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We aimed to study the disparity in the clinical profile and outcomes of hospitalized Multisystem Inflammatory Syndrome in Children (MIS-C) patients at our center. The second goal was to examine the temporal association with preceding SARS-CoV-2 infection by race/ethnicity in our community in Mississippi. We found the racial disparity in the prevalence of MIS-C exceeded its temporal association with SARS-CoV-2 infections. We included 51 consecutive MIS-C patients hospitalized, whose median age was 9 (interquartile range [IQR] 5-12) years, 58% were male, 71% were black, 25% were white, and 4% belonged to other groups. We found a delay between onset of symptoms and hospitalization in black patients compared with white patients with a median of 2 (IQR 0-7) vs median of 0 (0-5) urgent care visits ( P = .022), respectively. Black patients were hospitalized longer (median 8, IQR 2-39 days) than whites (median 5, IQR 3-14 days), P = .047. A total of 38.9% of blacks and 23.1% of whites were admitted to intensive care unit ( P = .498); 36.1% of blacks had severe cardiac involvement vs 23.1% of white patients, P = .531. Future studies of MIS-C are required to improve health equity for children.

Myocarditis comprises many clinical presentations ranging from asymptomatic to sudden cardiac death. The history, physical examination, cardiac biomarkers, inflammatory markers, and electrocardiogram are usually helpful in the initial assessment of suspected acute myocarditis. Echocardiography is the primary tool to detect ventricular wall motion abnormalities, pericardial effusion, valvular regurgitation, and impaired function. The advancement of cardiac magnetic resonance (CMR) imaging has been helpful in clinical practice for diagnosing myocarditis. A recent Scientific Statement by the American Heart Association suggested CMR as a confirmatory test to diagnose acute myocarditis in children. However, standard CMR parametric mapping parameters for diagnosing myocarditis are unavailable in pediatric patients for consistency and reliability in the interpretation. The present review highlights the unmet clinical needs for standard CMR parametric criteria for diagnosing acute and chronic myocarditis in children and differentiating dilated chronic myocarditis phenotype from idiopathic dilated cardiomyopathy. Of particular relevance to today’s practice, we also assess the potential and limitations of CMR to diagnose acute myocarditis in children exposed to severe acute respiratory syndrome coronavirus-2 infections. The latter section will discuss the multi-inflammatory syndrome in children (MIS-C) and mRNA coronavirus disease 19 vaccine-associated myocarditis.

Diastolic dysfunction (DD) refers to abnormalities in the mechanical function of the left ventricle (LV) during diastole. Severe LVDD can cause symptoms and the signs of heart failure (HF) in the setting of normal or near normal LV systolic function and is referred to as diastolic HF or HF with preserved ejection fraction (HFpEF). Pediatric cardiologists have long speculated HFpEF in children with congenital heart disease and cardiomyopathy. However, understanding the risk factors, clinical course, and validated biomarkers predictive of the outcome of HFpEF in children is challenging due to heterogeneous etiologies and overlapping pathophysiological mechanisms. The natural history of HFpEF varies depending upon the patient's age, sex, race, geographic location, nutritional status, biochemical risk factors, underlying heart disease, and genetic-environmental interaction, among other factors. Pediatric onset HFpEF is often not the same disease as in adults. Advances in the noninvasive evaluation of the LV diastolic function by strain, and strain rate analysis with speckle-tracking echocardiography, tissue Doppler imaging, and cardiac magnetic resonance imaging have increased our understanding of the HFpEF in children. This review addresses HFpEF in children and identifies knowledge gaps in the underlying etiologies, pathogenesis, diagnosis, and management, especially compared to adults with HFpEF.

