Surgical Modulation of Pulmonary Artery Shear Stress: A Patient-Specific CFD Analysis of the Norwood Procedure.

PURPOSR: This study created 3D CFD models of the Norwood procedure for hypoplastic left heart syndrome (HLHS) using standard angiography and echocardiogram data to investigate the impact of shunt characteristics on pulmonary artery (PA) hemodynamics. Leveraging routine clinical data offers advantages such as availability and cost-effectiveness without subjecting patients to additional invasive procedures. METHODS: Patient-specific geometries of the intrathoracic arteries of two Norwood patients were generated from biplane cineangiograms. "Virtual surgery" was then performed to simulate the hemodynamics of alternative PA shunt configurations, including shunt type (modified Blalock-Thomas-Taussig shunt (mBTTS) vs. right ventricle-to-pulmonary artery shunt (RVPAS)), shunt diameter, and pulmonary artery anastomosis angle. Left-right pulmonary flow differential, Qp/Qs, time-averaged wall shear stress (TAWSS), and oscillatory shear index (OSI) were evaluated. RESULTS: There was strong agreement between clinically measured data and CFD model output throughout the patient-specific models. Geometries with a RVPAS tended toward more balanced left-right pulmonary flow, lower Qp/Qs, and greater TAWSS and OSI than models with a mBTTS. For both shunt types, larger shunts resulted in a higher Qp/Qs and higher TAWSS, with minimal effect on OSI. Low TAWSS areas correlated with regions of low flow and changing the PA-shunt anastomosis angle to face toward low TAWSS regions increased TAWSS. CONCLUSION: Excellent correlation between clinically measured and CFD model data shows that 3D CFD models of HLHS Norwood can be developed using standard angiography and echocardiographic data. The CFD analysis also revealed consistent changes in PA TAWSS, flow differential, and OSI as a function of shunt characteristics.

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.

A Deep Learning Pipeline for Assessing Ventricular Volumes from a Cardiac MRI Registry of Patients with Single Ventricle Physiology.

Purpose To develop an end-to-end deep learning (DL) pipeline for automated ventricular segmentation of cardiac MRI data from a multicenter registry of patients with Fontan circulation (Fontan Outcomes Registry Using CMR Examinations [FORCE]). Materials and Methods This retrospective study used 250 cardiac MRI examinations (November 2007-December 2022) from 13 institutions for training, validation, and testing. The pipeline contained three DL models: a classifier to identify short-axis cine stacks and two U-Net 3+ models for image cropping and segmentation. The automated segmentations were evaluated on the test set (n = 50) by using the Dice score. Volumetric and functional metrics derived from DL and ground truth manual segmentations were compared using Bland-Altman and intraclass correlation analysis. The pipeline was further qualitatively evaluated on 475 unseen examinations. Results There were acceptable limits of agreement (LOA) and minimal biases between the ground truth and DL end-diastolic volume (EDV) (bias: -0.6 mL/m2, LOA: -20.6 to 19.5 mL/m2) and end-systolic volume (ESV) (bias: -1.1 mL/m2, LOA: -18.1 to 15.9 mL/m2), with high intraclass correlation coefficients (ICCs > 0.97) and Dice scores (EDV, 0.91 and ESV, 0.86). There was moderate agreement for ventricular mass (bias: -1.9 g/m2, LOA: -17.3 to 13.5 g/m2) and an ICC of 0.94. There was also acceptable agreement for stroke volume (bias: 0.6 mL/m2, LOA: -17.2 to 18.3 mL/m2) and ejection fraction (bias: 0.6%, LOA: -12.2% to 13.4%), with high ICCs (>0.81). The pipeline achieved satisfactory segmentation in 68% of the 475 unseen examinations, while 26% needed minor adjustments, 5% needed major adjustments, and in 0.4%, the cropping model failed. Conclusion The DL pipeline can provide fast standardized segmentation for patients with single ventricle physiology across multiple centers. This pipeline can be applied to all cardiac MRI examinations in the FORCE registry. Keywords: Cardiac, Adults and Pediatrics, MR Imaging, Congenital, Volume Analysis, Segmentation, Quantification Supplemental material is available for this article. © RSNA, 2023.

