Longitudinal Assessment of Cardiac Outcomes of Multisystem Inflammatory Syndrome in Children Associated With COVID-19 Infections.

Background In multisystem inflammatory syndrome in children, there is paucity of longitudinal data on cardiac outcomes. We analyzed cardiac outcomes 3 to 4 months after initial presentation using echocardiography and cardiac magnetic resonance imaging. Methods and Results We included 60 controls and 60 cases of multisystem inflammatory syndrome in children. Conventional echocardiograms and deformation parameters were analyzed at 4 time points: (1) acute phase (n=60), (2) subacute phase (n=50; median, 3 days after initial echocardiography), (3) 1-month follow-up (n=39; median, 22 days), and (4) 3- to 4-month follow-up (n=25; median, 91 days). Fourteen consecutive cardiac magnetic resonance imaging studies were reviewed for myocardial edema or fibrosis during subacute (n=5) and follow-up (n=9) stages. In acute phase, myocardial injury was defined as troponin-I level ≥0.09 ng/mL (>3 times normal) or brain-type natriuretic peptide >800 pg/mL. All deformation parameters, including left ventricular global longitudinal strain, peak left atrial strain, longitudinal early diastolic strain rate, and right ventricular free wall strain, recovered quickly within the first week, followed by continued improvement and complete normalization by 3 months. Median time to normalization of both global longitudinal strain and left atrial strain was 6 days (95% CI, 3-9 days). Myocardial injury at presentation (70% of multisystem inflammatory syndrome in children cases) did not affect short-term outcomes. Four patients (7%) had small coronary aneurysms at presentation, all of which resolved. Only 1 of 9 patients had residual edema but no fibrosis by cardiac magnetic resonance imaging. Conclusions Our short-term study suggests that functional recovery and coronary outcomes are good in multisystem inflammatory syndrome in children. Use of sensitive deformation parameters provides further reassurance that there is no persistent subclinical dysfunction after 3 months.

Analysis of Inlet Velocity Profiles in Numerical Assessment of Fontan Hemodynamics.

Computational fluid dynamic (CFD) simulations are widely utilized to assess Fontan hemodynamics that are related to long-term complications. No previous studies have systemically investigated the effects of using different inlet velocity profiles in Fontan simulations. This study implements real, patient-specific velocity profiles for numerical assessment of Fontan hemodynamics using CFD simulations. Four additional, artificial velocity profiles were used for comparison: (1) flat, (2) parabolic, (3) Womersley, and (4) parabolic with inlet extensions [to develop flow before entering the total cavopulmonary connection (TCPC)]. The differences arising from the five velocity profiles, as well as discrepancies between the real and each of the artificial velocity profiles, were quantified by examining clinically important metrics in TCPC hemodynamics: power loss (PL), viscous dissipation rate (VDR), hepatic flow distribution, and regions of low wall shear stress. Statistically significant differences were observed in PL and VDR between simulations using real and flat velocity profiles, but differences between those using real velocity profiles and the other three artificial profiles did not reach statistical significance. These conclusions suggest that the artificial velocity profiles (2)-(4) are acceptable surrogates for real velocity profiles in Fontan simulations, but parabolic profiles are recommended because of their low computational demands and prevalent applicability.

Qualitative echocardiographic assessment of aortic valve regurgitation with quantitative cardiac magnetic resonance: a comparative study.

This study examined the correlation of echocardiography (ECHO) and Cardiac Magnetic Resonance (CMR) in the assessment of aortic valve regurgitation (AR) in children and young adults with congenital heart disease. We hypothesized that qualitative ECHO assessment correlates insufficiently with quantitative CMR data and compared subjective ECHO evaluations with objective measurement of regurgitant fractions (RF) by CMR. Patients who had both ECHO and CMR assessments of AR within 60 days of each other were included. The qualitative ECHO assessment (mild, moderate, severe) of AR and left ventricular dimension at end diastole were recorded. RF was quantified by CMR using phase-contrast velocity mapping. Repeat ECHO review and grading of AR was performed by a blinded single reader in a randomly chosen subgroup of patients. In 43 patients studied, statistical significance was observed in the CMR-RF between mild and moderate, and between mild and severe ECHO grades. There was significant overlap of objective RF between subjective grades. Mild ECHO AR corresponded to an RF (%) of 0-29, moderate 1-40, and severe 5-58. Overlap was more significant at moderate and severe grades. Results were similar in the group in whom a single reader interpreted the ECHO assessment. In conclusion, results derived from a real-life multiple-reader ECHO laboratory showed inconsistencies in ECHO grading of AR, with a wide range of objectively measured RF within a given ECHO grade. ECHO is less reliable in identifying more severe AR, often overestimating severity. Quantitative CMR is a potentially useful supplement to ECHO for management decisions and assessments of medical and surgical therapies in children and young adults with AR.