Surgical options for coarctation of aorta (CoA) with atrioventricular septal defect (AVSD) include single-stage repair vs. staged approach with neonatal CoA repair and delayed AVSD repair. The durability of left atrioventricular valve (LAVV) function after neonatal repair is questioned, and the optimal approach remains controversial. Eighteen CoA-AVSD patients who underwent single-stage repair 2005-2015 by a single surgeon were retrospectively analyzed. Fifteen patients had complete and three had partial AVSD. Birth weight was 3.19 kg (2.17-4.08). Age at surgery was 16 days (6-127). One- and ten-year survival were 80% and 69%. Freedom from reintervention was 60% and 40% at one and ten-year respectively. Reinterventions included relief of left ventricular outflow tract obstruction (LVOTO) (n = 4), repair of cleft LAVV (n = 3), and LAVV and aortic valve replacement (n = 1). Freedom from LAVV reintervention was 85.6% and 66% at 1 and 10 years respectively. There were four deaths: two post-operative and two following hospital discharge. Mortality was due to sepsis in three patients, and heart failure related to LVOTO and LAVV insufficiency in one. At 68-month (0.6-144) follow-up the majority had mild or less LAVV regurgitation, and all had normal LV dimension and systolic function. There was no recurrent arch obstruction. Single-stage surgical repair of CoA-AVSD is feasible and reasonable. Survival and freedom from reintervention in our cohort approximate those outcomes of two-stage repair with durable left AV valve function and no recurrent arch obstruction. These patients are frequently syndromic and demonstrate mortality risk from non-cardiac causes. Consideration of a single-staged approach is warranted for appropriate patients with CoA-AVSD.

Patients with repaired Tetralogy of Fallot (rTOF) have decreased exercise capacity (XC) and restrictive lung function (RLF). Our objective was to determine the association between RLF and impaired XC in patients with rTOF. This was a single center retrospective review of patients with rTOF who underwent a cardiopulmonary treadmill exercise testing and spirometry from 2005 to 2015. Patients with a respiratory exchange ratio ≥ 1.05 and peak heart rate > 90% of predicted value were included. Forced vital capacity (FVC) and Forced expiratory volume in 1st second of forceful expiration (FEV1) were used to classify the lung function. Exercise parameters such as peak oxygen uptake (VO2), % of predicted VO2 (%VO2), Metabolic equivalents (METS), and exercise time (ET) were compared between the two groups (i) compared patients with normal lung function (normal FEV1, FVC, and FEV1/FVC > 80%) (ii) RLF (FVC 80%). In our cohort (n = 151, 52% male, mean age ± SD of 22.3 ± 9.1 years), patients with RLF (n = 73) compared to those with normal lung function (n = 86) had a lower peak VO2 (30.8 ± 8.6 vs. 36.6 ± 9.8 mL/kg/min; p

PURPOSE We compared the left ventricular (LV) systolic function in children with cancer before initiation of chemotherapy with matched controls using speckle tracking echocardiography. METHODS AND RESULTS In this retrospective study, we analyzed the echocardiograms of 89 cancer patients before the initiation of chemotherapy and 82 age- (8.4 ± 5.2 vs. 8.9 ± 3.9 years, P = .4) and gender-matched (64% vs. 67%, males, P = .4) healthy controls. Peak systolic LV longitudinal strain (LS) was significantly lower in cancer patients in apical two (-19.8 ± 3.0 vs. -23.5 ± 4.0, P 0.05). CONCLUSION Our data demonstrating abnormalities in LV GLS in pediatric cancer patients even prior to initiation of chemotherapy are novel and perplexing. Further longitudinal follow-up is required to assess the implications of this abnormal LV function in these patients.

Symptomatic, circulatory collapse occurred in an unvaccinated child with repaired congenital heart and a backup pacemaker during an Influenza B viral infection with complete atrioventricular block and pacemaker non-capture. Ventricular arrhythmias occurred during her collapse. Atrioventricular conduction recovered within 24 hours. Influenza-associated cardiac inflammation can adversely affect patients with repaired CHD. Proactive immunisation is strongly recommended.

During initial repair of supracardiac total anomalous pulmonary venous connection (TAPVC), the vertical vein (VV) is sometimes left patent (not ligated or divided) in the hope that this strategy may reduce the likelihood or severity of postoperative pulmonary hypertensive crises. We report a case of a 35-year-old pregnant patient with previously repaired supracardiac TAPVC who presented with atrial arrhythmia and right heart dilation. A cardiac magnetic resonance imaging study confirmed the diagnosis of patency of the vertical vein and right heart dilation. The VV was occluded with a catheter-delivered vascular occlusion device through a percutaneous approach, resulting in resolution of right heart dilation and arrhythmia. This case highlights the role of cross-sectional imaging as an adjunct to echocardiography in adults with repaired congenital heart disease.