Diagnostic Approach to Recurrent Intracardiac Masses That Present With Systemic Symptoms.

Cardiac masses are difficult to diagnose in the pediatric population, especially in the setting of systemic symptoms. Although multiple imaging modalities are available to characterize cardiac masses, it is important to consider a different diagnostic approach in the setting of recurrent cardiac masses and nonspecific systemic symptoms. We present a case involving a previously healthy adolescent with multiple hospitalizations because of persistent fevers, cachexia, and recurrent cardiac masses. Echocardiography and cardiac computed tomography imaging suggested endocarditis, but the patient failed to respond to multiple intravenous antibiotic treatments. He developed recurrent cardiac masses in the right atrium and right ventricle that were debulked and biopsied. The biopsy did not yield a conclusive diagnosis. The patient returned to the hospital with hemoptysis and large pulmonary pseudoaneurysms that had to be occluded during cardiac catheterization. Given his constellation of symptoms and improvement with steroids during surgical procedures, he was ultimately diagnosed with a variant of Behcet's disease known as Hughes-Stovin syndrome. His symptoms resolved completely with steroids and immunosuppression therapy. Our report reveals the limitations of the standard diagnostic approach toward cardiac masses and the importance of considering response to treatment as a clue to the etiology of an unusual cardiac mass.

Statistical modeling of extracellular vesicle cargo to predict clinical trial outcomes for hypoplastic left heart syndrome.

Cardiac-derived c-kit+ progenitor cells (CPCs) are under investigation in the CHILD phase I clinical trial (NCT03406884) for the treatment of hypoplastic left heart syndrome (HLHS). The therapeutic efficacy of CPCs can be attributed to the release of extracellular vesicles (EVs). To understand sources of cell therapy variability we took a machine learning approach: combining bulk CPC-derived EV (CPC-EV) RNA sequencing and cardiac-relevant in vitro experiments to build a predictive model. We isolated CPCs from cardiac biopsies of patients with congenital heart disease (n = 29) and the lead-in patients with HLHS in the CHILD trial (n = 5). We sequenced CPC-EVs, and measured EV inflammatory, fibrotic, angiogeneic, and migratory responses. Overall, CPC-EV RNAs involved in pro-reparative outcomes had a significant fit to cardiac development and signaling pathways. Using a model trained on previously collected CPC-EVs, we predicted in vitro outcomes for the CHILD clinical samples. Finally, CPC-EV angiogenic performance correlated to clinical improvements in right ventricle performance.

Cardiac Magnetic Resonance Imaging to Determine Single Ventricle Function in a Pediatric Population is Feasible in a Large Trial Setting: Experience from the Single Ventricle Reconstruction Trial Longitudinal Follow up.

The Single Ventricle Reconstruction (SVR) Trial was a randomized prospective trial designed to determine survival advantage of the modified Blalock-Taussig-Thomas shunt (BTTS) vs the right ventricle to pulmonary artery conduit (RVPAS) for patients with hypoplastic left heart syndrome. The primary aim of the long-term follow-up (SVRIII) was to determine the impact of shunt type on RV function. In this work, we describe the use of CMR in a large cohort follow up from the SVR Trial as a focused study of single ventricle function. The SVRIII protocol included short axis steady-state free precession imaging to assess single ventricle systolic function and flow quantification. There were 313 eligible SVRIII participants and 237 enrolled, ages ranging from 10 to 12.5 years. 177/237 (75%) participants underwent CMR. The most common reasons for not undergoing CMR exam were requirement for anesthesia (n = 14) or ICD/pacemaker (n = 11). A total of 168/177 (94%) CMR studies were diagnostic for RVEF. Median exam time was 54 [IQR 40-74] minutes, cine function exam time 20 [IQR 14-27] minutes, and flow quantification time 18 [IQR 12-25] minutes. There were 69/177 (39%) studies noted to have intra-thoracic artifacts, most common being susceptibility artifact from intra-thoracic metal. Not all artifacts resulted in non-diagnostic exams. These data describe the use and limitations of CMR for the assessment of cardiac function in a prospective trial setting in a grade-school-aged pediatric population with congenital heart disease. Many of the limitations are expected to decrease with the continued advancement of CMR technology.

