Ventricular function and tissue characterization by cardiac magnetic resonance imaging following hospitalization for multisystem inflammatory syndrome in children: a prospective study.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) is a severe life-threatening manifestation of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection that often presents with acute cardiac dysfunction and cardiogenic shock. While recovery from acute illness is excellent, the long-term myocardial impact is unknown. OBJECTIVE: To compare cardiac MRI findings in children 6-9 months after their hospitalization with MIS-C against MRI findings in healthy controls to assess for residual myocardial disease. MATERIALS AND METHODS: We prospectively performed cardiac MRI on 13 children 6-9 months following their hospitalization with MIS-C: eight of these children had a history of left ventricle ejection fraction (LVEF) < 50%, persistent symptoms, or electrocardiogram (ECG) abnormalities and underwent clinical MRI; five of these children without cardiac abnormalities during their hospitalization underwent research MRIs. We compared their native T1 and T2 mapping values with those of 20 normal controls. RESULTS: Cardiac MRI was performed at 13.6 years of age (interquartile range [IQR] 11.9-16.4 years) and 8.2 months (IQR 6.8-9.6 months) following hospitalization. Twelve children displayed normal ejection fraction: left ventricle (LV) 57.2%, IQR 56.1-58.4; right ventricle (RV) 53.1%, IQR 52.0-55.7. One had low-normal LVEF (52%). They had normal extracellular volume (ECV) and normal T2 and native T1 times compared to controls. There was no qualitative evidence of edema. One child had late gadolinium enhancement (LGE) with normal ejection fraction, no edema, and normal T1 and T2 times. When stratifying children who had MIS-C according to history of LVEF <55% on echocardiography, there was no difference in MRI values. CONCLUSION: Although many children with MIS-C present acutely with cardiac dysfunction, residual myocardial damage 6-9 months afterward appears minimal. Long-term implications warrant further study.

3D-Printed Cardiac Models for Fetal Counseling: A Pilot Study and Novel Approach to Improve Communication.

A fetal cardiology consultation involves using two-dimensional drawings to explain the cardiac anatomy which can result in inherent variation in how the congenital heart disease (CHD) is conveyed. In this pilot study, we incorporated three-dimensional printed (3DP) models into fetal counseling to demonstrate feasibility and evaluate the impact on parental knowledge, understanding, and anxiety. Parents with a prenatal diagnosis of a muscular ventricular septal defect (VSD) and/or coarctation of aorta were enrolled. Providers were randomized into a Model or Drawing Group and crossed after six months. Parents completed a survey after the consultation which evaluated knowledge of the CHD lesion, expectant surgical management, self-rated understanding, attitude towards the visualization tool, and anxiety. Twenty-nine patients enrolled over a 12 month period. Twelve consultations were done for coarctation of aorta, 13 for VSD, and four for coarctation with a VSD. Both Model and Drawing groups scored similarly in self-reported understanding and confidence, helpfulness of and improvement in communication with the visualization tool. The Model group had higher scores on questions related to the CHD anatomy and surgical intervention [5 [4-5] versus 4 [3.5-5]], p = 0.23 although this didn't reach statistical significance. For the majority (83%) of consultations, the cardiologist agreed that the 3D model improved communication. In this pilot study, we demonstrate the use of 3DP cardiac models during prenatal CHD counseling is feasible and produces results related to parental understanding and knowledge that are equal to and possibly better than the current standard of care.

Evaluation of self-calibrated non-linear phase-contrast correction in pediatric and congenital cardiovascular magnetic resonance imaging.

