Heart Disease In Children Research Articles

Neurodevelopmental assessment of children with congenital heart diseases using Trivandrum developmental screening chart.

Congenital heart diseases (CHDs) are one of the most important congenital anomalies in children which have high-risk for neurodevelopment delay. This study was conducted to determine the proportion of developmental delay in children with CHD and comparison of delay between acyanotic and cyanotic heart diseases in children. A cross-sectional study was conducted on children admitted in pediatric ward of rural hospital from 6 month to 6 years of age who are diagnosed with CHD by 2D ECHO and further classified into acyanotic congenital heart disease (ACHD) and cyanotic congenital heart disease (CCHD). Neurodevelopmental assessment was done using Trivandrum development screening chart (TDSC). Out of total 50 children in study population, 24 children had TDSC delay, distribution as 11 (22%) ACHD and 13 (26%) CCHD. Out of 24 children in the age group of 0-3 years, 13 (54.2%) were ACHD and 11 (45.8%) were CCHD. Out of 26 children in the age group of 3-6 years, 15 (57.7%) were ACHD and 11 (42.3%) were CCHD. Among different ACHD included in the study population (0-3 years) children with ventricular septal defect (VSD) were maximum (n = 5) next in the decreasing order was atrial septal defect (ASD) (n = 3). ACHD included in the study population (3-6 years) children with VSD was maximum (n = 6) next in the order was ASD (n = 4). Proportion of delay in children with ACHD was 22% as compared to 26% in children with CCHD. There is a high proportion of neurodevelopmental delay in children with CHD which can be detected using TDSC which is a simple screening tool and can be used by any health-care professional without training for the assessment of neurodevelopmental outcome in these children. Delay was more in children with CCHD than ACHD.

E45 Kawasaki syndrome-related heart disease before and during the COVID-19 pandemic

Abstract Introduction Kawasaki disease (kDa) is an acute febrile systemic panvascularitis that affects medium-caliber arteries with a strong predilection for the coronary arteries. It is the leading cause of acquired heart disease in children under 5 years of age. However, after the COVID-19 pandemic, we see a change in the profile of cardiac involvement. Objective To describe the clinical, paraclinical, and evolutionary characteristics of cardiac damage in children managed for Kawasaki or Kawasaki-Like syndrome before and during the Covid19 pandemic. Materials and Methods This is a retrospective, descriptive study conducted over a period of five years (1st January 2017 to 31st December 2021). Result We collected 30 cases of patients hospitalized with Kawasaki disease, between January 2017 and December 2021: 11 cases with Kawasaki syndrome before COVID-19; and 19 cases with Kawasaki syndrome after COVID-19. The mean age and standard deviation (SD) at diagnosis was 42 (±37.8) months before the pandemic vs 81 (±34.7) months after the pandemic. The sex ratio was 0.5 (4 girls and 7 boys) before the pandemic vs 0.7 (8 girls and 11 boys) after the pandemic. Heart damage was found in 2 cases before the pandemic (coronary aneurysm type) vs 3 cases after the pandemic, mainly cardiogenic shock. The mean time from the first transthoracic ultrasound to fever was 11.3 days before the pandemic, vs 5.8 days after the pandemic. Transthoracic ultrasound showed 2 cases of coronary aneurysm before the pandemic, with a Z score exceeding 2.5SD; vs 7 cases of heart failure after the pandemic with a decrease in left ventricular ejection fraction. The ASAI score (a score that assesses the risk of coronary artery disease) was >6 in 1 case before the pandemic vs in 0 cases after the pandemic. The average predictive score for cardiac involvement was 4.2 (±1.4) before the pandemic, vs 3.5 (±1.5) after the pandemic. The outcome was favorable in all cases despite a more severe picture after the COVID-19 pandemic. Conclusion The change in the pattern of cardiac involvement in Kawasaki disease following the emergence of COVID-19 must be taken into account in order to manage an influx of patients with severe Kawasaki disease.

A Deep Learning Framework for Image-Based Screening of Kawasaki Disease.