Cardiac Magnetic Resonance Findings of Coronavirus Disease 2019 (COVID-19) Vaccine-Associated Myopericarditis at Intermediate Follow-Up: A Comparison with Classic Myocarditis.

OBJECTIVE: To report intermediate cardiac magnetic resonance (CMR) findings of coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis (C-VAM) and compare with classic myocarditis. STUDY DESIGN: Retrospective cohort study including children diagnosed with C-VAM from May 2021 through December 2021 with early and intermediate CMR. Patients with classic myocarditis from January 2015 through December 2021 and intermediate CMR were included for comparison. RESULTS: There were 8 patients with C-VAM and 20 with classic myocarditis. Among those with C-VAM, CMR performed at a median 3 days (IQR 3, 7) revealed 2 of 8 patients with left ventricular ejection fraction <55%, 7 of 7 patients receiving contrast with late gadolinium enhancement (LGE), and 5 of 8 patients with elevated native T1 values. Borderline T2 values suggestive of myocardial edema were present in 6 of 8 patients. Follow-up CMRs performed at a median 107 days (IQR 97, 177) showed normal ventricular systolic function, T1, and T2 values; 3 of 7 patients had LGE. At intermediate follow-up, patients with C-VAM had fewer myocardial segments with LGE than patients with classic myocarditis (4/119 vs 42/340, P = .004). Patients with C-VAM also had a lower frequency of LGE (42.9 vs 75.0%) and lower percentage of left ventricular ejection fraction <55% compared with classic myocarditis (0.0 vs 30.0%), although these differences were not statistically significant. Five patients with classic myocarditis did not receive an early CMR, leading to some selection bias in study design. CONCLUSIONS: Patients with C-VAM had no evidence of active inflammation or ventricular dysfunction on intermediate CMR, although a minority had persistent LGE. Intermediate findings in C-VAM revealed less LGE burden compared with classic myocarditis.

Transcatheter Hepatic Conduit-Azygous Vein Connection Reduces Pulmonary Arteriovenous Malformations in a Cyanotic Fontan Patient.

The authors report a closed-chest, transcatheter large-vessel connection (hepatic conduit to azygous vein) to reverse pulmonary arteriovenous malformations in a 10-year-old patient after Fontan for heterotaxy/interrupted inferior vena cava, with an increase in oxygen saturation from 78% to 96%. Computational fluid dynamics estimated a 14-fold increase in hepatic blood flow to the left pulmonary artery (from 1.3% to 14%). (Level of Difficulty: Advanced.).

Recommendations for Cardiac Point-of-Care Ultrasound in Children: A Report from the American Society of Echocardiography.

Cardiac point-of-care ultrasound has the potential to improve patient care, but its application to children requires consideration of anatomic and physiologic differences from adult populations, and corresponding technical aspects of performance. This document is the product of an American Society of Echocardiography task force composed of representatives from pediatric cardiology, pediatric critical care medicine, pediatric emergency medicine, pediatric anesthesiology, and others, assembled to provide expert guidance. This diverse group aimed to identify common considerations across disciplines to guide evolution of indications, and to identify common requirements and infrastructure necessary for optimal performance, training, and quality assurance in the practice of cardiac point-of-care ultrasound in children. The recommendations presented are intended to facilitate collaboration among subspecialties and with pediatric echocardiography laboratories by identifying key considerations regarding (1) indications, (2) imaging recommendations, (3) training and competency assessment, and (4) quality assurance.

