Pei-Ni Jone

Acute coronary syndrome (ACS) remains underrecognized in patients with Kawasaki disease (KD). The population at risk for KD-associated ACS is increasing, yet current ACS guidelines are largely derived from atherosclerotic disease and do not adequately address the distinctive mechanisms, anatomy, and thrombosis risk associated with KD. Because the pathophysiology and management of KD-associated ACS differ in fundamental ways from those of atherosclerotic coronary artery disease, its management requires a coordinated team response, with collaboration between pediatric and adult cardiologists. Recent advances in coronary imaging, interventional techniques, and antithrombotic therapies provide an opportunity to establish a more disease-specific approach. This review synthesizes current evidence on the epidemiology and pathophysiology of coronary artery aneurysms, interventional coronary imaging, acute management, revascularization strategies, antithrombotic therapy, and post-ACS surveillance in patients with KD to guide clinical decision-making and treatment for patients with KD presenting with ACS.

Right ventricular (RV) failure is a principal determinant of morbidity and mortality in children with pulmonary hypertension, making accurate RV assessment a cornerstone of risk stratification and long-term management. Noninvasive imaging plays a central role in this evaluation; however, commonly used modalities, including 2- and 3-dimensional echocardiography and cardiac magnetic resonance imaging, each have distinct advantages and limitations in the pediatric population. Consequently, an integrated, multimodal imaging strategy is required. This review provides a contemporary, critical appraisal of the existing evidence and key knowledge gaps related to noninvasive multimodality imaging of the RV in pediatric pulmonary hypertension. The discussion is structured around fundamental aspects of RV physiology, including chamber size and mass, systolic function, diastolic function and stiffness, RV-left ventricle interactions, ventriculoarterial coupling, and exercise assessment. Echocardiography and cardiac magnetic resonance imaging are presented in parallel to highlight their complementary roles within a multimodality framework. Current prognostic thresholds in pediatric pulmonary hypertension imaging are largely supported by level of evidence C, underscoring persistent gaps that limit the development of definitive clinical recommendations and a unified approach. We propose a roadmap to guide future research efforts and collaborative initiatives among pediatric pulmonary hypertension and imaging specialists, emphasizing the role of professional networks in advancing the field.

BACKGROUND: 2D-speckle tracking echocardiography may help detect subclinical ventricular dysfunction, but data in multisystem inflammatory syndrome in children (MIS-C) are scarce. We investigated left ventricular (LV) strain parameters in MIS-C and their association with outcomes. METHODS: We performed an ambi-directional, 32-center cohort study on hospitalized MIS-C patients (March 2020–November 2021) with at least 1 echocardiogram read by the Core Lab. Generalized estimating equation modeling was used to test associations between LV strain and a composite in-hospital adverse cardiovascular outcome (vasoactive support, arrhythmias, cardiac arrest, extracorporeal support, death, or heart transplant). RESULTS: Of 349 patients (median age, 8.7 years [interquartile range, 5.3–12.9]), 35% had decreased LV ejection fraction during hospitalization, and 45% had depressed LV strain (either 4-chamber LV longitudinal strain [4CH-LVLS] or mid-ventricular LV circumferential strain [mid-LVCS]). The worst 4CH-LVLS and mid-LVCS occurred at ≈5 days of illness; 50% of abnormal LV strain normalized within 1 week, and 95% within 50 days. In-hospital adverse outcomes occurred in 35% of patients; these patients were older ( P =0.003) and, at admission, had more likely abnormal troponin ( P P P P =0.015), worse LV ejection fraction ( P P =0.002; mid-LVCS, P =0.001). Covariate-adjusted individual models for each strain parameter showed that 4CH-LVLS (adjusted odds ratio, 1.09 [95% CI, 1.07–1.12]), mid-LVCS (adjusted odds ratio, 1.06 [95% CI, 1.04–1.09]), worst LV strain Z score between 4CH-LVLS and mid-LVCS (adjusted odds ratio, 1.30 [95% CI, 1.21–1.41]), and early diastolic longitudinal strain rate (EDLSR; adjusted odds ratio, 1.68 [95% CI, 1.26–2.23]) at admission were found to be associated with adverse outcomes. CONCLUSIONS: About half of MIS-C patients had abnormal LV strain during hospitalization. 4CH-LVLS, mid-LVCS, the most abnormal strain Z score, and early diastolic longitudinal strain rate at admission were independently associated with in-hospital adverse cardiovascular outcome. These data may help early characterization and prognostication in MIS-C.