Kawasaki disease (KD) is a leading cause of acquired heart disease in children and is characterized by the presence of a combination of five clinical signs assessed during the physical examination. Timely treatment of intravenous immunoglobin is needed to prevent coronary artery aneurysm formation, but KD is usually diagnosed when pediatric patients are evaluated by a clinician in the emergency department days after onset. One or more of the five clinical signs usually manifests in pediatric patients prior to ED admission, presenting an opportunity for earlier intervention if families receive guidance to seek medical care as soon as clinical signs are observed along with a fever for at least five days. We present a deep learning framework for a novel screening tool to calculate the relative risk of KD by analyzing images of the five clinical signs. The framework consists of convolutional neural networks to separately calculate the risk for each clinical sign, and a new algorithm to determine what clinical sign is in an image. We achieved a mean accuracy of 90% during 10-fold cross-validation and 88% during external validation for the new algorithm. These results demonstrate the algorithms in the proposed screening tool can be utilized by families to determine if their child should be evaluated by a clinician based on the number of clinical signs consistent with KD.Clinical Relevance- This screening framework has the potential for earlier clinical evaluation and detection of KD to reduce the risk of coronary artery complications.

Prophylactic use of levosimendan in preoperative setting for surgical repair of congenital heart disease in children.

Low cardiac output syndrome (LCOS) is a significant cause of morbidity and the leading cause of mortality after pediatric cardiac surgery. Levosimendan has been shown safe and effective in pediatrics to treat LCOS. We aimed to review our local strategy with preoperative prophylactic Levosimendan infusion to minimize LCOS after heart surgery in identified high-risk patients. Retrospective monocentric study. As there is no reliable cardiac output measurement in children, we recorded hemodynamic parameters as surrogates of cardiac output after extracorporeal circulation through an electronic patient survey system at different time points. Seventy-two children received Levosimendan before surgery between 2010 and 2019. As expected, most patients were newborns and infants with prolonged open-heart surgeries. Median cardiopulmonary bypass time was 182 [137-234] min, and aortic clamping time was 95 [64-126] min. The postoperative hemodynamic parameters, vasoactive-inotropic score, and urine output remained stable throughout the first 48 h. Only a tiny portion of the patients had combined surrogate markers of LCOS with a maximal median arterial lactate of 2.6 [1.9-3.5] mmol/L during the first six postoperative hours, which then progressively normalized. The median arterio-venous difference in oxygen saturation was 31 [23-38] % between 12 and 18 h post-surgery and gradually decreased. The median venous-to-arterial CO2 difference was the highest at 10 [7-12] mmHg between 12 and 18 h post-surgery. Nine patients (13%) required extracorporeal membrane oxygenation. No patient required dialysis or hemofiltration. Mortality was 0%. Before congenital heart surgery, preoperative prophylactic administration of Levosimendan seems effective and safe for decreasing occurrence and duration of LCOS in high-risk children.

Factors associated with the uncorrectable congenital heart disease in children with pulmonary arterial hypertension

BACKGROUND Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common complication of uncorrected left-to-right shunt defects in acyanotic CHD and a frequent type of pulmonary hypertension in youth. The standards for operability in left-to-right shunts with increased pulmonary vascular resistance are not universally agreed upon. This study aimed to identify variables associated with uncorrectable lesion in children with PAH-CHD.
 METHODS This retrospective study used a database of all children who underwent cardiac catheterization at Sanglah Hospital, Bali, from May 2009 to April 2021. Pulmonary hypertension was defined as pulmonary artery pressure of >25 mmHg, while correctability was a fall of >20% in the pulmonary arterial resistance index (PARI) with final value of <6 WU/m2 when doing an acute vasoreactivity test using 100% oxygen. The analyses were carried out using SPSS software version 22.0 (IBM Corp., USA).
 RESULTS A total of 104 children were included. Cardiac catheterization showed that the uncorrectable group had a higher PARI (14.4 [8.88] WU/m2 versus 8.43 [3.85] WU/m2) and lower flow ratio (1.27 [0.83] versus 1.47 [0.77]) at baseline. In terms of correctability, pre-tricuspid lesions (OR = 0.05; 95% CI = 0.01–0.47; p = 0.01) and younger age group (OR = 0.32; 95% CI = 0.12–0.85; p = 0.01) were protective variables, whilst high baseline PARI (OR = 4.54; 95% CI = 1.64–12.57; p = 0.01) was unfavorable.
 CONCLUSIONS High baseline PARI was the most significant variable in predicting uncorrectable left-to-right shunt defects in PAH-CHD.