Highlights of the Virtual Society for Cardiovascular Magnetic Resonance 2022 Scientific Conference: CMR: improving cardiovascular care around the world.

The 25th Society for Cardiovascular Magnetic Resonance (SCMR) Annual Scientific Sessions saw 1524 registered participants from more than 50 countries attending the meeting virtually. Supporting the theme "CMR: Improving Cardiovascular Care Around the World", the meeting included 179 invited talks, 52 sessions including 3 plenary sessions, 2 keynote talks, and a total of 93 cases and 416 posters. The sessions were designed so as to showcase the multifaceted role of cardiovascular magnetic resonance (CMR) in identifying and prognosticating various myocardial pathologies. Additionally, various social networking sessions as well as fun activities were organized. The major areas of focus for the future are likely to be rapid efficient and high value CMR exams, automated and quantitative acquisition and post-processing using artificial intelligence and machine learning, multi-contrast imaging and advanced vascular imaging including 4D flow.

Autologous Cardiac Stem Cell Injection in Patients with Hypoplastic Left Heart Syndrome (CHILD Study).

Mortality in infants with hypoplastic left heart syndrome (HLHS) is strongly correlated with right ventricle (RV) dysfunction. Cell therapy has demonstrated potential improvements of RV dysfunction in animal models related to HLHS, and neonatal human derived c-kit+ cardiac-derived progenitor cells (CPCs) show superior efficacy when compared to adult human cardiac-derived CPCs (aCPCs). Neonatal CPCs (nCPCs) have yet to be investigated in humans. The CHILD trial (Autologous Cardiac Stem Cell Injection in Patients with Hypoplastic Left Heart Syndrome) is a Phase I/II trial aimed at investigating intramyocardial administration of autologous nCPCs in HLHS infants by assessing the feasibility, safety, and potential efficacy of CPC therapy. Using an open-label, multicenter design, CHILD investigates nCPC safety and feasibility in the first enrollment group (Group A/Phase I). In the second enrollment group, CHILD uses a randomized, double-blinded, multicenter design (Group B/Phase II), to assess nCPC efficacy based on RV functional and structural characteristics. The study plans to enroll 32 patients across 4 institutions: Group A will enroll 10 patients, and Group B will enroll 22 patients. CHILD will provide important insights into the therapeutic potential of nCPCs in patients with HLHS.Clinical Trial Registration https://clinicaltrials.gov/ct2/home NCT03406884, First posted January 23, 2018.

Cardiac Magnetic Resonance Findings after Multisystem Inflammatory Syndrome in Children.

OBJECTIVE: To describe the cardiac magnetic resonance (MR) findings of children recovered from multisystem inflammatory syndrome in children (MIS-C) longer than 3 months after acute illness. STUDY DESIGN: We performed a retrospective cohort study of children hospitalized with MIS-C at a single institution receiving cardiac MR imaging between July 2020 and May 2021. Patient demographics, echocardiogram data from diagnosis through follow-up, and cardiac MR data obtained at approximately 3 months after hospitalization were recorded. RESULTS: In total, 51 children with a median age of 11.3 years were included; 80% of patients had left ventricular ejection fraction <55%, 65% of patients developed valvular regurgitation, and 20% of patients developed coronary artery dilation during acute illness. Cardiac MR was performed at a median time of 105 days after diagnosis; 8% of patients had left ventricular ejection fraction <55%; 1 patient had residual valvular regurgitation; and 2 patients had residual coronary artery dilation. Two of 51 patients were found to have late gadolinium enhancement, T1 mapping abnormalities, and abnormal or borderline extracellular volume calculations suggesting myocardial fibrosis. No patient had T2 mapping abnormalities corresponding with edema, and no patient met the modified Lake Louise criteria for acute myocarditis; 10 of 51 patients had isolated elevated T1 values. CONCLUSIONS: At 3-5 months following diagnosis, cardiac MR reveals no evidence of acute myocarditis as described by the modified Lake Louise criteria in patients with MIS-C. Two patients were observed to have myocardial fibrosis without regional wall motion abnormalities, and 10 had isolated imaging changes (elevated T1 values) in the absence of macroscopic fibrosis.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

BACKGROUND: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria. OBJECTIVES: The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. METHODS: CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. RESULTS: Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. CONCLUSIONS: CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

An Anterior Anastomosis for the Modified Fontan Connection: A Hemodynamic Analysis.

