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.

Assessing Left Ventricular Pathology in Patients with Ebstein Anomaly Using Cardiovascular Magnetic Resonance: Looking Past the Right Heart.

Ebstein Anomaly (EA) is a malformation of the right heart, but there is data to suggest that the left ventricle (LV) can suffer from intrinsic structural and functional abnormalities which affect surgical outcomes. The LV in patients with EA is hypertrabeculated with abnormalities in LV function and strain. In this retrospective single-center study, patients with EA who underwent pre-operative cardiac MRI (CMR) between the periods of 2014-2024 were included along with a group of healthy-age-matched controls. Left ventricular and right ventricular volume, function and strain analyses were performed on standard SSFP imaging. LV noncompacted: compacted (NC/C) ratio and the displacement index of the tricuspid valve were measured. Forty-seven EA patients were included with mean age of 21.0 ± 17.6 years. Seventeen EA patients (36%) had mild pre-operative LV dysfunction on CMR and 1 (2.1%) had moderate LV dysfunction. Out of these 18 patients with LV dysfunction, only 2 were detected to have dysfunction on Echocardiogram. The global circumferential and longitudinal strain were significantly lower in the reduced LVEF group compared to those with preserved LVEF (- 14.8% vs. - 17%, p = 0.02 and - 11.9% vs. - 15.0%; p = 0.05; respectively) on CMR. A single EA patient met criteria for LVNC with a maximal NC/C ratio > 2.3. There was no statistically significant difference in NC/C ratio in the EA population (1.4 ± 0.6) vs. controls (1.1 ± 0.2), p = 0.17. There was an inverse correlation of LV ejection fraction with right ventricular end-diastolic volume and displacement index. All patients underwent the Da Silva Cone procedure at our center. Patients with preoperative LV dysfunction had longer duration of epinephrine use in the immediate postoperative period (33.7 ± 21.4 vs 10.2 ± 25.6 h, p = 0.02) and longer length of hospital stay (6.3 ± 3.2 vs 4.4 ± 1.2 days, p = 0.01). This is the largest study to date to evaluate preoperative LV structure and function in EA patients by CMR. In this cohort of 47 patients, preoperative LV dysfunction is fairly common and CMR has high sensitivity in detecting LV dysfunction as compared to Echo. True LV non-compaction was rare in this cohort. The presence of LV dysfunction is relevant to perioperative management and further study with larger cohorts and longer follow up are necessary.

Multimodality Imaging in Ebstein Anomaly.

Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine.

Statistical shape modeling reveals the link between right ventricular shape, hemodynamic force, and myocardial function in patients with repaired tetralogy of Fallot.

Patients with repaired tetralogy of Fallot (rTOF) can develop chronic pulmonary insufficiency (PI) with right ventricular (RV) dilation, progressive RV dysfunction, and decreased exercise capacity. Pulmonary valve replacement (PVR) can help reduce the amount of PI and RV dilation; however, optimal timing remains controversial; a better understanding of rTOF pathophysiology is of fundamental importance to inform clinical management of patients with rTOF and optimal timing of PVR. In this study, we hypothesize a tight interplay between RV shape, intracardiac biomechanics, and ventricular function in patients with rTOF. To explore this hypothesis and derive quantitative measures, we combined statistical shape modeling with physics-based analysis of in vivo 4D flow data in 36 patients with rTOF. Our study demonstrated for the first time a correlation between regional RV shape variations, hemodynamic forces (HDF), and clinical dysfunction in patients with rTOF. The main findings of this work include 1) general increase in RV size, due to both volume overload and physiological growth, correlated with decrease in strain magnitude in the respective directions, and with increased QRS; 2) regional PI-induced remodeling accounted for ∼10% of the shape variability of the population, and was associated with increased diastolic HDF along the diaphragm-to-right ventricular outflow tract (RVOT) direction, resulting in a net RV deformation along the same direction and decreased tricuspid annular plane systolic excursion (TAPSE); and 3) three shape modes independently correlated with systolic HDF and exercise capacity. Identification of patients based on the shape variations described in this study could help identify those at risk for irreversible dysfunction and guide optimal timing of PVR.NEW & NOTEWORTHY We combine statistical shape modeling with physics-based analysis of 4D flow data to elucidate the interplay between RV shape, hemodynamic forces, and clinical dysfunction in repaired tetralogy of Fallot. We are the first to show that ventricular remodeling is related to hemodynamic force magnitude and direction, global and regional functional parameters, and exercise intolerance. Identification of patients based on the shape variations described in this study could help identify those at risk for irreversible dysfunction.

