Background Centers from Europe and United States have reported an exceedingly high number of children with a severe inflammatory syndrome in the setting of COVID-19, which has been termed multisystem inflammatory syndrome in children (MIS-C). Objectives This study aimed to analyze echocardiographic manifestations in MIS-C. Methods We retrospectively reviewed 28 MIS-C, 20 healthy controls and 20 classic Kawasaki disease (KD) patients. We reviewed echocardiographic parameters in acute phase of MIS-C and KD groups, and during subacute period in MIS-C group (interval: 5.2 ± 3 days). Results Only 1 case in MIS-C (4%) manifested coronary artery dilatation (z score=3.15) in acute phase, showing resolution during early follow up. Left ventricular (LV) systolic and diastolic function measured by deformation parameters, were worse in MIS-C compared to KD. Moreover, MIS-C patients with myocardial injury (+) were more affected than myocardial injury (-) MIS-C with respect to all functional parameters. The strongest parameters to predict myocardial injury in MIS-C were global longitudinal strain (GLS), global circumferential strain (GCS), peak left atrial strain (LAS) and peak longitudinal strain of right ventricular free wall (RVFWLS) (Odds ratio: 1.45 (1.08-1.95), 1.39 (1.04-1.88), 0.84 (0.73-0.96), 1.59 (1.09-2.34) respectively). The preserved LVEF group in MIS-C showed diastolic dysfunction. During subacute period, LVEF returned to normal (median: from 54% to 64%, p<0.001) but diastolic dysfunction persisted. Conclusions Unlike classic KD, coronary arteries may be spared in early MIS-C, however, myocardial injury is common. Even preserved EF patients showed subtle changes in myocardial deformation, suggesting subclinical myocardial injury. During an abbreviated follow-up, there was good recovery of systolic function but persistence of diastolic dysfunction and no coronary aneurysms. Condensed abstract Multisystem inflammatory syndrome in children (MIS-C) is an illness that resembles Kawasaki Disease (KD) or toxic shock, reported in children with a recent history of COVID-19 infection. This study analyzed echocardiographic manifestations of this illness. In our cohort of 28 MIS-C patients, left ventricular systolic and diastolic function were worse than in classic KD. These functional parameters correlated with biomarkers of myocardial injury. However, coronary arteries were typically spared. The strongest predictors of myocardial injury were global longitudinal strain, right ventricular strain, and left atrial strain. During subacute period, there was good recovery of systolic function, but diastolic dysfunction persisted.

Background Prenatal diagnosis allows for improved peri-operative outcomes of fetuses with certain forms of congenital heart disease (CHD). Variability in prenatal diagnosis has been demonstrated in other countries, leading to efforts to improve fetal imaging protocols and access to care, but has not been examined across the United States. Objective To evaluate national variation in prenatal detection across geographic region and defect type in neonates and infants with CHD undergoing heart surgery. Methods Cardiovascular operations performed in patients ≤ 6 months of age within the United States and included in the STS-CHS Surgical Database (2006–2012) were eligible for inclusion. Centers with >15% missing prenatal diagnosis data were excluded from the study. Prenatal diagnosis rates were compared across geographic location of residence and defect type using the Chi-square test. Results Overall, the study included 31,374 patients from 91 STS-CHS participating centers across the United States. Prenatal detection occurred in 34% and increased every year from 26% (2006) to 42% (2012). There was significant geographic variation in rates of prenatal diagnosis across states (range 11.8 – 53.4%, p < 0.0001). Significant variability by defect type was also observed with higher rates for lesions identifiable on 4-chamber view versus those requiring outflow tract visualization (57% versus 32%, p < 0.0001). Conclusions Rates of prenatal CHD detection in the United States remain low for patients undergoing surgical intervention, with significant variability between states and across defect type. Further studies are needed to identify reasons for this variation and the potential impact on patient outcomes.

Background-Transcatheter closure of secundum atrial septal defects (ASD) using the Amplatzer septal occluder is generally safe and effective, but erosion into the pericardial space or aorta has been described. Although the absolute risk of this complication is low, there has been no assessment of relative risk factors. Methods and Results-All erosions reported to St. Jude Medical after ASD closure with an Amplatzer septal occluder (cases) were compared with controls (matched 2:1) who underwent ASD closure but did not develop an erosion. A total of 125 erosions were reported between 2002 and 2014, including 95 with an available echocardiogram. The median duration from implant to erosion was 14 days, but was >1 year in 16 patients. Nine patients (all age ≥17 years) who died were more likely to have an oversized device, and to have erosion into the aorta, than survivors. Aortic or superior vena cava rim deficiencies were more common in cases than in controls. In addition, larger balloon-sized ASD diameter, Amplatzer septal occluder device size, and device size-ASD diameter difference, and smaller weight:device size ratio were associated with erosion. On multivariable analysis, deficiency of any rim, device >5 mm larger than ASD diameter, and weight:device size ratio were associated with erosion. Conclusions-In addition to aortic rim deficiency, which was almost universal among erosion cases, there were several relative risk factors for erosion after ASD closure with the Amplatzer septal occluder device. To understand the mechanisms of and absolute risk factors for this uncommon but serious complication, an adequately powered prospective study with thorough echocardiographic evaluation will be critical.

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.