HPC+ in the medical field: Overview and current examples.

To say data is revolutionising the medical sector would be a vast understatement. The amount of medical data available today is unprecedented and has the potential to enable to date unseen forms of healthcare. To process this huge amount of data, an equally huge amount of computing power is required, which cannot be provided by regular desktop computers. These areas can be (and already are) supported by High-Performance-Computing (HPC), High-Performance Data Analytics (HPDA), and AI (together "HPC+"). This overview article aims to show state-of-the-art examples of studies supported by the National Competence Centres (NCCs) in HPC+ within the EuroCC project, employing HPC, HPDA and AI for medical applications. The included studies on different applications of HPC in the medical sector were sourced from the National Competence Centres in HPC and compiled into an overview article. Methods include the application of HPC+ for medical image processing, high-performance medical and pharmaceutical data analytics, an application for pediatric dosimetry, and a cloud-based HPC platform to support systemic pulmonary shunting procedures. This article showcases state-of-the-art applications and large-scale data analytics in the medical sector employing HPC+ within surgery, medical image processing in diagnostics, nutritional support of patients in hospitals, treating congenital heart diseases in children, and within basic research. HPC+ support scientific fields from research to industrial applications in the medical area, enabling researchers to run faster and more complex calculations, simulations and data analyses for the direct benefit of patients, doctors, clinicians and as an accelerator for medical research.

Study to assess incidence and type of congenital heart defect in children with down syndrome presenting to tertiary care teaching hospital of Southern Rajasthan

Background: Down syndrome occurs in people of all races and economic levels. Cardiac anomalies with a prevalence of about 50% are the most common anomaly responsible for death during the 1st two years of life in children with down syndrome. Objective of the study was to Assess the incidence and type of congenital heart disease in children with down syndrome presenting to a tertiary care hospital. Methods: Hospital based descriptive study. Participants: All children (0 to 18 years) diagnosed (clinically or on karyotyping) with Down syndrome, presenting in RNT Medical College, Udaipur Each patient was evaluated with a pre-set Performa which contained detailed sociodemographic profile, maternal and clinical history including age of conception, detailed physical examination to look for features of down syndrome. Degree of Intellectual / Developmental retardation was assessed by Vineland Social Maturity Scale. 2D- echo, karyotyping was done in all patient. CBC, thyroid profile, CXR, USG- abdomen, Invertogram were done as per need. Results: Total 144 participants included in the study, 75 (52%) were females and rest were males 48%. Out of these 111 (77%) were aware about dog bite causes disease and among these 52 (46.8%) were aware that dog bite causes rabies. Among the participants, 138 (95.8%) were aware about the vaccine availability and 125 (90.5%) knew vaccine is available in Government Hospital. In the study 88% of dog bite victims received treatment. Conclusions: More than half of children with Down syndrome have congenital heart disease, mostly Acyanotic, more specifically VSD. Early screening and diagnosis re the key to avoid irreversible hemodynamic consequences.

Clinical analysis and medium-term follow-up of simultaneous interventional therapy for compound congenital heart disease in children: a single-center retrospective study.

