Utilizing Artificial Intelligence-Based Deformable Registration for Global and Layer-Specific Cardiac MRI Strain Analysis in Healthy Children and Young Adults.

RATIONALE AND OBJECTIVES: The absence of published reference values for multilayer-specific strain measurement using cardiac magnetic resonance (CMR) in young healthy individuals limits its use. This study aimed to establish normal global and layer-specific strain values in healthy children and young adults using a deformable registration algorithm (DRA). MATERIALS AND METHODS: A retrospective study included 131 healthy children and young adults (62 males and 69 females) with a mean age of 16.6 ± 3.9 years. CMR examinations were conducted using 1.5T scanners, and strain analysis was performed using TrufiStrain research prototype software (Siemens Healthineers, Erlangen, Germany). Global and layer-specific strain parameters were extracted from balanced Steady-state free precession cine images. Statistical analyses were conducted to evaluate the impact of demographic variables on strain measurements. RESULTS: The peak global longitudinal strain (LS) was -16.0 ± 3.0%, peak global radial strain (RS) was 29.9 ± 6.3%, and peak global circumferential strain (CS) was -17.0 ± 1.8%. Global LS differed significantly between males and females. Transmural strain analysis showed a consistent pattern of decreasing LS and CS from endocardium to epicardium, while radial strain increased. Basal-to-apical strain distribution exhibited decreasing LS and increasing CS in both global and layer-specific analysis. CONCLUSION: This study uses DRA to provide reference values for global and layer-specific strain in healthy children and young adults. The study highlights the impact of sex and age on LS and body mass index on RS. These insights are vital for future cardiac assessments in children, particularly for early detection of heart diseases.

"From Vision to Reality: Virtual Reality's Impact on Baffle Planning in Congenital Heart Disease".

This study aims to evaluate the feasibility and utility of virtual reality (VR) for baffle planning in congenital heart disease (CHD), specifically by creating patient-specific 3D heart models and assessing a user-friendly VR interface. Patient-specific 3D heart models were created using high-resolution imaging data and a VR interface was developed for baffle planning. The process of model creation and the VR interface were assessed for their feasibility, usability, and clinical relevance. Collaborative and interactive planning within the VR space were also explored. The study findings demonstrate the feasibility and usefulness of VR in baffle planning for CHD. Patient-specific 3D heart models generated from imaging data provided valuable insights into complex spatial relationships. The developed VR interface allowed clinicians to interact with the models, simulate different baffle configurations, and assess their impact on blood flow. The VR space's collaborative and interactive planning enhanced the baffle planning process. This study highlights the potential of VR as a valuable tool in baffle planning for CHD. The findings demonstrate the feasibility of using patient-specific 3D heart models and a user-friendly VR interface to enhance surgical planning and patient outcomes. Further research and development in this field are warranted to harness the full benefits of VR technology in CHD surgical management.

Case report: A novel combination of anomalies in a patient with 22q11.2 deletion syndrome.

A frequently occurring genetic disorder, 22q11.2 deletion syndrome can manifest with various abnormalities. The range of cardiac anomalies associated with this syndrome is extensive, with conotruncal defects being the most prevalent. In this study, we report the case of a patient with a unique combination of anatomical abnormalities such as crisscross pulmonary arteries, a cervical aortic arch with coarctation of the aorta, and a ventricular septal defect. The patient underwent initial surgical intervention, which resulted in significant clinical improvement.

Common Arterial Trunk Coexisting With Double-Barreled Aorta.

This case describes the first example of a double-barreled aorta in the setting of a common arterial trunk. Our use of annotated and segmented 3-dimensional models greatly enhanced our ability to elucidate the complex anatomy. (Level of Difficulty: Advanced.).

Pouch Transfer for Single Coronary Artery With Nodal Artery Variant in Arterial Switch Operation.

The arterial switch operation with single coronary artery variance is an independent risk factor for increased operative mortality. There are reports of technical modifications, such as the double-barreled sinus pouch configuration, to improve geometric reimplantation of the single coronary into the neoaortic sinus. We describe the novel application of this technique for transferring a single coronary artery with a separate nodal artery emanating from the opposite sinus during an arterial switch operation.

Palliative Procedures for Congenital Heart Disease: Imaging Findings and Complications.