BACKGROUND: Multisystem Inflammatory Syndrome in Children is characterized by high rates of acute cardiovascular involvement with rapid recovery of organ dysfunction. However, information regarding long-term sequelae is lacking. We sought to characterize the systolic function and myocardial tissue properties using cardiac magnetic resonance (CMR) imaging in a multicenter observational cohort of Multisystem Inflammatory Syndrome in Children patients. METHODS: In this observational cohort study, comprising 32 centers in North America, CMR studies were analyzed by a core laboratory to assess ventricular volumetric data, tissue characterization, and coronary involvement. RESULTS: A total of 263 CMRs from 255 Multisystem Inflammatory Syndrome in Children patients were analyzed. The mean patient age was 11.4±4.4 years. Most studies were performed at 3 months (33%) or 6 months (45%) after hospitalization. Left ventricular dysfunction was present in 17 (6.7%) of the first CMRs and was never worse than mild. Dysfunction was observed in 4/7 (57%) patients at admission, 5/87 (6.9%) patients at 3 months, and 6/129 (4.6%) patients imaged either at 6 months or 1 year post-hospitalization. Late gadolinium enhancement was present in 2 (0.8%) patients, 1 at 3 months and another at 6 months following hospitalization. Coronary artery dilation was present in 13 of the 174 (7.5%) patients. Nine patients met the Lake Louise criteria for myocarditis (3.5%) at the time of CMR. CONCLUSIONS: In this largest published multiinstitutional longitudinal CMR evaluation of confirmed Multisystem Inflammatory Syndrome in Children patients, the prevalence of ventricular dysfunction and myocardial tissue characterization abnormalities on medium-term follow-up was low. However, a small number of patients had mild residual abnormalities at 6 months and 1 year following hospitalization. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT05287412.

Background Pediatric echocardiograms are performed daily on inpatient hospital units, predominantly in the Cardiac Intensive Care Unit. Understanding the precise study indication from the ordering providers is critical for completing imaging. Given the volume of echocardiograms ordered daily and staffing shortages, timely completion of echocardiograms is important for maintaining efficiency and productivity. The aim of this study was to identify areas to improve efficiency using quality improvement methodologies. Methods The SMART aim was to decrease the total echo completion time by 10% within 6 months. Using a fishbone diagram, limitations in the ordering system and inadequate ordering provider knowledge were identified as areas for improvement. Primary process and outcome metrics included total echo completion time, pre-echocardiogram buffer time, and order documentation of pertinent clinical history. PDSA 1 implemented order updates to provide increased specificity in orders with a picklist of common reasons for inpatient echocardiograms. PDSA 2 implemented a physician alert for STAT studies to reduce the number of inappropriate orders. PDSA 3 implemented additional order updates and staff education. Results Total echo completion time decreased with each cycle for total 25% reduction. This correlated with a decrease in the pre-echocardiogram buffer time of 56% without any significant changes in the number of images obtained per study or the daily inpatient volume. The documentation of pertinent clinical history increased by 18% and maintained at 99% appropriate documentation for the final two PDSA cycles. Pre and post intervention surveys indicated an overall improvement in the ordering process for both the inpatient ordering providers and echocardiography laboratory staff. Conclusions Echocardiography laboratory efficiency significantly improved throughout the course of this project without negative effects on the ordering providers or echocardiography laboratory staff. Our project demonstrates the ability to affect clinically relevant improvements utilizing simple workflow process changes in the pediatric echocardiography laboratory.

Echocardiography is the main modality in diagnosing acquired and congenital heart disease (CHD) in fetal and pediatric patients. However, operator variability, complex image interpretation, and lack of experienced sonographers and cardiologists in certain regions are the main limitations existing in fetal and pediatric echocardiography. Advances in artificial intelligence (AI), including machine learning (ML) and deep learning (DL), offer significant potential to overcome these challenges by automating image acquisition, image segmentation, CHD detection, and measurements. Despite these promising advancements, challenges such as small number of datasets, algorithm transparency, physician comfort with AI, and accessibility must be addressed to fully integrate AI into practice. This review highlights AI’s current applications, challenges, and future directions in fetal and pediatric echocardiography.

Importance: The prevalence, pathophysiology, and long-term outcomes of COVID-19 (post-acute sequelae of SARS-CoV-2 [PASC] or “Long COVID”) in children and young adults remain unknown. Studies must address the urgent need to define PASC, its mechanisms, and potential treatment targets in children and young adults. Observations: We describe the protocol for the Pediatric Observational Cohort Study of the NIH’s REsearching COVID to Enhance Recovery (RECOVER) Initiative. RECOVER-Pediatrics is an observational meta-cohort study of caregiver-child pairs (birth through 17 years) and young adults (18 through 25 years), recruited from more than 100 sites across the US. This report focuses on two of four cohorts that comprise RECOVER-Pediatrics: 1) a de novo RECOVER prospective cohort of children and young adults with and without previous or current infection; and 2) an extant cohort derived from the Adolescent Brain Cognitive Development (ABCD) study (n = 10,000). The de novo cohort incorporates three tiers of data collection: 1) remote baseline assessments (Tier 1, n = 6000); 2) longitudinal follow-up for up to 4 years (Tier 2, n = 6000); and 3) a subset of participants, primarily the most severely affected by PASC, who will undergo deep phenotyping to explore PASC pathophysiology (Tier 3, n = 600). Youth enrolled in the ABCD study participate in Tier 1. The pediatric protocol was developed as a collaborative partnership of investigators, patients, researchers, clinicians, community partners, and federal partners, intentionally promoting inclusivity and diversity. The protocol is adaptive to facilitate responses to emerging science. Conclusions and relevance: RECOVER-Pediatrics seeks to characterize the clinical course, underlying mechanisms, and long-term effects of PASC from birth through 25 years old. RECOVER-Pediatrics is designed to elucidate the epidemiology, four-year clinical course, and sociodemographic correlates of pediatric PASC. The data and biosamples will allow examination of mechanistic hypotheses and biomarkers, thus providing insights into potential therapeutic interventions. Clinical trials.gov identifier: Clinical Trial Registration: http://www.clinicaltrials.gov. Unique identifier: NCT05172011.