This study aimed to explore the safety and efficacy of simultaneous interventional therapy for compound congenital heart disease (CCHD) in children. In total, 155 children with CCHD who received simultaneous interventional therapy at the Children's Hospital of Chongqing Medical University between January 2007 and December 2021 were included in study. Data on clinical manifestations, transthoracic echocardiography, electrocardiogram, and follow-up were retrospectively analyzed. The most common type of CCHD was atrial septal defect (ASD) combined with ventricular septal defect (VSD), accounting for 32.3% of the patients. Simultaneous interventional therapy was successfully administered to 151 children (97.4%). The pulmonary gradient of patients with pulmonary stenosis decreased from 47.3 ± 21.9 mmHg to 15.2 ± 12.2 mmHg (P < 0.05) immediately after the procedure. One patient had failed PBPV as he had residual PS >40 mmHg post procedure. The right ventricular dimension and left ventricular end-diastolic dimension significantly decreased in the first month after the procedure in patients with ASD combined with VSD. Twenty-five (16.1%) patients had mild residual shunt, which spontaneously disappeared in more than half of these patients 6 months after the procedure. The major adverse events were minimal (n = 4, 2.58%), including one patient requiring drug treatment for complete atrioventricular block and three patients receiving surgical treatment because of cardiac erosion, anterior tricuspid valve chordae rupture, and hemolysis, respectively. ASD combined with VSD is the most common type of CCHD in children, and simultaneous interventional therapy for CCHD in children is safe and effective with satisfactory results. Ventricular remodeling can be reversed in patients with ASD combined with VSD 1 month after the procedure. Most adverse events associated with interventional therapy are mild and manageable.

Incidence and clinical outcomes of pneumonia in persons with down syndrome in the United States

BackgroundPersons with Down syndrome (DS) experience an increased risk of pneumonia. We determined the incidence and outcomes of pneumonia and relationship to underlying comorbidities in persons with and without DS in the United States. MethodsThis retrospective matched cohort study used de-identified administrative claims data from Optum. Persons with DS were matched 1:4 to persons without DS on age, sex, and race/ethnicity. Pneumonia episodes were analyzed for incidence, rate ratios and 95 % confidence intervals, clinical outcomes, and comorbidities. ResultsDuring 1-year follow-up among 33796 persons with and 135184 without DS, the incidence of all-cause pneumonia (pneumonia) was substantially higher among people with DS than those without DS (12427 vs. 2531 episodes/100000 person-years; 4.7–5.7 fold increase). Persons with DS and pneumonia were more likely to be hospitalized (39.4 % vs. 13.9 %) or admitted to the ICU (16.8 % vs. 4.8 %). Mortality was higher 1 year after first pneumonia (5.7 % vs. 2.4 %; P < 0.0001). Results were similar for episodes of pneumococcal pneumonia. Specific comorbidities were associated with pneumonia, particularly heart disease in children and neurologic disease in adults, which only partially mediated the effect of DS on pneumonia. ConclusionsAmong persons with DS, incidence of pneumonia and associated hospitalizations were increased; mortality among those with pneumonia was comparable at 30 days, but higher at 1 year. DS should be considered an independent risk condition for pneumonia.

Clinical profile of congenital heart disease in children with special reference to echo correlation

Background: Congenital heart disease (CHD) is the most frequently occurring congenital disorder responsible for 1/3rd of all congenital birth defects. Diagnostic accuracy of CHD is crucial for timely intervention and optimal patient outcome. So, the study has been done to look for the clinical profile and establishing correlation of clinical diagnosis with echocardiography diagnosis. Methods: This is a prospective cross-sectional study conducted in the department of pediatrics MKCG MCH, Berhampur over a period of 2 years. All children between 1 month to 14 years admitted for suspected CHD were included in the study. Clinical diagnosis was made based on detailed history and physical examination supported by chest X-ray and ECG. Final diagnosis was confirmed by echocardiography and clinico-ECHO correlation was deduced. Results: The 188 patients with CHD were included in this study. The most common age of presentation was 1 month to 1 year with 71.2%, male to female ratio was 1.06:1. Acyanotic CHD (ACHD) was found to be 70.29%, while 29.7% had congenital cyanotic heart disease (CCHD). Clinically specific diagnosis was attributed to 67.1% of cases and no specific diagnosis could be made in 32.9%. Isolated CHD was found to be 66.4% and Complex CHD with multiple defects was found to be 33.6%. Clinic echo correlation was accurate in 39.4% cases, partially correlated in 32% and no correlation in 28.6% cases. Conclusions: So, echocardiography remains the gold standard for diagnosis of CHD and guiding the treatment. Clinical diagnosis alone may not detect associated CHD.