Impact of ferumoxytol vs gadolinium on 4D flow cardiovascular magnetic resonance measurements in small children with congenital heart disease.

BACKGROUND: Cardiovascular magnetic resonance (CMR) allows for time-resolved three-dimensional phase-contrast (4D Flow) analysis of congenital heart disease (CHD). Higher spatial resolution in small infants requires thinner slices, which can degrade the signal. Particularly in infants, the choice of contrast agent (ferumoxytol vs. gadolinium) may influence 4D Flow CMR accuracy. Thus, we investigated the accuracy of 4D Flow CMR measurements compared to gold standard 2D flow phase contrast (PC) measurements in ferumoxytol vs. gadolinium-enhanced CMR of small CHD patients with shunt lesions. METHODS: This was a retrospective study consisting of CMR studies from complex CHD patients less than 20 kg who had ferumoxytol or gadolinium-enhanced 4D Flow and standard two-dimensional phase contrast (2D-PC) flow collected. 4D Flow clinical software (Arterys) was used to measure flow in great vessels, systemic veins, and pulmonary veins. 4D Flow accuracy was defined as percent difference or correlation against conventional measurements (2D-PC) from the same vessels. Subgroup analysis was performed on two-ventricular vs single-ventricular CHD, arterial vs venous flow, as well as low flows (defined as < 1.5 L/min) in 1V CHD. RESULTS: Twenty-one ferumoxytol-enhanced and 23 gadolinium-enhanced CMR studies were included, with no difference in age (2.1 ± 1.6 vs. 2.3 ± 1.9 years, p = 0.70), patient body surface area (0.50 ± 0.2 vs. 0.52 ± 0.2 m2, p = 0.67), or vessel diameter (11.4 ± 5.2 vs. 12.4 ± 5.6 mm, p = 0.22). Ten CMR studies with single ventricular CHD were included. Overall, ferumoxytol-enhanced 4D flow CMR measurements demonstrated less percent difference to 2D-PC when compared to gadolinium-enhanced 4D Flow CMR studies. In subgroup analyses of arterial vs. venous flows (high velocity vs. low velocity) and low flow in single ventricle CHD, ferumoxytol-enhanced 4D Flow CMR measurements had stronger correlation to 2D-PC CMR. The contrast-to-noise ratio (CNR) in ferumoxytol-enhanced studies was higher than the CNR in gadolinium-enhanced studies. CONCLUSIONS: Ferumoxytol-enhanced 4D Flow CMR has improved accuracy when compared to gadolinium 4D Flow CMR, particularly for infants with small vessels in CHD.

Cardiac echocardiogram findings of severe acute respiratory syndrome coronavirus-2-associated multi-system inflammatory syndrome in children.

