineation of the perfused lumen and the VNR compared to GdD and non-contrast-enhanced imaging. Standard deviations of through-plane and in-plane velocity-measurements (phase-noise) were significantly reduced after GdT administration (p < 0.05). No significant differences (p > 0.05) were measured regarding absolute flow values prior to and after the administration of GdD and GdT. PC flow imaging benefits from the administration of an intravascular contrast agent by improving the delineation of the perfused lumen and reducing phase noise in flow measurements.

It has been shown that sublingual nitroglycerin (NTG) improves image quality of coronary lumen magnetic resonance angiography. Our aim was to investigate the influence of NTG on coronary lumen and vessel wall image quality using a combined, single sequence approach (i-T2prep), which is able to image both within the known time frame of action of NTG. Ten healthy volunteers underwent right coronary artery lumen and vessel wall imaging using the i-T2prep sequence before and after administration of NTG. Image quality was assessed qualitatively and quantitatively. Diameter, length and wall thickness were also measured using dedicated semi-automatic software. NTG induced coronary vasodilatation (lumen diameter increased from 2.16 ± 0.32 to 2.52 ± 0.59 mm; p = 0.036). As a result, visualized lumen length (9.8 ± 2.6 to 11.4 ± 3.3 cm; p = 0.025) and qualitative lumen image quality (median 3 (interquartile range 2-3.25) vs. median 3 (interquartile range 3-4); p = 0.046) both improved. Vessel wall imaging also demonstrated a significant improvement in vessel wall sharpness after NTG (24.8 vs. 27.3 %; p = 0.036). This study demonstrates the benefits of NTG for coronary lumen and vessel wall imaging using a combined sequence, i-T2prep. The methodology described here has great potential for future pathophysiological studies.

An updated version of the European Association of Cardiovascular Imaging (EACVI) Core Syllabus for the European Cardiovascular Magnetic Resonance (CMR) Certification Exam is now available online. The syllabus lists key elements of knowledge in CMR. It represents a framework for the development of training curricula and provides expected knowledge-based learning outcomes to the CMR trainees, in particular those intending to demonstrate CMR knowledge in the European CMR exam, a core requirement in the CMR certification process., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.)

Background: The clinical applicability of time-resolved 3D flow cardiovascular magnetic resonance (CMR) remains compromised by the long scan times associated with phase-contrast imaging. The present work demonstrates the applicability of 8-fold acceleration of Cartesian time-resolved 3D flow CMR in 10 volunteers and in 9 patients with different congenital heart diseases (CHD). It is demonstrated that accelerated 3D flow CMR data acquisition and image reconstruction using k-t PCA (principal component analysis) can be implemented into clinical workflow and results are sufficiently accurate relative to conventional 2D flow CMR to permit for comprehensive flow quantification in CHD patients., Methods: The fidelity of k-t PCA was first investigated on retrospectively undersampled data for different acceleration factors and compared to k-t SENSE and fully sampled reference data. Subsequently, k-t PCA with 8-fold nominal undersampling was applied on 10 healthy volunteers and 9 CHD patients on a clinical 1.5 T MR scanner. Quantitative flow validation was performed in vessels of interest on the 3D flow datasets and compared to 2D through-plane flow acquisitions. Particle trace analysis was used to qualitatively visualise flow patterns in patients., Results: Accelerated time-resolved 3D flow data were successfully acquired in all subjects with 8-fold nominal scan acceleration. Nominal scan times excluding navigator efficiency were on the order of 6 min and 7 min in patients and volunteers. Mean differences in stroke volume in selected vessels of interest were 2.5 ± 8.4 ml and 1.63 ± 4.8 ml in volunteers and patients, respectively. Qualitative flow pattern analysis in the time-resolved 3D dataset revealed valuable insights into hemodynamics including circular and helical patterns as well as flow distributions and origin in the Fontan circulation., Conclusion: Highly accelerated time-resolved 3D flow using k-t PCA is readily applicable in clinical routine protocols of CHD patients. Nominal scan times of 6 min are well tolerated and allow for quantitative and qualitative flow assessment in all great vessels.

Kawasaki disease (KD) is a vasculitis affecting the coronary and systemic arteries. Myocardial inflammation is also a common finding in KD post-mortem evaluation during the acute phase of the disease. Coronary artery aneurysms (CAAs) develop in 15-25% of untreated children. Although 50-70% of CAAs resolve spontaneously 1-2 years after the onset of KD, the remaining unresolved CAAs can develop stenotic lesions at either their proximal or distal end and can develop thrombus formation leading to ischemia and/or infarction. Cardiovascular magnetic resonance (CMR) has the ability to perform non-invasive and radiation-free evaluation of the coronary artery lumen. Recently tissue characterization of the coronary vessel wall was provided by CMR. It can also image myocardial inflammation, ischemia and fibrosis. Therefore CMR offers important clinical information during the acute and chronic phase of KD. In the acute phase, it can identify myocardial inflammation, microvascular disease, myocardial infarction, deterioration of left ventricular function, changes of the coronary artery lumen and changes of the coronary artery vessel wall. During the chronic phase, CMR imaging might be of value for risk stratification and to guide treatment.