The ‘Ironclad friendship’ of China-Cambodia, lays the first step in the foundation of early diagnosis and treatment of asymptomatic congenital heart Defects- A multi-national screening and intervention project, 2017–2020

BackgroundCongenital heart disease (CHD) is the leading cause of mortality in childhood worldwide. However, a large number of children with CHD are not diagnosed promptly in low- and middle-income regions, due to limited healthcare resources and lack the ability of prenatal and postnatal ultrasound examinations. The research on asymptomatic CHD in the community is still blank, resulting in a large number of children with asymptomatic CHD can not be found and treated in time. Through the China-Cambodia collaborative health care initiative, the project team conducted research, screened children’s CHD through a sampling survey in China and Cambodia, collected relevant data, and retrospectively analyzed the data of all eligible patients.ObjectivesThe project aimed to evaluate the prevalence of asymptomatic CHD in a sample population of 3-18years old and effects on their growth status and treatment outcomes.MethodsWe examined the prevalence of ‘asymptomatic CHD’ among 3-18years old children and adolescents at the township/county levels in the two participating. A total of eight provinces in China and five provinces in Cambodia were analyzed from 2017 to 2020. During 1 year follow-up after treatment, the differences in heights and weights of the treated and control groups were evaluated.ResultsAmong the 3,068,075 participants screened from 2017 to 2020, 3967 patients with asymptomatic CHD requiring treatment were identified [0.130%, 95% confidence interval (CI) 0.126 -0.134%]. The prevalence rate of CHD ranged from 0.02 to 0.88%, and was negatively related to local per capita GDP (p = 0.028). The average height of 3310 treated CHD patients were 2.23% (95% CI: -2.51%~-1.9%) lower than that of the standard group and the average weight was − 6.41% (95% CI: -7.17%~-5.65%) lower, the developmental gap widening with advancing age. One year after treatment, the relative height difference remained comparable while that, in weight was reduced by 5.68% (95% CI: 4.27% ~7.09%).ConclusionsAsymptomatic CHD now is often overlooked and is an emerging public health challenge. Early detection and treatment are essential to lower the potential burden of heart diseases in children and adolescents.

Differentiation between Lymph-Node-First Presentation of Kawasaki Disease from Bacterial Cervical Lymphadenitis

Kawasaki disease (KD) is the leading cause of acquired heart disease in children. If the patients are left untreated, coronary artery aneurysms may occur. Among the major criteria for KD, lymphadenopathy is the least common. Thus, lymph node-first presentation KD (NFKD) may be overlooked, resulting in coronary artery dilatation. Pediatric patients with fever and cervical lymphadenopathy should be cautiously evaluated for an accurate diagnosis and to minimize complications. Laboratory findings and imaging studies may help make a diagnosis in these patients. We discussed eight previous studies and compared the results of laboratory and imaging findings to differentiate between NFKD from bacterial cervical lymphadenitis (BCL).

A Case of a Third Attack of Recurrent Node-First Kawasaki Disease with Retropharyngeal Edema

Kawasaki disease (KD) is currently the leading cause of acquired heart disease in children. Since the diagnosis of KD still depends on clinical features excluding underlying diseases, it remains a challenge if clinical signs of KD and other infections overlap. We experienced a case of a 6-year-old boy who initially presented with a severe suppurative neck infection. The patient repeatedly showed similar clinical findings three times, with severe inflammation of the skin on the enlarged cervical lymph nodes, but remained refractory to antibiotics treatment. Cervical computed tomography revealed retropharyngeal edema, and in the operative field, the patient showed a serous discharge from the edematous retropharyngeal wall. All underlying microbial pathogen tests were negative. The patient subsequently presented typical features of KD on the 6th day from the onset of fever. He was treated with intravenous immunoglobulin and aspirin, and he antibiotics were discontinued. Here, we report a case of a third attack of recurrent node-first KD with a literature review.