BACKGROUND: A novel paediatric disease, multi-system inflammatory syndrome in children, has emerged during the 2019 coronavirus disease pandemic. OBJECTIVES: To describe the short-term evolution of cardiac complications and associated risk factors in patients with multi-system inflammatory syndrome in children. METHODS: Retrospective single-centre study of confirmed multi-system inflammatory syndrome in children treated from 29 March, 2020 to 1 September, 2020. Cardiac complications during the acute phase were defined as decreased systolic function, coronary artery abnormalities, pericardial effusion, or mitral and/or tricuspid valve regurgitation. Patients with or without cardiac complications were compared with chi-square, Fisher's exact, and Wilcoxon rank sum. RESULTS: Thirty-nine children with median (interquartile range) age 7.8 (3.6-12.7) years were included. Nineteen (49%) patients developed cardiac complications including systolic dysfunction (33%), valvular regurgitation (31%), coronary artery abnormalities (18%), and pericardial effusion (5%). At the time of the most recent follow-up, at a median (interquartile range) of 49 (26-61) days, cardiac complications resolved in 16/19 (84%) patients. Two patients had persistent mild systolic dysfunction and one patient had persistent coronary artery abnormality. Children with cardiac complications were more likely to have higher N-terminal B-type natriuretic peptide (p = 0.01), higher white blood cell count (p = 0.01), higher neutrophil count (p = 0.02), severe lymphopenia (p = 0.05), use of milrinone (p = 0.03), and intensive care requirement (p = 0.04). CONCLUSION: Patients with multi-system inflammatory syndrome in children had a high rate of cardiac complications in the acute phase, with associated inflammatory markers. Although cardiac complications resolved in 84% of patients, further long-term studies are needed to assess if the cardiac abnormalities (transient or persistent) are associated with major cardiac events.

Moving beyond size: vorticity and energy loss are correlated with right ventricular dysfunction and exercise intolerance in repaired Tetralogy of Fallot.

BACKGROUND: The global effect of chronic pulmonary regurgitation (PR) on right ventricular (RV) dilation and dysfunction in repaired Tetralogy of Fallot (rTOF) patients is well studied by cardiovascular magnetic resonance (CMR). However, the links between PR in the RV outflow tract (RVOT), RV dysfunction and exercise intolerance are not clarified by conventional measurements. Not all patients with RV dilation share the same intracardiac flow characteristics, now measurable by time resolved three-dimensional phase contrast imaging (4D flow). In our study, we quantified regional vorticity and energy loss in rTOF patients and correlated these parameters with RV dysfunction and exercise capacity. METHODS: rTOF patients with 4D flow datasets were retrospectively analyzed, including those with transannular/infundibular repair and conduit repair. Normal controls and RV dilation patients with atrial-level shunts (Qp:Qs > 1.2:1) were included for comparison. 4D flow was post-processed using IT Flow (Cardioflow, Japan). Systolic/diastolic vorticity (ω, 1/s) and viscous energy loss (VEL, mW) in the RVOT and RV inflow were measured. To characterize the relative influence of diastolic vorticity in the two regions, an RV Diastolic Vorticity Quotient (ωRVOT-Diastole/ωRV Inflow-Diastole, RV-DVQ) was calculated. Additionally, RVOT Vorticity Quotient (ωRVOT-Diastole/ωRVOT-Systole, RVOT-VQ) and RVOT Energy Quotient (VELRVOT-Diastole/VELRVOT-Systole, RVOT-EQ) was calculated. In rTOF, measurements were correlated against conventional CMR and exercise stress test results. RESULTS: 58 rTOF patients, 28 RV dilation patients and 12 controls were included. RV-DVQ, RVOT-VQ, and RVOT-EQ were highest in rTOF patients with severe PR compared to rTOF patients with non-severe PR, RV dilation and controls (p < 0.001). RV-DVQ positively correlated with RV end-diastolic volume (0.683, p < 0.001), PR fraction (0.774, p < 0.001) and negatively with RV ejection fraction (- 0.521, p = 0.003). Both RVOT-VQ, RVOT-EQ negatively correlated with VO2-max (- 0.587, p = 0.008 and - 0.617, p = 0.005) and % predicted VO2-max (- 0.678, p = 0.016 and - 0.690, p = 0.001). CONCLUSIONS: In rTOF patients, vorticity and energy loss dominate the RVOT compared to tricuspid inflow, correlating with RV dysfunction and exercise intolerance. These 4D flow-based measurements may be sensitive biomarkers to guide surgical management of rTOF patients.

Altered hemodynamics by 4D flow cardiovascular magnetic resonance predict exercise intolerance in repaired coarctation of the aorta: an in vitro study.

