Delivering Serious News in Pediatric Cardiology-A Pilot Program.

Pediatric cardiology fellows receive limited training on delivering serious news. This is a teachable skill through simulation-based communication. While studies have shown the use of communication courses in pediatrics, there have been none in pediatric cardiology. Pediatric cardiologists recognize the importance of good communication and desire further development of these skills. Based on an internal needs assessment, three cases were developed; fetal hypoplastic left heart syndrome, teenager with new hypertrophic cardiomyopathy, and young-adult with Fontan failure. A 4-h simulation course using evidence-based methods to teach delivering serious news was designed, consisting of a didactic session, case demonstration and small group case-based encounters with simulated patients. Trainees completed standardized pre/post-course surveys to assess perception of skill and preparedness. Paired survey responses were compared. Six pediatric cardiology fellows participated. Only 33% had received formal training in delivering serious news and 17% in techniques of responding to patient's emotions. The proportion of participants who felt good about their ability to deliver serious news and deal with a family's emotions increased from 0 to 83%. The proportion of participants who felt prepared to provide serious news about a patient's illness increased from 17 to 67%. Given the small number of participants, results were not statistically significant. All participants felt that the course was valuable in improving communication skills. A formal communication course increased perception of skill and preparedness among trainees. We provide an evidence-based framework and clinical cases for delivering serious news in pediatric cardiology, which is generalizable to other training programs.

Kawasaki Disease in the Time of COVID-19 and MIS-C: The International Kawasaki Disease Registry.

BACKGROUND: Patients with multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) have overlapping clinical features. We compared demographics, clinical presentation, management, and outcomes of patients according to evidence of previous SARS-CoV-2 infection. METHODS: The International Kawasaki Disease Registry (IKDR) enrolled KD and MIS-C patients from sites in North, Central, and South America, Europe, Asia, and the Middle East. Evidence of previous infection was defined as: Positive (household contact or positive polymerase chain reaction [PCR]/serology), Possible (suggestive clinical features of MIS-C and/or KD with negative PCR or serology but not both), Negative (negative PCR and serology and no known exposure), and Unknown (incomplete testing and no known exposure). RESULTS: Of 2345 enrolled patients SARS-CoV-2 status was Positive for 1541 (66%) patients, Possible for 89 (4%), Negative for 404 (17%) and Unknown for 311 (13%). Clinical outcomes varied significantly among the groups, with more patients in the Positive/Possible groups presenting with shock, having admission to intensive care, receiving inotropic support, and having longer hospital stays. Regarding cardiac abnormalities, patients in the Positive/Possible groups had a higher prevalence of left ventricular dysfunction, and patients in the Negative and Unknown groups had more severe coronary artery abnormalities. CONCLUSIONS: There appears to be a spectrum of clinical features from MIS-C to KD with a great deal of heterogeneity, and one primary differentiating factor is evidence for previous acute SARS-CoV-2 infection/exposure. SARS-CoV-2 Positive/Possible patients had more severe presentations and required more intensive management, with a greater likelihood of ventricular dysfunction but less severe coronary artery adverse outcomes, in keeping with MIS-C.

Obesity and Outcomes of Kawasaki Disease and COVID-19-Related Multisystem Inflammatory Syndrome in Children.