Palliative procedures are performed for congenital heart diseases that are not amenable for definitive surgical procedures or as a component of hybrid procedures along with transcatheter interventions. Multimodality imaging plays an important role in the follow-up of these palliative procedures, mainly for the timely detection of complications and for planning any subsequent palliative or definitive procedure. Echocardiography is the first-line imaging modality, with CT and MRI used as complementary techniques in indeterminate cases. MRI provides anatomic, functional, flow, and tissue characterization information. CT is performed for the evaluation of vascular anatomy and when MRI cannot be performed due to contraindications, challenges, or artifacts. The modified Blalock-Taussig shunt procedure is the most common systemic-pulmonary artery (PA) shunt procedure, with thrombus being the most serious complication. Other complications of systemic-PA shunts include shunt stenosis, infection, pulmonary overcirculation, and cardiac failure. The Glenn shunt procedure is the second stage of palliation in single ventricle physiology, with thrombus, stenosis, superior vena cava syndrome, and infection being the common complications. The Fontan shunt procedure is the third stage of palliation in single ventricle physiology. Complications can be cardiovascular (heart failure, valve regurgitation, thromboembolism, shunt stenosis, arteriovenous malformation), venolymphatic (collaterals, protein-losing enteropathy, plastic bronchitis), or hepatic (congestion, cirrhosis, portal hypertension). PA banding is used to decrease pulmonary flow or to train the systemic ventricle. Complications include stenosis, thrombus, erosion, pseudoaneurysm, and subaortic obstruction. Atrial septostomy and atrial switch procedures are performed for increasing intracardiac mixing. Complications of atrial septostomy can be mechanical, traumatic, embolic, or electrical. Complications of the atrial switch procedure include baffle stenosis, baffle leak, and systemic ventricle failure. The authors review the role of multimodality imaging in the evaluation of these palliative procedures. © RSNA, 2023 See the invited commentary by Bardo and Popescu in this issue. Quiz questions for this article are available through the Online Learning Center.

Two-staged surgical repair of Berry syndrome type 2B.

Berry syndrome is a rare congenital heart disease that requires complete corrective surgery. In certain extreme cases, such as ours, a two-stage as opposed to single-stage repair is a possibility. In doing so, we also used annotated and segmented three-dimensional models for the first time in Berry syndrome, adding to growing evidence that such models enhance the understanding of complex anatomy for surgical planning.

Subclinical cardiac involvement in student athletes after COVID-19 infection - Evaluation using feature tracking cardiac MRI strain analysis.

OBJECTIVES: To evaluate subclinical cardiac dysfunction in student athletes after COVID-19 infection using feature tracking cardiac MRI strain analysis. METHODS: Student athletes with history of COVID-19 infection underwent cardiac MRI as part of screening before return to competitive play. Subjects were enrolled if they had no or mild symptoms, normal cardiac MRI findings with no imaging evidence of myocarditis. Feature tracking strain analysis was performed using short and long axis cine MRI images of athletes and a separate cohort of healthy controls. Differences between the cardiac strain parameters were statistically analyzed by Mann-Whitney U test. RESULTS: The study cohort included 122 athletes (49 females, mean age 20 years ± 1.5 standard deviations) who had a history of COVID-19, and 35 healthy controls (24 females, mean age 34 years ± 18 standard deviations). COVID-19 positive athletes had normal physiologic cardiac adaptations, including significantly higher left and right ventricle end-diastolic volumes (p = 0.00001) when compared to healthy controls. There was no significant difference between biventricular ejection fraction between athletes and control subjects (p > 0.05). Cardiac MRI parameters, including left ventricle global longitudinal strain (LV-GLS), global circumferential strain (LV-GCS), and global radial strain (LV-GRS) values were normal but slightly lower in athletes compared to controls. LV-GCS and LV-GRS were significantly lower in athletes compared to controls (p = 0.007 and p = 0.005 respectively), but there was no significant difference for LV-GLS (p = 0.088). CONCLUSION: In this study of 122 athletes, there was no evidence of subclinical myocardial alterations following recovery from COVID-19 found on cardiac MRI strain analysis. When compared to healthy controls, the competitive athletes had higher end-diastolic volume indices and reduced, albeit normal, strain values of LV-GLS, LV-GCS, and LV-GRS.

Case Report: Challenging Perioperative Decision-Making in a Neonate With Transposition of Great Arteries and Novel Coronary Anatomy.

Transposition of great arteries (d-TGA) is often associated with various coronary artery (CA) patterns. These anomalous patterns can cause variable clinical symptoms of coronary ischemia including sudden death. CA pattern is one of the major determinants of outcome in TGA postoperatively. An advanced cardiac imaging and a multidisciplinary care approach are essential for a favorable outcome. Here, we describe a novel CA origin pattern in a neonate with d-TGA, who developed myocardial ischemia and required a coronary unroofing procedure for a full recovery.

The Diagnostic Role of Echocardiographic Strain Analysis in Patients Presenting with Chest Pain and Elevated Troponin: A Multicenter Study.