BACKGROUND: Coarctation of the aorta (CoA) is associated with decreased exercise capacity despite successful repair. Altered flow patterns have been identified due to abnormal aortic arch geometry. Our previous work demonstrated aorta size mismatch to be associated with exercise intolerance in this population. In this study, we studied aortic flow patterns during simulations of exercise in repaired CoA using 4D flow cardiovascular magnetic resonance (CMR) using aortic replicas connected to an in vitro flow pump and correlated findings with exercise stress test results to identify biomarkers of exercise intolerance. METHODS: Patients with CoA repair were retrospectively analyzed after CMR and exercise stress test. Each aorta was manually segmented and 3D printed. Pressure gradient measurements from ascending aorta (AAo) to descending aorta (DAo) and 4D flow CMR were performed during simulations of rest and exercise using a mock circulatory flow loop. Changes in wall shear stress (WSS) and secondary flow formation (vorticity and helicity) from rest to exercise were quantified, as well as estimated DAo Reynolds number. Parameters were correlated with percent predicted peak oxygen consumption (VO2max) and aorta size mismatch (DAAo/DDAo). RESULTS: Fifteen patients were identified (VO2max 47 to 126% predicted). Pressure gradient did not correlate with VO2max at rest or exercise. VO2max correlated positively with the change in peak vorticity (R = 0.55, p = 0.03), peak helicity (R = 0.54, p = 0.04), peak WSS in the AAo (R = 0.68, p = 0.005) and negatively with peak WSS in the DAo (R = - 0.57, p = 0.03) from rest to exercise. DAAo/DDAo correlated strongly with change in vorticity (R = - 0.38, p = 0.01), helicity (R = - 0.66, p = 0.007), and WSS in the AAo (R = - 0.73, p = 0.002) and DAo (R = 0.58, p = 0.02). Estimated DAo Reynolds number negatively correlated with VO2max for exercise (R = - 0.59, p = 0.02), but not rest (R = - 0.28, p = 0.31). Visualization of streamline patterns demonstrated more secondary flow formation in aortic arches with better exercise capacity, larger DAo, and lower Reynolds number. CONCLUSIONS: There are important associations between secondary flow characteristics and exercise capacity in repaired CoA that are not captured by traditional pressure gradient, likely due to increased turbulence and inefficient flow. These 4D flow CMR parameters are a target of investigation to identify optimal aortic arch geometry and improve long term clinical outcomes after CoA repair.

Society for Cardiovascular Magnetic Resonance 2020 Case of the Week series.

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). Case of the week is a case series hosted on the SCMR website ( https://www.scmr.org ) that demonstrates the utility and importance of CMR in the clinical diagnosis and management of cardiovascular disease. Each case consists of the clinical presentation and a discussion of the condition and the role of CMR in diagnosis and guiding clinical management. The cases are all instructive and helpful in the approach to patient management. We present a digital archive of the 2020 Case of the Week series of 11 cases as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

Normal right and left ventricular volumes prospectively obtained from cardiovascular magnetic resonance in awake, healthy, 0- 12 year old children.