Identification of key signaling pathways and hub genes related to immune infiltration in Kawasaki disease with resistance to intravenous immunoglobulin based on weighted gene co-expression network analysis.

Background: Kawasaki disease (KD) is an acute vasculitis, that is, the leading cause of acquired heart disease in children, with approximately 10%-20% of patients with KD suffering intravenous immunoglobulin (IVIG) resistance. Although the underlying mechanism of this phenomenon remains unclear, recent studies have revealed that immune cell infiltration may associate with its occurrence. Methods: In this study, we downloaded the expression profiles from the GSE48498 and GSE16797 datasets in the Gene Expression Omnibus database, analyzed differentially expressed genes (DEGs), and intersected the DEGs with the immune-related genes downloaded from the ImmPort database to obtain differentially expressed immune-related genes (DEIGs). Then CIBERSORT algorithm was used to calculate the immune cell compositions, followed by the WGCNA analysis to identify the module genes associated with immune cell infiltration. Next, we took the intersection of the selected module genes and DEIGs, then performed GO and KEGG enrichment analysis. Moreover, ROC curve validation, Spearman analysis with immune cells, TF, and miRNA regulation network, and potential drug prediction were implemented for the finally obtained hub genes. Results: The CIBERSORT algorithm showed that neutrophil expression was significantly higher in IVIG-resistant patients compared to IVIG-responsive patients. Next, we got differentially expressed neutrophil-related genes by intersecting DEIGs with neutrophil-related module genes obtained by WGCNA, for further analysis. Enrichment analysis revealed that these genes were associated with immune pathways, such as cytokine-cytokine receptor interaction and neutrophil extracellular trap formation. Then we combined the PPI network in the STRING database with the MCODE plugin in Cytoscape and identified 6 hub genes (TLR8, AQP9, CXCR1, FPR2, HCK, and IL1R2), which had good diagnostic performance in IVIG resistance according to ROC analysis. Furthermore, Spearman's correlation analysis confirmed that these genes were closely related to neutrophils. Finally, TFs, miRNAs, and potential drugs targeting the hub genes were predicted, and TF-, miRNA-, and drug-gene networks were constructed. Conclusion: This study found that the 6 hub genes (TLR8, AQP9, CXCR1, FPR2, HCK, and IL1R2) were significantly associated with neutrophil cell infiltration, which played an important role in IVIG resistance. In a word, this work rendered potential diagnostic biomarkers and prospective therapeutic targets for IVIG-resistant patients.

Frequency of Cyanotic Heart Disease in Infants of Diabetic Mothers

Aim: To determine the frequency of cyanotic heart disease in infants born to diabetic mothers referred to Tertiary Care Hospital. Materials: This Cross-Sectional Study was conducted in the Pediatrics Department of Hayatabad Medical Complex, Peshawar from July 2021 to July 2022. In this study, a total of 262 patients were to determine the frequency of cyanotic heart disease in infants born to diabetic mothers referred to the Pediatrics Department of Hayatabad Medical Complex, Peshawar. The sampling technique was non-probability consecutive sampling. Results: The age of the participants among 262 patients was analyzed as n= 1-15 Days was 170(64.9%) 16-28 Days was 92(35.1%). The mean age was 9±1.87 days. The distribution of gender among 262 patients was analyzed as n= Male was 142(54.2%) and Female was 120(45.8%).The distribution of weight among 262 patients was analyzed as n= less than or Equal to 4.5 kg was 126(48.1%) and More than 4.5 kg was 136(51.9%). Mean weight 5.1±1.87kg. Distribution of Duration of diabetes in mothers (years) among 262 patients was analyzed as n= 1-5 Years was 135(51.5%) and More than 6 Years was 127(48.5%). Mean duration was7.1±1.12 years. The distribution of Cyanotic Heart Disease among 262 patients was analyzed as n= Yes was 48(18.3%) and No was 214(81.7%). Practical implication: To determine the frequency of cyanotic heart diseases in children born of a diabetic mother, its prompt diagnosis and management to reduce neonatal morbidity and mortality. Conclusion: In this high-risk population useful assessment and early CHD diagnosis are strongly suggested, and echocardiography is advised as soon as feasible for all newborns of diabetes mothers. Prenatal CHD screening program for neonates of diabetes moms are required in our setup. Therefore, additional confirmation is anticipated in a study of a larger diabetic population. Keywords: Gestational diabetes mellitus, Congenital heart diseases, pre-gestational diabetes mellitus, Diabetic mothers

Access to Care and Therapy for Kawasaki Disease in the Arab Countries: A Kawasaki Disease Arab Initiative (Kawarabi) Multicenter Survey.