Multi-center transferability of a breath-hold T2 technique for myocardial iron assessment.

BACKGROUND: Cardiac iron overload is the leading cause of death in thalassemia major and is usually assessed using myocardial T2* measurements. Recently a cardiovascular magnetic resonance (CMR) breath-hold T2 sequence has been developed as a possible alternative. This cardiac T2 technique has good interstudy reproducibility, but its transferability to different centres has not yet been investigated. METHODS AND RESULTS: The breath-hold black blood spin echo T2 sequence was installed and validated on 1.5T Siemens MR scanners at 4 different centres across the world. Using this sequence, 5-10 thalassemia patients from each centre were scanned twice locally within a week for local interstudy reproducibility (n = 34) and all were rescanned within one month at the standardization centre in London (intersite reproducibility). The local interstudy reproducibility (coefficient of variance) and mean difference were 4.4% and -0.06 ms. The intersite reproducibility and mean difference between scanners were 5.2% and -0.07 ms. CONCLUSION: The breath-hold myocardial T2 technique is transferable between Siemens scanners with good intersite and local interstudy reproducibility. This technique may have value in the diagnosis and management of patients with iron overload conditions such as thalassemia.

Identification, imaging, functional assessment and management of congenital coronary arterial abnormalities in children.

The coronary arteries, the vessels through which both substrate and oxygen are provided to the cardiac muscle, normally arise from paired stems, right and left, each arising from a separate and distinct sinus of the aortic valve. The right coronary artery runs through the right atrioventricular groove, terminating in the majority of instances in the inferior interventricular groove. The main stem of the left coronary artery bifurcates into the anterior descending, or interventricular, and the circumflex branches. Origin of the anterior descending and circumflex arteries from separate orifices from the left sinus of Valsalva occurs in about 1% of the population, while it is also frequent to find the infundibular artery arising as a separate branch from the right sinus of Valsalva. Anomalies of the coronary arteries can result from rudimentary persistence of an embryologic coronary arterial structure, failure of normal development or normal atrophy as part of development, or misplacement of connection of a an otherwise normal coronary artery. Anomalies, therefore, can be summarized in terms of abnormal origin or course, abnormal number of coronary arteries, lack of patency of the orifice of coronary artery, or abnormal connections of the arteries. Anomalous origin of the left coronary artery from the pulmonary trunk occurs with an incidence of approximately 1 in 300,000 children. The degree of left ventricular dysfunction produced likely relates to the development of collateral vessels that arise from the right coronary artery, and provide flow into the left system. Anomalous origin of either the right or the left coronary artery from the opposite sinus of Valsalva can be relatively innocuous, but if the anomalous artery takes an interarterial course between the pulmonary trunk and the aorta, this can underlie sudden death, almost invariably during or immediately following strenuous exercise or competitive sporting events. Distal anomalies of the coronary arteries most commonly involve abnormal connections, or fistulas, between the right or left coronary arterial systems and a chamber or vessel. We discuss the current techniques available for imaging these various lesions, along with their functional assessment, concluding with a summary of current strategies for management.

Preliminary investigations into a new method of functional assessment of the fetal heart using a novel application of 'real-time' cardiac magnetic resonance imaging.

OBJECTIVES: Because of quantitative echocardiographic limitations of fetal ventricular volumes as well as poor windows, we sought to determine if real-time magnetic resonance imaging (MRI) could be used. METHODS: Real-time, functional, true fast imaging with steady-state precession, cardiac MRI was performed on 2 fetuses (one with hypoplastic left heart syndrome and one with ductal constriction). Fetal echocardiography was performed and cardiac index by Doppler was used to validate volume measures by MRI. RESULTS: This technique was able to visualize the beating heart and assess ventricular volumes. Cardiac index and assessment of right ventricular hypertrophy and dilation by echocardiography were consistent with the ventricular volumes and right ventricular hypertrophy obtained by cardiac MRI. CONCLUSION: Real-time, functional fetal cardiac MRI is possible and can be used to quantitatively assess ventricular volumes and cardiac index in utero.