INTRODUCTION: Pediatric z scores are necessary to describe size and structure of the heart in growing children, however, development of an accurate z score calculator requires robust normal datasets, which are difficult to obtain with cardiovascular magnetic resonance (CMR) in children. Motion-corrected (MOCO) cines from re-binned, reconstructed real-time cine offer a free-breathing, rapid acquisition resulting in cines with high spatial and temporal resolution. In combination with child-friendly positioning and entertainment, MOCO cine technique allows for rapid cine volumetry in patients of all ages without sedation. Thus, our aim was to prospectively enroll normal infants and children birth-12 years for creation and validation of a z score calculator describing normal right ventricular (RV) and left ventricular (LV) size. METHODS: With IRB approval and consent/assent, 149 normal children successfully underwent a brief noncontrast CMR on a 1.5 T scanner including MOCO cines in the short axis, and RV and LV volumes were measured. 20% of scans were re-measured for interobserver variability analyses. A general linear modeling (GLM) framework was employed to identify and properly represent the relationship between CMR-based assessments and anthropometric data. Scatter plots of model fit and Akaike's information criteria (AIC) results were used to guide the choice among alternative models. RESULTS: A total of 149 subjects aged 22 days-12 years (average 5.1 ± 3.6 years), with body surface area (BSA) range 0.21-1.63 m2 (average 0.8 ± 0.35 m2) were scanned. All ICC values were > 95%, reflecting excellent agreement between raters. The model that provided the best fit of volume measure to the data included BSA with higher order effects and gender as independent variables. Compared with earlier z score models, there is important additional growth inflection in early toddlerhood with similar z score prediction in later childhood. CONCLUSIONS: Free-breathing, MOCO cines allow for accurate, reliable RV and LV volumetry in a wide range of infants and children while awake. Equations predicting fit between LV and RV normal values and BSA are reported herein for purposes of creating z scores. TRIAL REGISTRATION: clinicaltrials.gov NCT02892136, Registered 7/21/2016.

Motion-corrected cardiac MRI is associated with decreased anesthesia exposure in children.

BACKGROUND: The benefits of cardiac magnetic resonance imaging (MRI) in the pediatric population must be balanced with the risk and cost of anesthesia. Segmented imaging using multiple averages attempts to avoid breath-holds requiring general anesthesia; however, cardiorespiratory artifacts and prolonged scan times limit its use. Thus, breath-held imaging with general anesthesia is used in many pediatric centers. The advent of free-breathing, motion-corrected (MOCO) cines by real-time re-binned reconstruction offers reduced anesthesia exposure without compromising image quality. OBJECTIVE: This study evaluates sedation utilization in our pediatric cardiac MR practice before and after clinical introduction of free-breathing MOCO imaging for cine and late gadolinium enhancement. MATERIALS AND METHODS: In a retrospective study, patients referred for a clinical cardiac MR who would typically be offered sedation for their scan (n=295) were identified and divided into two eras, those scanned before the introduction of MOCO cine and late gadolinium enhancement sequences and those scanned following their introduction. Anesthesia use was compared across eras and disease-specific cohorts. RESULTS: The incidence of non-sedation studies performed in children nearly tripled following the introduction of MOCO imaging (25% [pre-MOCO] to 69% [post-MOCO], P<0.01), with the greatest effect in patients with simple congenital heart disease. Eleven percent of the post-MOCO cohort comprised infants younger than 3 months of age who could forgo sedation with the combination of MOCO imaging and a "feed-and-bundle" positioning technique. CONCLUSION: Implementation of cardiac MR with MOCO cine and late gadolinium enhancement imaging in a pediatric population is associated with significantly decreased sedation utilization.

X-ray fused with MRI guidance of pre-selected transcatheter congenital heart disease interventions.

OBJECTIVES: To determine whether X-ray fused with MRI (XFM) is beneficial for select transcatheter congenital heart disease interventions. BACKGROUND: Complex transcatheter interventions often require three-dimensional (3D) soft tissue imaging guidance. Fusion imaging with live X-ray fluoroscopy can potentially improve and simplify procedures. METHODS: Patients referred for select congenital heart disease interventions were prospectively enrolled. Cardiac MRI data was overlaid on live fluoroscopy for procedural guidance. Likert scale operator assessments of value were recorded. Fluoroscopy time, radiation exposure, contrast dose, and procedure time were compared to matched cases from our institutional experience. RESULTS: Forty-six patients were enrolled. Pre-catheterization, same day cardiac MRI findings indicated intervention should be deferred in nine patients. XFM-guided cardiac catheterization was performed in 37 (median age 8.7 years [0.5-63 years]; median weight 28 kg [5.6-110 kg]) with the following prespecified indications: pulmonary artery (PA) stenosis (n = 13), aortic coarctation (n = 12), conduit stenosis/insufficiency (n = 9), and ventricular septal defect (n = 3). Diagnostic catheterization showed intervention was not indicated in 12 additional cases. XFM-guided intervention was performed in the remaining 25. Fluoroscopy time was shorter for XFM-guided intervention cases compared to matched controls. There was no significant difference in radiation dose area product, contrast volume, or procedure time. Operator Likert scores indicated XFM provided useful soft tissue guidance in all cases and was never misleading. CONCLUSIONS: XFM provides operators with meaningful three-dimensional soft tissue data and reduces fluoroscopy time in select congenital heart disease interventions.