Kawasaki Disease (KD) is still the most common acquired heart disease in children below the age of five years; it has been well described in the developed world; however, data from the Arab world are limited to case reports or single-center case series. In an effort of optimizing KD research in the Arab world, a group of physicians and researchers established the KD Arab Initiative (Kawarabi) in 2021, and published the first survey, which showed disparities in the availability of intravenous immunoglobulin (IVIG); this had prompted Kawarabi to assess the access to care and therapy of KD patients in Arab countries. A 32 structured questions survey was conducted in thirteen Arab countries and addressed KD patients' access to healthcare in urban and rural settings. The survey results showed that access to care was uniform across large, mid-size cities and rural areas in 7/13 (54%) countries, while in 6/13 (46%) countries, it was in favor of large and mid-size cities over rural areas. The quality of medical services received by children with KD in large cities was rated as excellent in 6/13 or good in 7/13 countries compared to fair in 4/13 or poor in 4/13 countries in rural areas. Availability of IVIG was limited (23%) in mid-size cities and almost impossible (23%) in rural areas. The KD patients in mid-size cities and rural areas have limited access to standard healthcare in the Arab world. This survey laid the foundation for future Kawarabi endeavors to improve the care of children with KD.

Abstract 480: IL-33 Promotes Cardiovascular Lesions Development In A Mouse Model Of Kawasaki Disease

Kawasaki Disease (KD), a pediatric acute febrile illness and systemic vasculitis, is the leading cause of acquired heart diseases in children. Coronary artery aneurysms (CAAs) occur in up to 25% of untreated children, which is reduced to 5% with intravenous immunoglobulin (IVIG) treatment. However, up to 20% of KD patients are refractory to IVIG and at higher risk of CAAs, highlighting the need to characterize the mechanisms mediating KD vasculitis to develop more efficient therapies. IL-33 is released upon inflammation and tissue damage and exerts its effects by binding to its receptor ST2 ( IL1RL1 ). Levels of IL-33 are elevated in KD patients during the acute phase. However, how IL-33 contributes to the development of KD cardiovascular lesions remains unknown. Using the Lactobacillus casei cell wall extract (LCWE) murine model of KD vasculitis, we observed increased Il33 mRNA expression in hearts and abdominal aortas aneurysms of LCWE-injected mice. While ST2 was detected by immunofluorescence in the abdominal aortas of both control and LCWE-injected mice, IL-33 was mainly detected in tissues from LCWE-injected mice. Single-cell RNA-sequencing of abdominal aortas from LCWE-injected mice indicated that Il33 transcripts were expressed primarily by fibroblasts, endothelial cells, B cells, and vascular smooth muscle cells. In contrast, Il1rl1 was highly expressed by tissue infiltrating regulatory T cells, macrophages, eosinophils, and neutrophils. Blocking IL-33 with either anti-IL-33 or using Il33 -/- mice significantly attenuated LCWE-induced KD vasculitis. Our results indicate that IL-33 participates in LCWE-induced KD vasculitis in mice and could therapeutically be targeted to inhibit KD cardiovascular lesions.