Purpose: To investigate changes in image quality and observer variance between rest and higher-dose dobutamine stress MR imaging (DS-MR) in tetralogy of Fallot (TOF) patients and in a group of normal healthy volunteers using both axial and short axis orientation for cardiac volumetric assessment., Materials and Methods: Eighteen adult patients (age 34 ± 13) with TOF and severe pulmonary regurgitation and 10 healthy volunteers underwent ventricular MR volumetry at baseline and during dobutamine infusion of 10 μg/kg/min and 20 μg/kg/min. Inter-observer and intra-observer agreement was calculated by coefficient of variance (COV)., Results: Inter-observer comparison showed good agreement for left ventricle (LV) and right ventricle (RV) end-diastolic volumes at rest and both stress levels in TOF patients (axial geometry) and in normal volunteers (short axis). During dobutamine stress, the COV in TOF patients increased for LV end-systolic volume (LV-ESV) with each level, suggesting less agreement between observers. In contrast, RV-ESV was much more comparable with a COV < 10 at each condition. All volumetric measurements in normal volunteers showed good inter-observer agreement. Excellent intra-observer agreement could be seen for all volumetric parameters with COV levels below 7., Conclusion: Volumetric assessment during DS-MR shows excellent inter-observer agreement, except for LV-ESV in the TOF patients at higher doses of dobutamine. The axial geometry appears to be reproducible for assessment of RV parameters, and could be considered superior to short axis imaging in patients with repaired TOF., (Copyright © 2013 Wiley Periodicals, Inc.)

Objective: The study aims to assess whether the increasing use of cardiovascular magnetic resonance imaging in place of diagnostic cardiac catheterization in the management of pediatric patients with congenital heart disease has had an impact on pediatric cardiac care., Design: Retrospective analysis of data was used., Setting: The study was performed at the Evelina Children's Hospital Cardiology Department. PATIENTS. : Elective diagnostic cardiac catheterization or magnetic resonance imaging (MRI) from 2005-2010 are included (n = 896)., Outcome Measures: Indication, length of stay, and incidence of complications were recorded. In cases used to plan surgery, 30-day survival following the procedure was recorded. Surgical outcomes were compared between the two groups. Surgical outcomes planned using MRI were compared with national outcomes from Congenital Cardiac Audit Database., Results: For catheterizations (50 patients, [31 male, median age 3 years, interquartile range 1 to 12]), median hospital stay was 1 day (interquartile range 0 to 3), and complications occurred in 11 (22%). Median hospital stay for MRI (846 patients [517 male, median age 3 years, interquartile range 0 to 9]) was significantly shorter: 0 days (interquartile range 0 to 1, P <.001), with fewer complications (16 [1.9%], P <.0001). Twenty-four catheter and 283 MRI patients underwent surgery within 18 months. One catheter patient (2.0%) and four MRI patients (1.4%) died within 30 days (P =.48)., Conclusion: Replacing catheterization with cardiovascular magnetic resonance imaging has resulted in reduced rates of complication and shorter hospital stays without a significant impact on surgical outcome., (© 2013 Wiley Periodicals, Inc.)

Cardiovascular magnetic resonance (CMR) has taken on an increasingly important role in the diagnostic evaluation and pre-procedural planning for patients with congenital heart disease. This article provides guidelines for the performance of CMR in children and adults with congenital heart disease. The first portion addresses preparation for the examination and safety issues, the second describes the primary techniques used in an examination, and the third provides disease-specific protocols. Variations in practice are highlighted and expert consensus recommendations are provided. Indications and appropriate use criteria for CMR examination are not specifically addressed.