Computational Study of Pulmonary Flow Patterns After Repair of Transposition of Great Arteries.

Patients that undergo the arterial switch operation (ASO) to repair transposition of great arteries (TGA) can develop abnormal pulmonary trunk morphology with significant long-term complications. In this study, cardiovascular magnetic resonance was combined with computational fluid dynamics to investigate the impact of the postoperative layout on the pulmonary flow patterns. Three ASO patients were analyzed and compared to a volunteer control. Results showed the presence of anomalous shear layer instabilities, vortical and helical structures, and turbulent-like states in all patients, particularly as a consequence of the unnatural curvature of the pulmonary bifurcation. Streamlined, mostly laminar flow was instead found in the healthy subject. These findings shed light on the correlation between the post-ASO anatomy and the presence of altered flow features, and may be useful to improve surgical planning as well as the long-term care of TGA patients.

Anesthetic considerations for magnetic resonance imaging-guided right-heart catheterization in pediatric patients: A single institution experience.

Cardiac catheterization is an integral part of medical management for pediatric patients with congenital heart disease. Owing to age and lack of cooperation in children who need this procedure, general anesthesia is typically required. These patients have increased anesthesia risk secondary to cardiac pathology. Furthermore, multiple catheterization procedures result in exposure to harmful ionizing radiation. Magnetic resonance imaging-guided right-heart catheterization offers decreased radiation exposure and diagnostic imaging benefits over traditional fluoroscopy but potentially increases anesthetic complexity and risk. We describe our early experience with anesthetic techniques and challenges for pediatric magnetic resonance imaging-guided right-heart catheterization.

Respiratory variation in peak aortic velocity accurately predicts fluid responsiveness in children undergoing neurosurgery under general anesthesia.

The determination of fluid responsiveness in the critically ill child is of vital importance, more so as fluid overload becomes increasingly associated with worse outcomes. Dynamic markers of volume responsiveness have shown some promise in the pediatric population, but more research is needed before they can be adopted for widespread use. Our aim was to investigate effectiveness of respiratory variation in peak aortic velocity and pulse pressure variation to predict fluid responsiveness, and determine their optimal cutoff values. We performed a prospective, observational study at a single tertiary care pediatric center. Twenty-one children with normal cardiorespiratory status undergoing general anesthesia for neurosurgery were enrolled. Respiratory variation in peak aortic velocity (ΔVpeak ao) was measured both before and after volume expansion using a bedside ultrasound device. Pulse pressure variation (PPV) value was obtained from the bedside monitor. All patients received a 10 ml/kg fluid bolus as volume expansion, and were qualified as responders if stroke volume increased >15% as a result. Utility of ΔVpeak ao and PPV and to predict responsiveness to volume expansion was investigated. A baseline ΔVpeak ao value of greater than or equal to 12.3% best predicted a positive response to volume expansion, with a sensitivity of 77%, specificity of 89% and area under receiver operating characteristic curve of 0.90. PPV failed to demonstrate utility in this patient population. Respiratory variation in peak aortic velocity is a promising marker for optimization of perioperative fluid therapy in the pediatric population and can be accurately measured using bedside ultrasonography. More research is needed to evaluate the lack of effectiveness of pulse pressure variation for this purpose.

Acute Cardiac MRI Assessment of Radiofrequency Ablation Lesions for Pediatric Ventricular Arrhythmia: Feasibility and Clinical Correlation.