Background: Although anthracyclines are effective in the treatment of pediatric cancers, their utility is limited by progressive cardiotoxicity, which can occur years after the completion of chemotherapy. Recent studies have reported abnormalities in left ventricular (LV) global endocardial longitudinal strain (GLS) in patients with cancer even prior to exposure to chemotherapy. The aim of our study was to assess the effect of abnormalities in pre-chemotherapy GLS on the GLS after completion …

Aim:Anthracycline-associated cardiotoxicity in childhood cancer survivors may relate to global or segmental left ventricular abnormalities from associated thromboembolic events and myocardial microinfarcts. We characterized left ventricular segmental changes by two-dimensional speckle-tracking echocardiography in anthracycline-treated asymptomatic childhood cancer survivors.Methods and Results:Childhood cancer survivors’ echocardiograms with normal left ventricular fractional shortening >1 year after anthracycline chemotherapy were studied. Cancer-free control children had normal echocardiograms. Apical two-, three-, and four-chamber peak systolic left ventricular longitudinal and global longitudinal strain, and peak systolic left ventricular radial and circumferential strain at papillary muscle levels were analyzed. The mean (standard deviation) age was 12.7 (3.8) years in 41 childhood cancer survivors. The median (interquartile range) follow-up after anthracycline chemotherapy was 4.73 (2.15–8) years. The median (range) cumulative anthracycline dose was 160.2 (60–396.9) mg/m2. In childhood cancer survivors, the mean (standard deviation) left ventricular longitudinal strain was lower in two- (−18.6 [3.2] versus −21.3 [2.5], p < 0.001), three- (−16.3 [6.0] versus −21.7 [3.0], p < 0.001), and four- (−17.6 [2.7] versus −20.8 [2.0], p < 0.001) chamber views compared to controls. The left ventricular global longitudinal strain (−17.6 [2.7] versus −21.3 [2.0]) and circumferential strain (−20.8 [4.3] versus −23.5 [2.6], p < 0.001) were lower in childhood cancer survivors. Among childhood cancer survivors, 12 out of 16 left ventricular segments had significantly lower longitudinal strain than controls.Conclusions:Asymptomatic anthracycline-treated childhood cancer survivors with normal left ventricular fractional shortening had lower global longitudinal and circumferential strain. The left ventricular longitudinal strain was lower in majority of the segments, suggesting that anthracycline cardiotoxicity is more global than regional.

The anthracycline (AC) group of drugs is widely used for cancer chemotherapy and has improved outcomes in many childhood malignancies. However, cardiovascular complications are major causes of morbidity and mortality in AC recipients, with the greatest risk factor being a higher cumulative dosage. The purpose of this review is to describe the etio-pathogenesis and risk factors of AC induced cardiotoxicity, with emphasis on currently available and emerging modalities of non-invasive imaging in its surveillance, and to review guidelines on its prevention and treatment. Presently, ejection fraction and shortening fraction derived from two-dimensional echocardiography are the most widely used parameter for monitoring of cardiac function in childhood cancer survivors. The newer speckle tracking echocardiography has shown potential to detect abnormalities in ventricular function prior to the conventional measures such as ejection fraction and shortening fraction. When available, three-dimensional echocardiography should be used as it allows for more accurate estimation of ejection fraction. Newer magnetic resonance imaging (MRI) techniques, such as delayed enhancement and T1 mapping, are useful adjuncts for cardiac evaluation in cancer survivors, especially in patients with poor echocardiographic windows. Early detection and management of cardiovascular diseases is one of the major goals in the long-term follow-up of childhood cancer survivors. In addition to conventional two-dimensional echocardiography, newer techniques such as speckle tracking echocardiography and three-dimensional echocardiography should be incorporated due to its ability to detect early changes in anthracycline-induced cardiotoxicity. However further research are needed to guide changes in management due to abnormalities in speckle tracking echocardiography.

