Plasma Neutrophil Gelatinase-Associated Lipocalin (2 - 0) Index: A Precise and Sensitive Predictor of Acute Kidney Injury in Children Undergoing Cardiopulmonary Bypass.

OBJECTIVES: Children undergoing cardiopulmonary bypass (CPB) are at risk of acute kidney injury (AKI). Rise in serum creatinine occurs slowly and late, hence other markers are crucially needed to recognize AKI early. The aim of the study is to detect the efficacy of Neutrophil gelatinase-associated lipocalin (NGAL) in the early prediction of AKI. METHODS: A prospective observational study with enrollment of 174 patients, aged 6 months to 60 months, with congenital heart disease, undergoing CPB. Plasma NGAL measurement preoperatively and serially at 2, 12, 24, 36, and 48 hours post-CPB initiation. Patients were classified into 2 groups according to the development of postoperative AKI. RESULTS: Plasma NGAL levels post-CPB were significantly higher in the AKI group compared to the non AKI group with positive significant correlations between plasma NGAL level and severity of AKI. A rise in plasma NGAL of 500% from its preoperative basal level, when measured at 2 hours post-CPB initiation (NGAL 2-0 index), shows sensitivity and specificity of 83% and 64% respectively (AUC=0.667) and at 12 hours post-CPB initiation (NGAL 12-0 index) shows sensitivity and specificity of 66% and 64% respectively (AUC=0.762). CONCLUSIONS: Plasma NGAL is a predictive biomarker for acute kidney injury after pediatric cardiac surgery, and it may permit earlier intervention that improves the outcome of AKI. A 500% rise in plasma NGAL at 2 hours post-CPB initiation from its basal preoperative level (NGAL 2-0 index) is a precise, sensitive, and early predictor of AKI in children.

Kawarabi: Administrative Structuring of a Multicenter Research Collaborative to Study Kawasaki Disease in the Arab Countries.

Kawasaki disease (KD), the leading cause of acquired heart disease in children in developed countries, merits conducting detailed studies in Arab countries. We introduce Kawarabi, as a multicenter research collaborative effort dedicated to improving diagnosis, care, and outcome of children and adults with KD in the Arab world. During the COVID-19 pandemic, there emerged a new multisystem inflammatory syndrome in children; a disease similar to KD. This highlighted the challenges that Arab physicians face in diagnosing and managing children with KD and KD-like illnesses. Kawarabi brings together experts in North America and Arab nations to study this family of diseases in a not-for-profit, voluntary scientific collaborative setting. Bylaws addressing the vision, objectives, structure, and governance of Kawarabi were established, and vetted by the 45 organizing members in 2021. An initial scientific publication showed evidence of a decreased level of awareness of the disease in the general population, as well as the lack of access to resources available for physicians caring for children with KD in Arab countries. Kawarabi has since held several educational webinars and an inaugural yearly meeting. The groundwork for future initiatives targeted at increasing awareness and understanding of the management and the long-term outcomes of children with KD in the region was established. Data on KD in the Arab world are lacking. Kawarabi is a multicenter research collaborative organization that has the unique resources, diversified ethnic makeup, and energy, to accomplish significant advances in our understanding and management of KD and its variants.

Long-term outcomes of percutaneous closure of ventricular septal defects in children using different devices: A single centre experience from Egypt.