BACKGROUND: Arrhythmia ablation with current techniques is not universally successful. Inadequate ablation lesion formation may be responsible for some arrhythmia recurrences. Periprocedural visualization of ablation lesions may identify inadequate lesions and gaps to guide further ablation and reduce risk of arrhythmia recurrence. METHODS: This feasibility study assessed acute postprocedure ablation lesions by MRI, and correlated these findings with clinical outcomes. Ten pediatric patients who underwent ventricular tachycardia ablation were transferred immediately postablation to a 1.5T MRI scanner and late gadolinium enhancement (LGE) imaging was performed to characterize ablation lesions. Immediate and mid-term arrhythmia recurrences were assessed. RESULTS: Patient characteristics include median age 14 years (1-18 years), median weight 52 kg (11-81 kg), normal cardiac anatomy (n = 6), d-transposition of great arteries post arterial switch repair (n = 2), anomalous coronary artery origin post repair (n = 1), and cardiac rhabdomyoma (n = 1). All patients underwent radiofrequency catheter ablation of ventricular arrhythmia with acute procedural success. LGE was identified at the reported ablation site in 9/10 patients, all arrhythmia-free at median 7 months follow-up. LGE was not visible in 1 patient who had recurrence of frequent premature ventricular contractions within 2 hours, confirmed on Holter at 1 and 21 months post procedure. CONCLUSIONS: Ventricular ablation lesion visibility by MRI in the acute post procedure setting is feasible. Lesions identifiable with MRI may correlate with clinical outcomes. Acute MRI identification of gaps or inadequate lesions may provide the unique temporal opportunity for additional ablation therapy to decrease arrhythmia recurrence.

Radiation-free CMR diagnostic heart catheterization in children.

BACKGROUND: Children with heart disease may require repeated X-Ray cardiac catheterization procedures, are more radiosensitive, and more likely to survive to experience oncologic risks of medical radiation. Cardiovascular magnetic resonance (CMR) is radiation-free and offers information about structure, function, and perfusion but not hemodynamics. We intend to perform complete radiation-free diagnostic right heart catheterization entirely using CMR fluoroscopy guidance in an unselected cohort of pediatric patients; we report the feasibility and safety. METHODS: We performed 50 CMR fluoroscopy guided comprehensive transfemoral right heart catheterizations in 39 pediatric (12.7 ± 4.7 years) subjects referred for clinically indicated cardiac catheterization. CMR guided catheterizations were assessed by completion (success/failure), procedure time, and safety events (catheterization, anesthesia). Pre and post CMR body temperature was recorded. Concurrent invasive hemodynamic and diagnostic CMR data were collected. RESULTS: During a twenty-two month period (3/2015 - 12/2016), enrolled subjects had the following clinical indications: post-heart transplant 33%, shunt 28%, pulmonary hypertension 18%, cardiomyopathy 15%, valvular heart disease 3%, and other 3%. Radiation-free CMR guided right heart catheterization attempts were all successful using passive catheters. In two subjects with septal defects, right and left heart catheterization were performed. There were no complications. One subject had six such procedures. Most subjects (51%) had undergone multiple (5.5 ± 5) previous X-Ray cardiac catheterizations. Retained thoracic surgical or transcatheter implants (36%) did not preclude successful CMR fluoroscopy heart catheterization. During the procedure, two subjects were receiving vasopressor infusions at baseline because of poor cardiac function, and in ten procedures, multiple hemodynamic conditions were tested. CONCLUSIONS: Comprehensive CMR fluoroscopy guided right heart catheterization was feasible and safe in this small cohort of pediatric subjects. This includes subjects with previous metallic implants, those requiring continuous vasopressor medication infusions, and those requiring pharmacologic provocation. Children requiring multiple, serial X-Ray cardiac catheterizations may benefit most from radiation sparing. This is a step toward wholly CMR guided diagnostic (right and left heart) cardiac catheterization and future CMR guided cardiac intervention. TRIAL REGISTRATION: ClinicalTrials.gov NCT02739087 registered February 17, 2016.