BACKGROUND: Myocarditis presenting as acute chest pain with elevated troponins without significant cardiac compromise is rare in previously healthy children, often referred to as myopericarditis. Diagnosis is challenging, as conventional echocardiographic measures of systolic function can be normal. The aim of this study was to demonstrate the diagnostic utility of strain imaging in this scenario. METHODS: This was a multicenter, retrospective study including patients presenting with chest pain and elevated troponin from 10 institutions who underwent cardiac magnetic resonance imaging and transthoracic echocardiography within 30 days of each other (group 1). Findings were compared with those among 19 control subjects (group 2). Clinical data and conventional echocardiographic and cardiac magnetic resonance imaging data were collected. Echocardiography-derived strain was measured at the core laboratory. Group 1 was divided into subgroups as myocarditis positive (group 1a) or negative (group 1b) on cardiac magnetic resonance imaging on the basis of established criteria. RESULTS: Group 1 included 108 subjects (88 in group 1a, 20 in group 1b). Although all groups had normal mean fractional shortening and mean left ventricular ejection fraction, group 1 had significantly lower ejection fraction (56.8 ± 7.0%) compared with group 2 (62.3 ± 4.9%; P < .005) and fractional shortening (31.2 ± 4.9%) compared with group 2 (34.1 ± 3.5%; P < .05). Additionally, peak global longitudinal strain (GLS) was markedly abnormal in group 1 (-13.9 ± 3.4%) compared with group 2 (-19.8 ± 2.1%; P < .001). In subgroup analysis, GLS was markedly abnormal in group 1a (-13.2 ± 3.0%) compared with group 1b (-17.3 ± 2.6%; P < .001). Fifty-four subjects underwent follow-up echocardiography (46 in group 1a, eight in group 1b), with mean a follow-up time of 10 ± 11 months. At follow-up, whereas ejection fraction and fractional shortening returned to normal in all patients, abnormalities in strain persisted in group 1, with 22% still having abnormal GLS. Moreover, mean GLS was more abnormal in group 1a (-16.1 ± 2.6%) compared with group 1b (-17.4 ± 1.2%; P < .05). CONCLUSIONS: The present study demonstrates that echocardiographic GLS is significantly worse in subjects with myopericarditis presenting with chest pain and elevated troponins compared with control subjects even when conventional measures of systolic function are largely normal and that these abnormalities persisted over time.

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.

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Imaging of the Rare Association of Truncus Arteriosus in a Neonate with the Ductal Origin of the Left Subclavian Artery Using Multidetector CT Angiography and 3D Rendering.

We present the unreported case of a rare association of truncus arteriosus with the ductal origin of the left subclavian artery. Understanding and preoperative identification of these aortic variations are essential to guide optimal surgical management. In this study, the role of advanced visualization 3D modeling techniques in imaging these complex anomalies is discussed.

Infantile Scimitar Syndrome With Contralateral Pulmonary Vein Stenosis and Refractory Pulmonary Hypertension.

Infantile scimitar syndrome is associated with pulmonary hypertension which can be difficult to manage. We present a three-month-old infant with scimitar syndrome, who eventually developed refractory pulmonary hypertension, posing a significant management challenge. Further workup demonstrated contralateral pulmonary vein stenosis, which is rarely described in scimitar syndrome. Our index case highlights the importance of follow-up cardiac catheterizations in these patients with severe pulmonary hypertension.

Double-Outlet Right Ventricle, Pulmonary Atresia, and Discontinuous Branch Pulmonary Arteries Supplied by Bilateral Ducti.

We present a rare case of double-outlet right ventricle with pulmonary atresia and discontinuous branch pulmonary arteries supplied by bilateral ducti from a right aortic arch. To our knowledge, this is only the second documented case of double-outlet right ventricle with bilateral ducti. (Level of Difficulty: Advanced.).

Anomalous Left Anterior Descending Coronary Artery With Retroaortic Left Circumflex Artery: A Novel Coronary Pattern.

A novel coronary anatomy in the form of anomalous left anterior descending coronary artery from pulmonary artery with a retroaortic left circumflex arising from the right coronary artery is presented. This unreported anatomy was discovered in a 7-month-old girl with failure to thrive. (Level of Difficulty: Intermediate.).

Assessment of superior vena cava flow and cardiac output in different patterns of patent ductus arteriosus shunt.