This hemodynamic feasibility study examined total cavopulmonary connection (TCPC) designs connecting the extracardiac conduit to the anterior surface of pulmonary arteries (PAs) or superior vena cava (SVC) rather than to the inferior PA surface (traditional TCPC). The study involved twenty-five consecutive Fontan patients meeting inclusion criteria from a single institution. A virtual surgical platform mimicked the completed traditional TCPC and generated three anterior anastomosis designs: Anterior-PA, Middle-SVC, and SVC-Inn (Inn: innominate vein). Hemodynamic performance of anterior anastomosis designs was compared with the traditional TCPC regarding indexed power loss (iPL) and hepatic flow distribution (HFD). Compared to the traditional TCPC, the Anterior-PA design produces a similar iPL. The Middle-SVC design is also similar, though the iPL difference is positively correlated with the anastomosing height. The SVC-Inn design had significantly more iPL. The three anterior anastomosis designs did not have a significant difference in HFD (from traditional TCPC). Pulmonary flow distribution (PFD) has a stronger correlation with HFD from the anterior anastomosis designs than the traditional TCPC. This hemodynamic feasibility study examined anterior anastomosis, extracardiac TCPC designs that may offer surgeons clinical dexterity. The Anterior-PA design may be equivalent to the traditional TCPC. Fontan extracardiac conduit anastomosis just superior to the PAs (Middle-SVC) also preserves hemodynamic performance and avoids direct PA anastomosis. These designs could simplify surgical Fontan completion, and may particularly benefit patients requiring surgical dissection, having atypical PA orientation, or after PA stent angioplasty.

Factors Influencing Temporal Trends in Pediatric Inpatient Imaging Utilization.

BACKGROUND: Concern exists over exponential growth in cardiac imaging in adults, but there is paucity of such data for cardiac imaging trends in pediatric patients. The aims of this study were to determine temporal trends in the use of noninvasive cardiac imaging and compare these with trends in the use of noncardiac imaging and to identify factors influencing those trends using the Pediatric Health Information Service database. METHODS: Pediatric inpatient encounter data from January 2004 to December 2017 at 35 pediatric hospitals were extracted from the Pediatric Health Information Service database. Temporal imaging utilization trends in cardiac and noncardiac ultrasound or echocardiography, magnetic resonance imaging (MRI), and computed tomography (CT) were assessed using linear mixed-effects models. Models were adjusted for case-mix index, complex chronic conditions, patient age, length of stay, payer source, and cardiac surgical volume. RESULTS: A total of 5,869,335 encounters over 14 years were analyzed (median encounters per center per year, 11,411; median patient age, 4 years; median length of stay, 3 days). From 2004 to 2017, the rates of pediatric inpatient cardiac and noncardiac ultrasound and MRI increased, whereas the rate of noncardiac CT decreased. Cardiac CT use increased beginning in 2014 (+0.264 cardiac CT encounters per 1,000 encounters per year), surpassing the rate of rise of cardiac MRI. Case-mix index, cardiac surgical volume, and payer source affected the largest number of imaging trends. CONCLUSIONS: Among pediatric inpatients, utilization of cardiac and noncardiac ultrasound and MRI has steadily increased. Noncardiac CT use declined and cardiac CT use increased after 2014. Factors influencing imaging trends include case-mix index, cardiac surgical volume, and payer source. This study lays a foundation for investigations of imaging-related resource utilization and outcomes among pediatric inpatients.