BACKGROUND: The need for background error correction in phase-contrast flow analysis has historically posed a challenge in cardiac magnetic resonance (MR) imaging. While previous studies have shown that phantom correction improves flow measurements, it impedes scanner workflow. OBJECTIVE: To evaluate the efficacy of self-calibrated non-linear phase-contrast correction on flows in pediatric and congenital cardiac MR compared to phantom correction as the standard. MATERIALS AND METHODS: We retrospectively identified children who had great-vessel phase-contrast and static phantom sequences acquired between January 2015 and June 2015. We applied a novel correction method to each phase-contrast sequence post hoc. Uncorrected, non-linear, and phantom-corrected flows were compared using intraclass correlation. We used paired t-tests to compare how closely non-linear and uncorrected flows approximated phantom-corrected flows. In children without intra- or extracardiac shunts or significant semilunar valvular regurgitation, we used paired t-tests to compare how closely the uncorrected pulmonary-to-systemic flow ratio (Qp:Qs) and non-linear Qp:Qs approximated phantom-corrected Qp:Qs. RESULTS: We included 211 diagnostic-quality phase-contrast sequences (93 aorta, 74 main pulmonary artery [MPA], 21 left pulmonary artery [LPA], 23 right pulmonary artery [RPA]) from 108 children (median age 15 years, interquartile range 11-18 years). Intraclass correlation showed strong agreement between non-linear and phantom-corrected flow measurements but also between uncorrected and phantom-corrected flow measurements. Non-linear flow measurements did not more closely approximate phantom-corrected measurements than did uncorrected measurements for any vessel. In 39 children without significant shunting or regurgitation, mean non-linear Qp:Qs (1.07; 95% confidence interval [CI] = 1.01, 1.13) was no closer than mean uncorrected Qp:Qs (1.06; 95% CI = 1.00, 1.13) to mean phantom-corrected Qp:Qs (1.02; 95% CI = 0.98, 1.06). CONCLUSION: Despite strong agreement between self-calibrated non-linear and phantom correction, cardiac flows and shunt calculations with non-linear correction were no closer to phantom-corrected measurements than those without background correction. However, phantom-corrected flows also demonstrated minimal differences from uncorrected flows. These findings suggest that in the current era, more accurate phase-contrast flow measurements might limit the need for background correction. Further investigation of the clinical impact and optimal methods of background correction in the pediatric and congenital cardiac population is needed.

Trends in cardiac CT utilization for patients with pediatric and congenital heart disease: A multicenter survey study.

BACKGROUND: The use of cardiac CT (CCT) has increased dramatically in recent years among patients with pediatric and congenital heart disease (CHD), but little is known about trends and practice pattern variation in CCT utilization for this population among centers. METHODS: A 21-item survey was created to assess CCT utilization in the pediatric/CHD population in calendar years 2011 and 2021. The survey was sent to all non-invasive cardiac imaging directors of pediatric cardiology centers in North America in September 2022. RESULTS: Forty-one centers completed the survey. In 2021, 98% of centers performed CCT in pediatric and CHD patients (vs. 73% in 2011), and 61% of centers performed >100 CCTs annually (vs. 5% in 2011). While 62% of centers in 2021 utilized dual-source technology for high-pitch helical acquisition, 15% of centers reported primarily performing CCT on a 64-slice scanner. Anesthesia utilization, use of medications for heart rate control, and type of subspecialty training for physicians interpreting CCT varied widely among centers. 50% of centers reported barriers to CCT performance, with the most commonly cited concerns being radiation exposure, the need for anesthesia, and limited CT scan staffing or machine access. 37% (11/30) of centers with a pediatric cardiology fellowship program offer no clinical or didactic CCT training for categorical fellows. CONCLUSION: While CCT usage in the CHD/pediatric population has risen significantly in the past decade, there is broad center variability in CCT acquisition techniques, staffing, workflow, and utilization. Potential areas for improvement include expanding CT scanner access and staffing, formal CCT education for pediatric cardiology fellows, and increasing utilization of existing technological advances.

Ventricular Function and Tissue Characterization By Cardiac MRI in Children Following Hospitalization for Multisystem Inflammatory Syndrome in Children (MIS-C): A Prospective Study.