Usage of 3D models of tetralogy of Fallot for medical education: impact on learning congenital heart disease.

BACKGROUND: Congenital heart disease (CHD) is the most common human birth defect, and clinicians need to understand the anatomy to effectively care for patients with CHD. However, standard two-dimensional (2D) display methods do not adequately carry the critical spatial information to reflect CHD anatomy. Three-dimensional (3D) models may be useful in improving the understanding of CHD, without requiring a mastery of cardiac imaging. The study aimed to evaluate the impact of 3D models on how pediatric residents understand and learn about tetralogy of Fallot following a teaching session. METHODS: Pediatric residents rotating through an inpatient Cardiology rotation were recruited. The sessions were randomized into using either conventional 2D drawings of tetralogy of Fallot or physical 3D models printed from 3D cardiac imaging data sets (cardiac MR, CT, and 3D echocardiogram). Knowledge acquisition was measured by comparing pre-session and post-session knowledge test scores. Learner satisfaction and self-efficacy ratings were measured with questionnaires filled out by the residents after the teaching sessions. Comparisons between the test scores, learner satisfaction and self-efficacy questionnaires for the two groups were assessed with paired t-test. RESULTS: Thirty-five pediatric residents enrolled into the study, with no significant differences in background characteristics, including previous clinical exposure to tetralogy of Fallot. The 2D image group (n = 17) and 3D model group (n = 18) demonstrated similar knowledge acquisition in post-test scores. Residents who were taught with 3D models gave a higher composite learner satisfaction scores (P = 0.03). The 3D model group also had higher self-efficacy aggregate scores, but the difference was not statistically significant (P = 0.39). CONCLUSION: Physical 3D models enhance resident education around the topic of tetralogy of Fallot by improving learner satisfaction. Future studies should examine the impact of models on teaching CHD that are more complex and elaborate.

Native T1 values identify myocardial changes and stratify disease severity in patients with Duchenne muscular dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked, inherited disorder causing dilated cardiomyopathy with variable onset and progression. Currently we lack objective markers of the effect of therapies targeted towards preventing progression of subclinical cardiac disease. Thus, our aim was to compare the ability of native T1 and extracellular volume (ECV) measurements to differentiate risk of myocardial disease in DMD and controls. METHODS: Twenty boys with DMD and 16 age/gender-matched controls without history predisposing to cardiac fibrosis, but with a clinical indication for cardiovascular magnetic resonance (CMR) evaluation, underwent CMR with contrast. Data points collected include left ventricular ejection fraction (LVEF), left ventricular mass, and presence of late gadolinium enhancement (LGE). Native T1, and ECV regional mapping were obtained using both a modified Look-Locker (MOLLI) and saturation recovery single shot sequence (SASHA) on a 1.5T scanner. Using ordinal logistic regression models, controlling for age and LVEF, LGE-free septal we evaluated the ability native T1 and ECV assessments to differentiate levels of cardiomyopathy. RESULTS: Twenty DMD subjects aged 14.4 ± 4 years had an LVEF of 56.3 ± 7.4 %; 12/20 had LGE, all confined to the lateral wall. Sixteen controls aged 16.1 ± 2.2 years had an LVEF 60.4 ± 5.1 % and no LGE. Native T1 and ECV values were significantly higher in the DMD group (p < 0.05) with both MOLLI and SASHA imaging techniques. Native T1 demonstrated a 50 % increase in the ability to predict disease state (control, DMD without fibrosis, DMD with fibrosis). ECV demonstrated only the ability to predict presence of LGE, but could not distinguish between controls and DMD without fibrosis. CONCLUSIONS: LGE-spared regions of boys with DMD have significantly different native T1 and ECV values compared to controls. Native T1 measurements can identify early changes in DMD patients without the presence of LGE and help predict disease severity more effectively than ECV. Native T1 may be a novel outcome measure for early cardiac therapies in DMD and other cardiomyopathies.