BACKGROUND: The feasibility of percutaneous closure ventricular septal defects (VSD) in children has been previously proven. However, data on long-term outcomes are limited. We aim to evaluate the long-term outcome of our experience with percutaneous closure of VSD using various occluders. METHODS: Retrospective institutional analysis of children who underwent transcatheter closure of perimembranous and muscular VSDs between September 2012 and February 2020. Patient demographics, procedural, and long-term follow-up data were comprehensively analyzed. Patients who lost to follow-up within two years post-procedure were excluded. RESULTS: We identified 75 patients (54.7% males) with a median of 66 months (IQR, 46-96). The closure success rate at one year was 95.7%. Complete heart block was detected in two patients early post-procedure and resolved with steroids. The VSDs were perimembranous (52%), muscular (33.33%), and residual (14.67%). Implanted devices were Pfm Nit-Occlud LeˆVSD Coil (42.7%), HyperionTM VSD Muscular Occluder (28%), Amplatzer VSD muscular occluder (10.7%), Amplatzer Duct Occluder (14.7%), Occlutech Muscular VSD Occluder (2.7%), and Amplatzer Duct Occluder II (1.3%). No new arrhythmia or valve regurgitation was detected after two years post-procedure. Persisted complications on long-term follow-up included: residual shunting in 3(4%), mild tricuspid regurgitation in 2(2.7%), and aortic regurgitation in 2(2.7%), with one immediate post-catheterization mild aortic regurgitation worsened during follow-up, requiring surgical repair of VSD three years after device implantation. No deaths were reported. CONCLUSION: Long-term outcomes of pediatric transcatheter VSD closure using different devices are satisfactory. Post-procedural adverse events are limited, but long-term surveillance is necessary to monitor their progression.

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.

A deeper look into the functions of right ventricle using three-dimensional echocardiography: the forgotten ventricle in children with systemic lupus erythematosus.

Studies on the right ventricular dysfunction (RV) in systemic lupus erythematosus (SLE) patients are limited, particularly in the pediatric age group. The study aimed to identify subclinical RV alterations in childhood-onset SLE (c-SLE) using conventional and three-dimensional echocardiography (3DE). Forty SLE pediatric patients and 40 healthy controls were included. Disease activity and chronicity were evaluated by SLE disease activity index (SLEDAI) score and SLE damage index (SDI). Participants underwent detailed RV echocardiographic examination with conventional and 3DE assessment using 3D auto RV software. Patients included 35/40 (87.5%) females with mean age of 15.6 ± 1.7 years. Using conventional pulmonary artery systolic pressure echocardiography-derived measurement, none of the c-SLE patients had pulmonary hypertension. By 3DE, RV end-systolic and end-diastolic volumes (p = < 0.001, 0.02, respectively) were greater, whereas 3D-derived RV ejection fraction (p < 0.001), septal, and lateral longitudinal strain (both p < 0.001) were lower in SLE. SDI displayed a significant correlation with 3D auto RV ejection fraction (EF), tricuspid annular plane systolic excursion (TAPSE), fractional area change, and RV longitudinal strain (RVLS)-free wall (p = 0.01, 0.003, 0.007, and < 0.001, respectively). Cumulative SLEDAI score also showed a significant correlation with RV EF, TAPSE, FAC, and RVLS-free wall (p = 0.03, 0.007, 0.002, and < 0.001, respectively). By multivariate regression analysis, SDI remained an independent predictor of RVLS-free wall (ß coefficient - 0.4, p = 0.03) and TAPSE (ß - 0.5, p = 0.02).  Conclusion: Subtle right ventricular myocardial dysfunction could be detected in childhood-onset SLE patients, especially via 3D-derived auto RV echocardiographic parameters, despite the absence of evident pulmonary hypertension. These parameters correlate with the SLE disease activity and chronicity scores. What is Known: •Diseases of the cardiovascular system are one of the most common causes of morbidity and mortality in SLE patients. •RV labeled the forgotten ventricle in many diseases, was also forgotten in SLE patients and has been rarely addressed in adults, with scarce research in pediatrics. What is New: •Right ventricular functions are affected in children with SLE in comparison to healthy controls, especially three-dimensional echocardiography-derived parameters, which is an aspect that has not been investigated in previous research in the pediatric age group. •Some of the detected myocardial dysfunctions of the right ventricle correlated with SLE disease activity and chronicity-related scores.

Left Ventricular Pseudoaneurysm: Unexpected Sequel of Tamponade-Complicated Infectious Pericarditis in Infancy.

Left ventricular pseudoaneurysm (LV-PsA) is a critical finding that could result in a fatal outcome. It may complicate myocardial infarction, cardiac surgery, trauma, or endocarditis but rarely follows pericarditis. We report a case of infectious pericarditis complicated by pericardial tamponade in an infant. After effusion drainage and medical therapy, a large LV-PsA was detected. Successful closure of the pseudoaneurysmá¾½s neck was accomplished using a Gore-tex patch.