IL-33 promotes cardiovascular lesions development in a mouse model of Kawasaki Disease

Abstract Kawasaki Disease (KD), a pediatric acute febrile illness and systemic vasculitis, is the leading cause of acquired heart diseases in children. Coronary artery aneurysms (CAAs) occur in up to 25% of untreated children, which is reduced to 5% with intravenous immunoglobulin (IVIG) treatment. However, up to 20% of KD patients are refractory to IVIG and at higher risk of CAAs, highlighting the need to characterize the mechanisms mediating KD vasculitis to develop more efficient therapies. IL-33 is released upon inflammation and tissue damage and exerts its effects by binding to its receptor ST2 (IL1RL1). Levels of IL-33 are elevated in KD patients during the acute phase. However, how IL-33 contributes to the development of KD cardiovascular lesions remains unknown. Using the Lactobacillus casei cell wall extract (LCWE) murine model of KD vasculitis, we observed increased Il33 mRNA expression in hearts and abdominal aortas aneurysms of LCWE-injected mice. While ST2 was detected by immunofluorescence in the abdominal aortas of both control and LCWE-injected mice, IL-33 was mainly detected in tissues from LCWE-injected mice. Single-cell RNA-sequencing of abdominal aortas from LCWE-injected mice indicated that Il33 transcripts were expressed primarily by fibroblasts, endothelial cells, B cells, and vascular smooth muscle cells. In contrast, Il1rl1 was highly expressed by tissue infiltrating regulatory T cells, macrophages, eosinophils, and neutrophils. Blocking IL-33 with either anti-IL-33 antibody or using Il33 −/−mice significantly attenuated LCWE-induced KD vasculitis. Our results indicate that IL-33 promotes LCWE-induced KD vasculitis in mice and could therapeutically be targeted to inhibit KD cardiovascular lesions.

Case of acute recurrent myocardial infarction in a child

Information on the essential aspects of prevalence, etiology and pathogenesis of coronary heart disease, which is the leading cause of death in the world, is given in this article. It is emphasized that the incidence of ischemic coronary events is the result of a complex sequence of pathogenetic links, which involves the formation of atherosclerotic lesions and destabilization as the primary provoking factor. Purpose - the attraction of the medical community attention to the problem of ischemic heart disease in children. A clinical case of recurrent myocardial infarction in a child who was examined and treated at the MNPE «Ivano-Frankivsk Regional Children’s Clinical Hospital of the Ivano-Frankivsk Regional Council», is described here. The key points of the patient’s complaints, anamnesis of life and this disease are described, the data of the objective condition at the hospitalization and during dynamic observation of the child, which showed the signs of left ventricular overload, coronary blood flow disorder, dilatation of the left heart ventricles, decreased left ventricular contractility. The data of the main indices of laboratory and instrumental methods of research, including X-ray diagnostic methods, performed not only on the basis of the department, but also in the Scientific-Practical Medical Center of Pediatric Cardiology and Cardiac Surgery of the Ministry of Health of Ukraine (Kyiv). The treatment of this clinical case in the child is described in this article. The data of dynamic observation are given. The discussion and conclusions emphasize the problem of coronary insufficiency in pediatrics, which requires clinical understanding, the choice of the correct diagnostic algorithm and qualified medical care. The study was performed in accordance with the principles of the Declaration of Helsinki. Informed consent of the child’s parents was obtained for the research conduction. No conflict of interests was declared by the authors.

Morphological analysis of ventricular septal defect by echocardiography for prediction of aortic regurgitation in pediatric population

Ventricular septal defects (VSD) are the most common congenital heart diseases in children. Among them, perimembranous VSD (pm-VSD) have a higher risk of complications, including aortic valve prolapse and aortic regurgitation (AR). The aim of our study was to assess echocardiographic criteria associated with AR during follow-up of pm-VSD. Forty children with restrictive pm-VSD, followed-up in our unit and who underwent a workable echocardiographic evaluation between 2015 and 2019 were included and retrospectively analyzed. The propensity score was used to match 15 patients with AR to 15 patients without AR. Median age was 2.2 year [1.4–5.7]. Median weight was 14 kg [9.9–20.3]. Aortic annulus z-score, Valsalva sinus z-score, sinotubular junction z-score, valve prolapse and commissure commitment were significantly different between the two groups (p = 0.047, p = 0.001, p = 0.010, p = 0.007, p < 0.001 respectively). Aortic root dilatation, aortic valve prolapse and commissure commitment to a perimembranous VSD are associated to aortic regurgitation.