Importance: Obesity may affect the clinical course of Kawasaki disease (KD) in children and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. Objective: To compare the prevalence of obesity and associations with clinical outcomes in patients with KD or MIS-C. Design, Setting, and Participants: In this cohort study, analysis of International Kawasaki Disease Registry (IKDR) data on contemporaneous patients was conducted between January 1, 2020, and July 31, 2022 (42 sites, 8 countries). Patients with MIS-C (defined by Centers for Disease Control and Prevention criteria) and patients with KD (defined by American Heart Association criteria) were included. Patients with KD who had evidence of a recent COVID-19 infection or missing or unknown COVID-19 status were excluded. Main Outcomes and Measures: Patient demographic characteristics, clinical features, disease course, and outcome variables were collected from the IKDR data set. Using body mass index (BMI)/weight z score percentile equivalents, patient weight was categorized as normal weight (BMI <85th percentile), overweight (BMI ≥85th to <95th percentile), and obese (BMI ≥95th percentile). The association between adiposity category and clinical features and outcomes was determined separately for KD and MIS-C patient groups. Results: Of 1767 children, 338 with KD (median age, 2.5 [IQR, 1.2-5.0] years; 60.4% male) and 1429 with MIS-C (median age, 8.7 [IQR, 5.3-12.4] years; 61.4% male) were contemporaneously included in the study. For patients with MIS-C vs KD, the prevalence of overweight (17.1% vs 11.5%) and obesity (23.7% vs 11.5%) was significantly higher (P < .001), with significantly higher adiposity z scores, even after adjustment for age, sex, and race and ethnicity. For patients with KD, apart from intensive care unit admission rate, adiposity category was not associated with laboratory test features or outcomes. For patients with MIS-C, higher adiposity category was associated with worse laboratory test values and outcomes, including a greater likelihood of shock, intensive care unit admission and inotrope requirement, and increased inflammatory markers, creatinine levels, and alanine aminotransferase levels. Adiposity category was not associated with coronary artery abnormalities for either MIS-C or KD. Conclusions and Relevance: In this international cohort study, obesity was more prevalent for patients with MIS-C vs KD, and associated with more severe presentation, laboratory test features, and outcomes. These findings suggest that obesity as a comorbid factor should be considered at the clinical presentation in children with MIS-C.

Cardiac Biomarkers Aid in Differentiation of Kawasaki Disease from Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show clinical overlap and both lack definitive diagnostic testing, making differentiation challenging. We sought to determine how cardiac biomarkers might differentiate KD from MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C pediatric patients from 42 sites from January 2020 through June 2022. The study population included 118 KD patients who met American Heart Association KD criteria and compared them to 946 MIS-C patients who met 2020 Centers for Disease Control and Prevention case definition. All included patients had at least one measurement of amino-terminal prohormone brain natriuretic peptide (NTproBNP) or cardiac troponin I (TnI), and echocardiography. Regression analyses were used to determine associations between cardiac biomarker levels, diagnosis, and cardiac involvement. Higher NTproBNP (≥ 1500 ng/L) and TnI (≥ 20 ng/L) at presentation were associated with MIS-C versus KD with specificity of 77 and 89%, respectively. Higher biomarker levels were associated with shock and intensive care unit admission; higher NTproBNP was associated with longer hospital length of stay. Lower left ventricular ejection fraction, more pronounced for MIS-C, was also associated with higher biomarker levels. Coronary artery involvement was not associated with either biomarker. Higher NTproBNP and TnI levels are suggestive of MIS-C versus KD and may be clinically useful in their differentiation. Consideration might be given to their inclusion in the routine evaluation of both conditions.

COVID-19 Positive Versus Negative Complete Kawasaki Disease: A Study from the International Kawasaki Disease Registry.

To determine clinical differences for children with complete Kawasaki disease (KD) with and without evidence of preceding SARS-CoV-2 infection. From January 2020, contemporaneous patients with complete KD criteria were classified as either SARS-CoV-2 positive (KDCOVID+; confirmed household exposure, positive PCR and/or serology) or SARS-CoV-2 negative (KDCOVID-; negative testing and no exposure) and compared. Of 744 patients in the International Kawasaki Disease Registry, 52 were KDCOVID- and 61 were KDCOVID+. KDCOVID+ patients were older (median 5.5 vs. 3.7 years; p < 0.001), and all additionally met diagnostic criteria for multisystem inflammatory syndrome in children (MIS-C). They were more likely to have abdominal pain (60% vs. 35%; p = 0.008) and headache (38% vs. 10%; p < 0.001) and had significantly higher CRP, troponin, and BUN/creatinine, and lower hemoglobin, platelets, and lymphocytes. KDCOVID+ patients were more likely to have shock (41% vs. 6%; p < 0.001), ICU admission (62% vs. 10%; p < 0.001), lower left ventricular ejection fraction (mean lowest LVEF 53% vs. 60%; p < 0.001), and to have received inotropic support (60% vs. 10%; p < 0.001). Both groups received IVIG (2 doses in 22% vs. 18%; p = 0.63), but KDCOVID+ were more likely to have received steroids (85% vs. 35%; p < 0.001) and anakinra (60% vs. 10%; p = 0.002). KDCOVID- patients were more likely to have medium/large coronary artery aneurysms (CAA, 12% vs. 0%; p = 0.01). KDCOVID+ patients differ from KDCOVID-, have more severe disease, and greater evidence of myocardial involvement and cardiovascular dysfunction rather than CAA. These patients may be a distinct KD phenotype in the presence of a prevalent specific trigger.