BackgroundAcute pulmonary embolism is a life-threatening condition and rarely occurs in children. In adults, catheter-directed therapy emerges as a potentially safer and effective therapeutic option. However, there is a paucity of data on the safety and efficacy of catheter-directed therapy for pulmonary embolism in children. We report a single-centred experience of catheter-directed therapy for acute pulmonary embolism in children.MethodsThis is a retrospective study of children who had no CHD and underwent catheter-directed therapy at Detroit Medical Center during a 12-year period from 2005 to 2017. Demographic and clinical data associated with pulmonary embolism were collected along with the outcome.ResultsA total of nine patients of median age 16 years with the range from 12 to 20 received catheter-directed therapy for sub-massive (n = 6) and massive pulmonary embolism (n = 3). Among nine patients, one patient received Angiojet thrombectomy and balloon angioplasty, whereas eight patients received catheter-directed thrombolysis using tissue plasminogen activator through infusion catheters (n = 3) or EkoSonic ultrasound-accelerated thrombolysis system (n = 5). In four out of five patients treated with EkoSonic, significant clinical improvement was noticed within 24 hours. Among seven patients who survived, two patients had minor gastrointestinal bleeding with median hospital stay of 8 days with the range from 5 to 24 days, and two patients with massive pulmonary embolism died possibly due to delayed institution of catheter-directed therapy.ConclusionCatheter-directed therapy with/without EkoSonic is an emerging alternative therapy for sub-massive and massive pulmonary embolism in children. A timely institution of catheter-directed therapy appeared important to improve the outcome.

Cardiovascular-related morbidity is a substantial health burden in survivors of childhood cancers. This burden is gaining importance as this population increases through advancements in therapy. Anthracyclines are commonly used agents that are known to cause late cardiotoxicity. Cardiotoxicity is also increased by other risk factors, such as concurrent radio- or chemotherapy, younger age at diagnosis, female sex, comorbidities, lifestyle factors, and genetic factors, such as hemochromatosis gene mutations. Treatment of late cardiotoxicity depends on the type of cardiac abnormalities and consists of pharmacotherapy, mechanical support, or heart transplantation. Because cardiotoxicity is progressive and often irreversible, prevention, risk reduction, and early detection are of utmost importance. The cardioprotectant dexrazoxane decreases anthracycline cardiotoxicity. Screening for other risk factors at the time of diagnosis may identify risk that when present, if used to tailor therapy, may reduce the severity of cardiac damage. The effects of exercise and other lifestyle changes in reducing the cardiovascular diseases in cancer survivors are unclear. However, it may be beneficial to encourage survivors to engage in physical activity tailored to survivor medical status, but with close monitoring.

Patients with Tetrology of Fallot (TOF) may have decreased exercise capacity (XC) and abnormal lung function, although the relationship between the two is unclear. We sought to determine the association, if any, between restrictive lung disease (RLD) and XC in patients with repaired TOF. This was a

Context: There is a lacuna of studies on Attention Deficit Hyperactivity Disorder (ADHD) in the Indian context. Aims: (i) To identify the prevalence of ADHD in primary school children, (ii) To identify the gender difference in the prevalence of ADHD, (iii) To compare the distribution of ADHD among different socioeconomic status, (iv) To identify the presence of any co-morbid factors associated with ADHD. Settings and Design: This is a cross sectional study of school aged children selected from four different schools in Coimbatore district. Materials and Methods: Seven hundred seventy children aged between 6 and 11 years were selected from four schools in Coimbatore district after obtaining informed consent from their parents. The presence of ADHD was assessed by using Conner's Abbreviated Rating Scale (CARS) given to parents and teachers. The children identified as having ADHD were assessed for the presence of any co-morbid factors by administering Children's Behavioural Questionnaire (CBQ) to the teachers and Personal Information Questionnaire to the parents. Statistical Analysis: Statistical Product and Service Solutions (SPSS) 10 software, Mean and Standard Deviation, and student's t test were used for statistical analysis. Results: The prevalence of ADHD among primary school children was found to be 11.32%. Prevalence was found to be higher among the males (66.7%) as compared to that of females (33.3%). The prevalence among lower socio-economic group was found to be 16.33% and that among middle socio-economic group was 6.84%. The prevalence was highest in the age group 9 and 10 years. Conclusion: The present study shows a high prevalence of ADHD among primary school children with a higher prevalence among the males than the females.