Objectives: This study sought to investigate changes in magnetic resonance imaging (MRI) ventricular volumes and vascular dimensions before hemi-Fontan (HF) and before total cavopulmonary connection (TCPC) in children with hypoplastic left heart syndrome (HLHS)., Background: The systemic right ventricle (RV) in HLHS is subject to significant changes in volume loading throughout the surgical stages of palliation, particularly after the HF., Methods: Fifty-eight patients had paired pre-HF and pre-TCPC MRI for assessment of changes of RV volumes, neoaortic flow, and vascular dimensions., Results: Comparison of pre-HF and pre-TCPC MRI results showed a decrease of indexed RV end-diastolic volume and end-systolic volume (98 ml/m(2) to 87 ml/m(2) and 50 ml/m(2) to 36 ml/m(2), respectively) with stroke volume remaining constant (49 ml/m(2) vs. 51 ml/m(2)), leading to an increased RV ejection fraction (51% vs. 59%). These findings persisted after excluding the 3 patients who underwent tricuspid valve repair as part of their HF procedure. Indexed RV end-diastolic volume plotted against neoaortic stroke volume demonstrated a Frank-Starling-like curve that shifted upward after HF. The indexed distal left and right cross-sectional pulmonary artery areas were reduced after HF., Conclusions: In HLHS, serial MRI shows the adaptation of the systemic RV after HF with volume reduction in the context of a preserved stroke volume and an increased ejection fraction. The staged palliation in HLHS may be a risk factor particularly for reduced left pulmonary artery growth in itself as no factors investigated in this study were found to significantly impact on this., (Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Coronary allograft vasculopathy is the leading cause of late death after heart transplantation in children. It is poorly detected by conventional angiography. Intravascular ultrasound is invasive and costly. This study shows that magnetic resonance imaging (MRI) late gadolinium enhancement (LGE) of the coronary vessel wall can detect and grade coronary allograft vasculopathy., Methods and Results: Twenty-four children (10 male; age range, 9-17 years) underwent coronary angiography, intravascular ultrasound, and MRI. Maximal intimal thickness and mean intimal index were recorded. MRI included coronary magnetic resonance angiogram and LGE vessel wall imaging with 1.5 T (n=12) and 3.0 T (n=12). Ten healthy control subjects also underwent LGE MRI. Mean time posttransplantation was 5.5 years (range, 0.25-14 years). Seven patients had Stanford grade IV coronary allograft vasculopathy on intravascular ultrasound, 3 of whom had angiographic disease. Maximal intimal thickness and mean intimal index were 0.73±0.50 mm and 20.9±10.6%, respectively. On MRI, mean diameter of enhancement of vessel wall was 6.57±4.91 mm, and mean enhancement index (indexed to vessel lumen size) was 1.10±1.72. The control group showed little or no LGE. Correlation of LGE with maximal intimal thickness using the Pearson coefficient was 0.80 (P<0.001) and with mean intimal index was 0.92 (P<0.001). An MRI diameter >7.5 mm gave 86% sensitivity and 93% specificity., Conclusions: LGE scores correlate well with traditional intravascular ultrasound measures. These promising early results encourage larger-scale clinical studies to investigate whether LGE MRI will allow closer follow-up and better prevention of coronary allograft vasculopathy in children.

Background: Cardiovascular magnetic resonance (CMR) is the current gold standard for the assessment of left ventricular (LV) function. Repeated breath-holds are needed for standard multi-slice 2D cine steady-state free precession sequences (M2D-SSFP). Accelerated single breath-hold techniques suffer from low contrast between blood pool and myocardium. In this study an intravascular contrast agent was prospectively compared to an extravascular contrast agent for the assessment of LV function using a single-breath-hold 3D-whole-heart cine SSFP sequence (3D-SSFP)., Methods: LV function was assessed in fourteen patients on a 1.5 T MR-scanner (Philips Healthcare) using 32-channel coil technology. Patients were investigated twice using a 3D-SSFP sequence (acquisition time 18-25 s) after Gadopentetate dimeglumine (GdD, day 1) and Gadofosveset trisodium (GdT, day 2) administration. Image acquisition was accelerated using sensitivity encoding in both phase encoding directions (4xSENSE). CNR and BMC were both measured between blood and myocardium. The CNR incorporated noise measurements, while the BMC represented the coeffiancy between the signal from blood and myocardium [1]. Contrast to noise ratio (CNR), blood to myocardium contrast (BMC), image quality, LV functional parameters and intra-/interobserver variability were compared. A M2D-SSFP sequence was used as a reference standard on both days., Results: All 3D-SSFP sequences were successfully acquired within one breath-hold after GdD and GdT administration. CNR and BMC were significantly (p < 0.05) higher using GdT compared to GdD, resulting in an improved endocardial definition. Using 3D-SSFP with GdT, Bland-Altman plots showed a smaller bias (95% confidence interval LVEF: 9.0 vs. 23.7) and regression analysis showed a stronger correlation to the reference standard (R2 = 0.92 vs. R2 = 0.71), compared to 3D-SSFP with GdD., Conclusions: A single-breath-hold 3D-whole-heart cine SSFP sequence in combination with 32-channel technology and an intravascular contrast agent allows for the accurate and fast assessment of LV function.

A 9-month-old girl presented with cyanosis after correction of total anomalous pulmonary venous return (TAPVR) to the coronary sinus in the neonatal period. During corrective surgery, the right superior vena cava (RSVC) was damaged and re-anastomosed to the right atrium. Echocardiography showed increased flow velocity in the pulmonary venous confluence. Therefore, pulmonary venous obstruction was suspected. However, subsequent cardiac MRI revealed a stenosed RSVC as well as a dilated left superior vena cava (LSVC) draining from the left innominate vein into the pulmonary venous confluence. The re-recruited LSVC drained deoxygenated blood into the systemic circulation, causing cyanosis. After uncomplicated placement of a stent in the narrowed RSVC and occlusion of the LSVC, transcutaneous saturations normalised immediately.