Longitudinal Assessment of Cardiac Function Following Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Multisystem inflammatory syndrome in children (MIS-C) after COVID-19 is commonly associated with cardiac involvement. Studies found myocardial dysfunction, as measured by decreased ejection fraction and abnormal strain, to be common early in illness. However, there is limited data on longitudinal cardiac outcomes. We aim to describe the evolution of cardiac findings in pediatric MIS-C from acute illness through at least 2-month follow-up. A retrospective single-center review of 36 patients admitted with MIS-C from April 2020 through September 2021 was performed. Echocardiographic data including cardiac function and global longitudinal strain (GLS) were analyzed at initial presentation, discharge, 2-4-week follow-up, and at least 2-month follow-up. Patients with mild and severe disease, normal and abnormal left ventricular ejection fraction (LVEF), and normal and abnormal GLS at presentation were compared. On presentation, 42% of patients with MIS-C had decreased LVEF < 55%. In patients in whom GLS was obtained (N = 18), 44% were abnormal (GLS < |- 18|%). Of patients with normal LVEF, 22% had abnormal GLS. There were no significant differences in troponin or brain natriuretic peptide between those with normal and abnormal LVEF. In most MIS-C patients with initial LVEF < 55% (90%), LVEF normalized upon discharge. At 2-month follow-up, all patients had normal LVEF with 21% having persistently abnormal GLS. Myocardial systolic dysfunction and abnormal deformation were common findings in MIS-C at presentation. While EF often normalized by 2 months, persistently abnormal GLS was more common, suggesting ongoing subclinical dysfunction. Our study offers an optimistic outlook for recovery in patients with MIS-C and carditis, however ongoing investigation for longitudinal effects is warranted.

Effect of treadmill exercise stress testing on troponin levels in children and adolescents.

BACKGROUND: Chest pain is a common complaint among paediatric patients and cardiac troponin (cTn) level is often part of the initial emergency department evaluation. It is well known that after intense endurance exercise cTn levels can be elevated in patients with otherwise healthy hearts, however the effect shorter duration exercise has on cTn levels in this population is not known. OBJECTIVE: Determine the behaviour of cTn levels in healthy children and adolescent patients after short burst, high-intensity aerobic exercise. METHODS: Patients without haemodynamically significant heart disease referred for a treadmill exercise stress test (EST) were recruited over a 6-month period. cTn levels were measured prior to exercise and 4 hours after exercise. RESULTS: Thirteen patients enrolled. Indications for EST were exertional syncope (six), chest pain (four), and long QT syndrome (three). The median exercise time was 12.9 (9.9-13.7) minutes with an average endurance at the fiftieth percentile for age and maximum heart rate rose to an average of 92 (74-98)% of the predicted peak for age. cTn levels prior to exercise were undetectable in all patients. There was no cTn rise in any patient after exercise. There were no ischaemic changes or arrhythmias on exercise electrocardiograms. CONCLUSION: Serum cTn levels do not rise significantly in healthy children after short duration, high-intensity aerobic exercise. Physicians evaluating paediatric patients with an elevated cTn level after less than prolonged strenuous activity likely cannot attribute this lab value solely to exercise and may need to undertake further cardiac investigation.

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Strength in numbers: Crowdsourcing the most relevant literature in pediatric cardiology.