Background Multisystem Inflammatory Syndrome in Children (MIS-C) is a severe life-threatening manifestation of SARS-CoV-2 infection. Acute cardiac dysfunction and resultant cardiogenic shock are common in children with MIS-C. While most children recover rapidly from acute illness, the long-term impact on the myocardium and cardiac function is unknown. Methods In this prospective study, cardiac MRI (CMR) was performed on patients <21 years of age with a history of MIS-C, 6-9 months following hospitalization. Per institutional protocol, patients with any history of LVEF<50%, persistent cardiorespiratory symptoms, or ECG abnormalities underwent clinical CMR. Research CMRs were offered to all others >10 years old. Native T1 and T2 mapping values were compared with 20 children with normal CMR examinations. Results We performed CMRs on 13 subjects at a median age of 13.6 years (interquartile range [IQR] 11.9-16.0) and a median time from hospitalization of 8.2 months (IQR 6.8-9.6). Twelve subjects displayed normal ventricular function with a median left ventricle ejection fraction (LVEF) of 57.2% (IQR 56.1-58.4) and median right ventricular (RV) EF of 53.1% (IQR 52.0-55.7). One subject had low normal EF (52%). There was normal T2 and native T1 as compared to normal controls. There was qualitatively no evidence of edema by T2 weighted imaging. One subject had late gadolinium enhancement (LGE) at the inferior insertion point and mid-ventricular inferolateral region, with normal EF, no evidence of edema or perfusion defects, and normal T1 and T2 times. When stratifying by a history of abnormal LVEF (LVEF <55%) on echocardiography, there was no difference in or parametric mapping values, though LVEF and LVEDV approached significance (p=0.06 and 0.05, respectively). Conclusions Although many children with MIS-C present acutely with cardiac dysfunction, myocardial recovery is overall excellent with minimal to no evidence of residual cardiac dysfunction or myocardial involvement. LVEF by CMR at 6-9 months among children with history of echocardiographic LV dysfunction is slightly lower, though does not meet statistical significance and is still within normal range. The long-term functional implications of this finding and the cardiac implications of MIS-C more broadly are unclear and warrant further study.

Longitudinal Outcomes for Multisystem Inflammatory Syndrome in Children.

BACKGROUND: In spring 2020, a novel hyperinflammatory process associated with severe acute respiratory syndrome coronavirus 2 multisystem inflammatory syndrome in children (MIS-C) was described. The long-term impact remains unknown. We report longitudinal outcomes from a New York interdisciplinary follow-up program. METHODS: All children <21 years of age, admitted to NewYork-Presbyterian with MIS-C in 2020, were included. Children were followed at 1 to 4 weeks, 1 to 4 months, and 4 to 9 months postdischarge. RESULTS: In total, 45 children were admitted with MIS-C. The median time to last follow-up was 5.8 months (interquartile range 1.3-6.7). Of those admitted, 76% required intensive care and 64% required vasopressors and/or inotropes. On admission, patients exhibited significant nonspecific inflammation, generalized lymphopenia, and thrombocytopenia. Soluble interleukin (IL) IL-2R, IL-6, IL-10, IL-17, IL-18, and C-X-C Motif Chemokine Ligand 9 were elevated. A total of 80% (n = 36) had at least mild and 44% (n = 20) had moderate-severe echocardiographic abnormalities including coronary abnormalities (9% had a z score of 2-2.5; 7% had a z score > 2.5). Whereas most inflammatory markers normalized by 1 to 4 weeks, 32% (n = 11 of 34) exhibited persistent lymphocytosis, with increased double-negative T cells in 96% of assessed patients (n = 23 of 24). By 1 to 4 weeks, only 18% (n = 7 of 39) had mild echocardiographic findings; all had normal coronaries. At 1 to 4 months, the proportion of double-negative T cells remained elevated in 92% (median 9%). At 4 to 9 months, only 1 child had persistent mild dysfunction. One had mild mitral and/or tricuspid regurgitation. CONCLUSIONS: Although the majority of children with MIS-C present critically ill, most inflammatory and cardiac manifestations in our cohort resolved rapidly.

Recommendations for risk stratified use of cardiac computed tomography for congenital heart disease during the COVID-19 pandemic.

The impact of the coronavirus disease (COVID-19) pandemic in the United States and around the world has required significant changes to medical practice. Amidst the rapidly evolving public health emergency, hospital centers have been required to postpone elective procedures, preserve personal protective equipment (PPE), practice social distancing and limit staff exposures. Patients with congenital heart disease (CHD) often need urgent evaluation, most commonly for preprocedural evaluation. We have stratified the most common indications for cardiac computed tomography (CCT) imaging in patients with CHD to help guide care for these patients during the COVID-19 pandemic including considerations for reopening.