Y-graft modification to the Fontan procedure: Increasingly balanced flow over time.

OBJECTIVE: The use of Y-grafts for Fontan completion is hypothesized to offer more balanced hepatic flow distribution (HFD) and decreased energy losses. The purpose of this study was to evaluate the hemodynamic performance of Y-grafts over time using serial cardiac magnetic resonance data and to compare their performance with extracardiac Fontan connections. METHODS: Ten Fontan patients with commercially available Y-graft connections and serial postoperative cardiac magnetic resonance data were included in this study. Patient-specific computational fluid dynamics simulations were used to estimate HFD and energy losses. Y-graft performance was compared with 3 extracardiac conduit Fontan groups (n = 10 for each) whose follow-up times straddle the Y-graft time points. RESULTS: Y-graft HFD became significantly more balanced over time (deviation from 50% decreased from 18% ± 14% to 8% ± 8%; P = .015). Total cavopulmonary connection resistance did not significantly change. Y-grafts at 3-year follow-up showed more balanced HFD than the extracardiac conduit groups at both the earlier and later follow-up times. Total cavopulmonary connection resistance was not significantly different between any Y-graft or extracardiac conduit group. CONCLUSIONS: Y-grafts showed significantly more balanced HFD over a 3-year follow-up without an increase in total cavopulmonary connection resistance, and therefore may be a valuable option for Fontan completion. Additional follow-up data at longer follow-up times are still needed to thoroughly characterize the potential advantages of Y-graft use.

Cardiomegaly on chest radiographs as a predictor of heart disease in the pediatric population.

BACKGROUND: Cardiomegaly on chest radiographs (CXR) in pediatric patients leads to multiple tests. We aimed to determine the positive predictive value (PPV) of cardiomegaly on CXR in predicting subsequent heart disease and to assess the utility of obtaining a B-type Natriuretic Peptide level (BNP) and/or electrocardiogram (EKG) in such patients. We hypothesized that an echocardiogram may not be appropriate in all cases of cardiomegaly on CXR, particularly in a patient with a normal EKG and BNP level. METHODS: We performed a retrospective cohort study of pediatric patients with cardiomegaly on their initial CXR between January 2015-December 2017. Patients without a subsequent echocardiogram or known congenital heart disease were excluded. A patient was deemed to have heart disease if they had structural abnormalities, functional abnormalities or a pericardial effusion on echocardiogram. The PPV of CXR and the PPV/NPV of the other tests (EKG, BNP) were calculated using contingency tables. RESULTS: Four hundred and eighty nine patients met inclusion criteria. The PPV of cardiomegaly on CXR alone without any other diagnostic testing in predicting subsequent heart disease was 15%. The PPV increased if there was either an abnormal EKG or a BNP >100 pg/ml and further increased if both of these were present. The PPV values were higher in patients <1 year of age. CONCLUSIONS: Cardiomegaly on CXR can often predict the presence of heart disease, particularly in infants. Further testing with EKG and BNP can better predict who may have heart disease, but it may not eliminate the need for echocardiography.

Cardiac Magnetic Resonance Imaging Macroscopic Fibro-Fatty Infiltration of the Myocardium in Pediatric Patients with Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia.

Background: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited, progressive form of cardiomyopathy, which is characterized by fibrofatty replacement of the myocardium. While the gold standard for diagnosis remains pathologic evaluation of biopsy, advances in noninvasive imaging, including cardiac magnetic resonance imaging (CMRI), have led to improved clinical diagnosis.Case report: We report three additional cases of pediatric patients that have pathologically confirmed ARVC/D with CMRI images, demonstrating extensive macroscopic fatty infiltration of the right and left ventricular myocardium. The identification using CMRI allowed timely transplantation and patient survival.Conclusion: Our study is designed to highlight how fibrofatty changes are minimal using CMRI in the pediatric population and how this can be a valuable tool to provide an additional method of diagnosis.