BACKGROUND: Despite the widespread use of superior vena cava (SVC) flow as a marker of systemic blood flow from the upper body, no previous studies have systematically evaluated the correlation between SVC flow and other echocardiography measures of systemic blood flow in the context of different patterns of patent ductus arteriosus (PDA) shunt direction METHODS: A retrospective cohort study of preterm infants (< 30 weeks, < 21 days of life) who underwent comprehensive targeted neonatal echocardiography (TnECHO) was performed. Patients were categorized as follows: (i) Hemodynamically significant left-to-right shunt; (ii): Bidirectional shunt; (iii) No PDA or insignificant shunt. SVC flow, as measured by two distinct methods, was compared to left and right ventricular outputs (LVO and RVO). Intra- and inter-observer reliability testing was performed RESULTS: In total, 45 patients were included (15 in each group) with a median [IQR] weight of 720 [539, 917] grams at the time of assessment. SVC dimensions and flow measurements were not different between the groups, although patients with left-to-right shunt had higher LVO/RVO ratio. SVC flow, as estimated using the modified method, had a strong correlation with LVO (r = .63, p = 0.012) and RVO (r = .635, p = 0.011) in patients with no PDA. Inter- and intra-observer reliability were both stronger for LVO and RVO when compared to SVC flow measurements CONCLUSION: SVC flow was comparable across all three groups irrespective of higher LVO and LVO/RVO ratio in patients with left-to-right shunts. This may reflect poor measurement reliability or compensation for left-to-right ductal shunt by increased LVO to maintain systemic perfusion.

Patient-Specific Patch for an Intra-Atrial Rerouting Procedure Developed Through Surgical Simulation.

BACKGROUND: In pediatric cardiac surgery, an application of three-dimensional (3D) modeling to develop custom-made prostheses is limited, and currently surgeons use their intraoperative visual estimation to develop 3D complex structures from 2D patch materials. Contemporary 3D designers are developing complex surfaces using surface modeling in other industries, which can be applied to pediatric cardiac surgery. However, its free-form nature may lead to intradesigner variability. METHODS: A patient with a body weight of 4 kg with partial anomalous pulmonary venous connection and preoperative computed tomography data was selected, and a patient-specific 3D heart model was obtained. Through collaboration with a pediatric cardiologist and a pediatric cardiac surgeon, a 3D designer developed two patient-specific 3D patches for an intra-atrial rerouting procedure (IAR) for the patient using different methods of surface modeling. The shape and size of two flattened patches were analyzed using a geometric morphometrics (GM) approach. Computational fluid dynamics (CFD) analysis was also performed to calculate pressure drop across streamlines and flow energy loss in the right atrium for both patches. RESULTS: The GM analysis showed that the size and shape of the two patches around the systemic vein orifice, crucial to prevent systemic venous obstruction, were almost equivalent. However, the CFD analysis showed that the pressure drop and flow energy loss were almost twice for one patch compared with the other. CONCLUSIONS: Our platform of developing a patient-specific 3D patch for an IAR procedure using surface modeling seemed promising, although intradesigner patch variability was not neglectable in our small-sized patient.

Multimodality Imaging of Transposition of the Great Arteries.

Transposition of the great arteries (TGA) is a congenital conotruncal abnormality characterized by discordant connections between the ventricles and great arteries, with the aorta originating from the right ventricle (RV), and the pulmonary artery (PA) originating from the left ventricle (LV). The two main types of TGA are complete transposition or dextro-transposition of the great arteries (D-TGA), commonly referred to as d-loop, and congenitally corrected transposition (CCTGA), commonly referred to as l-loop or L-TGA. In D-TGA, the connections between the ventricles and atria are concordant, whereas in CCTGA they are discordant, with the left atrium connected to the RV, and the right atrium connected to the LV. D-TGA manifests during the neonatal period and can be surgically managed by atrial switch operation (AtrSO), arterial switch operation (ASO), Rastelli procedure, or Nikaidoh procedure. Arrhythmia, systemic ventricular dysfunction, baffle stenosis, and baffle leak are the common complications of AtrSO, whereas supravalvular pulmonary or branch PA stenosis, neoaortic dilatation, and coronary artery narrowing are the common complications of ASO. CCTGA may manifest late in life, even in adulthood. Surgeries for associated lesions such as tricuspid regurgitation, subpulmonic stenosis, and ventricular septal defect may be performed. A double-switch operation that includes both the atrial and arterial switch operations constitutes anatomic correction for CCTGA. Imaging plays an important role in the evaluation of TGA, both before and after surgery, for helping define the anatomy, quantify hemodynamics, and evaluate complications. Transthoracic echocardiography is the first-line imaging modality for presurgical planning in children with TGA. MRI provides comprehensive morphologic and functional information, particularly in adults after surgery. CT is performed when MRI is contraindicated or expected to generate artifacts. The authors review the imaging appearances of TGA, with a focus on pre- and postsurgical imaging. Online supplemental material is available for this article. ©RSNA, 2021.