Association of Prenatally Diagnosed Isolated Single Left Superior Vena Cava and Postnatal Development of Coarctation of the Aorta.

To report the prevalence of coarctation of the aorta (CoA) in fetuses with single left superior vena cava (SL-SVC) and to evaluate changes in echocardiographic measurements. Additionally, to report the prevalence of associated malformations. Retrospective observational study of fetuses diagnosed with SL-SVC between 2012 and 2021 at a tertiary fetal cardiology unit. In fetuses without intracardiac abnormalities, Z-scores of the ventricles, great arteries, and Doppler flow patterns are reported. We identified 47 fetuses with SL-SVC of which 8/47 (17%) had abnormal intracardiac anatomy. One fetus was lost to follow-up. Of those with normal intracardiac anatomy and postnatal follow-up (38), karyotype abnormalities were confirmed in 2/38 (5%) and ECA in 8/38 (21%). 33/38 were live-born. None developed CoA postnatally. Paired analysis of Z-scores between early and late scans of 24 fetuses showed that diameters of the right heart structures and Doppler flows of tricuspid valve increased significantly during pregnancy, while the left heart structures and flow patterns did not change. The median risk of CoA did not change between the early and the late scan. We did not observe CoA in this cohort. A degree of ventricular asymmetry was present, but this was due to right heart dominance rather than hypoplasia of left heart structures. This likely reflects redistribution of blood and does not appear to confer increased risk of CoA. Predictive models of the postnatal development of CoA which set the dimensions of right and left heart structures in relation might not be applicable in this situation.

Signal Thresholding Segmentation of Ventricular Volumes in Young Patients with Various Diseases-Can We Trust the Numbers?

In many cardiac diseases, right and left ventricular volumes in systole and diastole are diagnostically and prognostically relevant. Measurements are made by segmentation of the myocardial borders on cardiac magnetic resonance (CMR) images. Automatic detection of myocardial contours is possible by signal thresholding techniques, but must be validated before use in clinical settings. Biventricular volumes were measured in end-diastole (EDVi) and in end-systole (ESVi) both manually and with the MassK application, with signal thresholds at 30%, 50%, and 70%. Stroke volumes (SV) and cardiac indices (CI) were calculated from volumetric measurements and from flow measured in the ascending aorta and the main pulmonary artery, and both methods were compared. Reproducibility of volumetric measurements was tested in 20 patients. Measurements were acquired in 94 patients aged 15 ± 9 years referred for various conditions. EDVi and ESVi of both ventricles were largest with manual segmentation and inversely proportional to the MassK threshold. Manual and k30 SV and CI corresponded best to flow measurements. Interobserver variability was low for all volumes manually and with MassK. In conclusion, manual and 30% threshold-based biventricular volume segmentation agree best with two-dimensional, phantom-corrected phase contrast flow measurements in a young cardiac referral population and are well reproducible.

Myocardial involvement in children with post-COVID multisystem inflammatory syndrome: a cardiovascular magnetic resonance based multicenter international study-the CARDOVID registry.

BACKGROUND: Recent evidence shows an association between coronavirus disease 2019 (COVID-19) infection and a severe inflammatory syndrome in children. Cardiovascular magnetic resonance (CMR) data about myocardial injury in children are limited to small cohorts. The aim of this multicenter, international registry is to describe clinical and cardiac characteristics of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 using CMR so as to better understand the real extent of myocardial damage in this vulnerable cohort. METHODS AND RESULTS: Hundred-eleven patients meeting the World Health Organization criteria for MIS-C associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), having clinical cardiac involvement and having received CMR imaging scan were included from 17 centers. Median age at disease onset was 10.0 years (IQR 7.0-13.8). The majority of children had COVID-19 serology positive (98%) with 27% of children still having both, positive serology and polymerase chain reaction (PCR). CMR was performed at a median of 28 days (19-47) after onset of symptoms. Twenty out of 111 (18%) patients had CMR criteria for acute myocarditis (as defined by the Lake Louise Criteria) with 18/20 showing subepicardial late gadolinium enhancement (LGE). CMR myocarditis was significantly associated with New York Heart Association class IV (p = 0.005, OR 6.56 (95%-CI 1.87-23.00)) and the need for mechanical support (p = 0.039, OR 4.98 (95%-CI 1.18-21.02)). At discharge, 11/111 (10%) patients still had left ventricular systolic dysfunction. CONCLUSION: No CMR evidence of myocardial damage was found in most of our MIS-C cohort. Nevertheless, acute myocarditis is a possible manifestation of MIS-C associated with SARS-CoV-2 with CMR evidence of myocardial necrosis in 18% of our cohort. CMR may be an important diagnostic tool to identify a subset of patients at risk for cardiac sequelae and more prone to myocardial damage. CLINICAL TRIAL REGISTRATION: The study has been registered on ClinicalTrials.gov, Identifier NCT04455347, registered on 01/07/2020, retrospectively registered.

Normal myocardial native T1 values in children using single-point saturation recovery and modified look-locker inversion recovery (MOLLI).

BACKGROUND: T1 mapping is useful to quantify diffuse myocardial processes such as fibrosis, edema, storage disorders, or hemochromatosis. Normal pediatric myocardial T1 values are scarce using modified Look-Locker inversion recovery (MOLLI) sequences and unavailable using Smart1Map, a single-point saturation recovery sequence that measures true T1 . PURPOSE/HYPOTHESIS: To establish normal pediatric myocardial T1 values by Smart1Map and to compare them with T1 by MOLLI. STUDY TYPE: Prospective cohort study. SUBJECTS: Thirty-four children and adolescents aged 8-18 years (14 males) without cardiovascular or inflammatory diseases. FIELD STRENGTH/SEQUENCES: 1.5T, MOLLI, Smart1Map. ASSESSMENT: Mean T1 values of the left ventricular myocardium, the interventricular septum, and the blood pool were measured with MOLLI and Smart1Map in basal, mid-ventricular, and apical short axis slices. STATISTICAL TESTS: T1 values were compared between locations and methods by paired samples t-tests, Wilcoxon signed ranks test, repeated-measures analysis of variance (ANOVA), or Friedman's test. Pearson's correlation coefficient was calculated. For interobserver variability, intraclass correlation coefficients and coefficients of variation were calculated, and Bland-Altman analyses were performed. RESULTS: T1 values were longer by Smart1Map than by MOLLI in all measured locations (myocardium: 1191-1221 vs. 990-1042 msec; all P < 0.001). T1 in basal vs. mid-ventricular slices differed both by MOLLI and by Smart1Map for myocardium and for blood (all P < 0.001). Myocardial T1 did not correlate with age, heart rate, right or left ventricular ejection fraction (all P > 0.05) by either method. Septal vs. total myocardial T1 values in each slice did not differ by MOLLI (basal P = 0.371; mid-ventricular P = 0.08; apical P = 0.378) nor by Smart1Map (basal P = 0.056; mid-ventricular P = 0.918; apical P = 0. 392), after artifacts had been carefully excluded. DATA CONCLUSION: We established pediatric normal native T1 values using the Smart1Map sequence and compared the results with T1 mapping with MOLLI. Septal T1 values did not differ from total myocardial T1 values in each of the myocardial slices. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2020;51:897-903.