OBJECTIVE: The growing body of medical literature in pediatric cardiology has made it increasingly difficult for individual providers to stay abreast of the most current, meaningful articles to help guide practice. Crowdsourcing represents a collaborative process of obtaining information from a large group of individuals, typically from an online or web-based community, and could serve a potential mechanism to pool individual efforts to combat this issue. This study aimed to utilize crowdsourcing as a novel way to generate a list of the most relevant, current publications in congenital heart disease, utilizing input from an international group of professionals in the field of pediatric cardiology. DESIGN AND SETTING: All members of the PediHeartNet Google group, an international email distribution list of medical professionals with an interest in pediatric cardiology, were queried in 2017 to submit literature that they considered to be most relevant to their current practice. A Google Form submission platform was used. The articles were evaluated by a multi-institutional panel of four experts in pediatric cardiology using the Delphi method via an electronic evaluation form until a consensus was reached regarding whether the article merited inclusion in the final list. RESULTS: In total, 260 articles were submitted by members of the PediHeartNet Google group. Expert review using the Delphi method resulted in a list of 108 articles. The final collection of articles was published on a publicly available educational website. CONCLUSIONS: Crowdsourcing represents a novel approach for generating a high-yield, comprehensive, yet practical list of the most relevant recent publications in pediatric cardiology. The same techniques could be easily applied to any medical subspecialty. By enlisting the input of frontline providers, the value and relevance of such a list will be significant. A web-based platform for publication of the list allows for real-time updates to ensure continued relevance.

Pediatric heart disease simulation curriculum: Educating the pediatrician.

BACKGROUND: Training guidelines state that pediatricians should be able to diagnose, manage, and triage patients with heart disease. Acutely ill cardiac patients present infrequently and with high acuity, yet residents receive less exposure to acute cardiac conditions than previous generations. Trainees must learn to manage these situations despite this gap. Simulation has been used successfully to train learners to provide acute care. We hypothesized that a simulation-based cardiac curriculum would improve residents' ability to manage cardiac patients. METHODS: Pediatric residents completed 4 simulation cases followed by debriefing and a computer presentation reviewing the learning objectives. Subjects returned at 1 month for postintervention cases and again at 4-6 months to measure knowledge retention. Cases were scored by 2 raters using a dichotomous checklist. We used repeated measure ANOVA and effect size to compare groups and intra-class correlation (ICC) to assess inter-rater reliability. RESULTS: Twenty-five participants were enrolled. Scores were low on pretesting but showed significant improvement (P < .05) in all 4 cases. No decay was noted on late testing. Pre-post effect sizes ranged from 1.1 to 2.1, demonstrating meaningful improvement. Inter-rater reliability (ICC) ranged from 0.61 to 0.93 across cases. CONCLUSIONS: This novel simulation-based curriculum targets a gap in pediatric training and offers an effective way to train pediatricians. We plan to expand this curriculum to new populations of participants and have integrated it into our resident cardiology rotation.

Diagnosis and Diagnostic Modalities in Pediatric Patients with Elevated Troponin.

Care of adults with coronary artery disease focuses on troponins to rapidly move patients to catheterization. Troponins are increasingly drawn in children, but emergent catheterization may not be indicted. We sought to establish etiologies of troponin elevation and ascertain the yield of diagnostic tests, in this population. Retrospective review of patients from January 1, 2002, to December 31, 2011, who had any elevated troponin during the study period. Patients were excluded for recent cardiac surgery, "significant" congenital heart disease, if they were neonates in the NICU or were on ECMO. Twenty-four patients made up our study group: 17/24 (71 %) had myocarditis or cardiomyopathy. Three had coronary-related diagnoses: 1 ALCAPA and 2 Kawasaki syndrome. The most useful testing for making or confirming the diagnoses included ECG, CXR and ECHO. Fourteen had right heart catheterization which was useful in 10/14. Nine had MRI which was useful in 7/9 (all five cases of suspected myocarditis). Left heart catheterization was completed in 10/24 cases, but in no case made or changed the diagnosis. This study confirms that children with elevated troponins differ from adults. The most common cause is myocarditis or cardiomyopathy, whereas coronary-related ischemia is rare. Diagnosis with ECG, CXR and ECHO is typically adequate. Focused use of right heart catheterization and MRI may be useful. In pediatric patients with elevated troponins, left heart catheterization and coronary angiography should be reserved for a highly selective group, and adult "door-to-balloon time" protocols should not be applied routinely.