Kawasaki Disease Arab Initiative [Kawarabi]: Establishment and Results of a Multicenter Survey.

Studies on Kawasaki disease (KD) in Arab countries are scarce, often providing incomplete data. This along with the benefits of multicenter research collaboratives led to the creation of the KD Arab Initiative [Kawarabi] consortium. An anonymous survey was completed among potential collaborative Arab medical institutions to assess burden of KD in those countries and resources available to physicians. An online 32-item survey was distributed to participating institutions after conducting face validity. One survey per institution was collected. Nineteen physicians from 12 countries completed the survey representing 19 out of 20 institutions (response rate of 95%). Fifteen (79%) institutions referred to the 2017 American Heart Association guidelines when managing a patient with KD. Intravenous immunoglobulin (IVIG) is not readily available at 2 institutions (11%) yet available in the country. In one center (5%), IVIG is imported on-demand. The knowledge and awareness among countries' general population was graded (0 to 10) at median/interquartiles (IQR) 3 (2-5) and at median/IQR 7 (6-8) in the medical community outside their institution. Practice variations in KD management and treatment across Arab countries require solid proactive collaboration. The low awareness and knowledge estimates about KD among the general population contrasted with a high level among the medical community. The Kawarabi collaborative will offer a platform to assess disease burden of KD, among Arab population, decrease practice variation and foster population-based knowledge.

Evaluation of the left ventricular systolic strain in hypocalcemic infants with two-dimensional speckle tracking echocardiography.

INTRODUCTION: Speckle tracking echocardiography (STE) is a new emerging method for evaluation of the cardiac systolic performance. We characterized left ventricular (LV) systolic functions of hypocalcemic infants, and assessed the effects of calcium and vitamin D supplementations on LV systolic functions using two-dimensional STE. PATIENTS AND METHODS: A prospective controlled study was conducted in Mansoura University Children's Hospital, Egypt from 2015 to 2018 including 88 hypocalcemic infants (patient group) and 30 healthy controls. We subdivided the patient group into vitamin D deficiency group (n = 32) and normal vitamin D group (n = 56). All infants were investigated for serum phosphorus, alkaline phosphatase and 25-hydroxy vitamin D levels. Both patients and controls were initially evaluated for LV systolic functions using two-dimensional STE. After correction of hypocalcemia and vitamin D deficiency, reevaluation of LV systolic functions was done for the patient group. RESULTS: LV systolic strains were lower in the patient group than controls (p < .001). After recovery of hypocalcemia of the patients, we reported significant improvement of strains and significant reductions of the end-diastolic and end-systolic volumes of the left ventricle (p < .001). Global longitudinal and circumferential strains were lower in patients with vitamin D deficiency than patients with normal vitamin D levels (p < .001). The LV systolic strain improved after correction of vitamin D deficiency and hypocalcemia (p < .001). CONCLUSION: Two-dimensional STE could detect and follow up early LV systolic dysfunction in infants with hypocalcemia and vitamin D deficiency.

The relevance of left ventricular functions to clinical and metabolic characteristics of prepubertal children with obesity.

BACKGROUND: Paediatric obesity is a worldwide health burden, with growing evidence linking obesity to myocardial function impairments. The study aims to evaluate left ventricular functions among prepubertal obese children to obesity-related clinical and metabolic parameters. METHODS: Between June 2019 and March 2020, 40 prepubertal children with obesity were recruited and compared to 40 healthy controls. Patients were assessed for body mass index z scores, waist circumference, body adiposity by bioimpedance analysis, and obesity-related laboratory tests, for example, serum chemerin. Left ventricular functions were assessed using variable echocardiographic modalities, such as M-mode, tissue Doppler, and two-dimensional speckle tracking. RESULTS: Mean patients' age was 9.25 ± 1.05 years. Left ventricular mass index, E/E', and myocardial performance index were significantly increased in obese children than controls. Although M-mode-derived ejection fraction was comparable in both groups, two-dimensional speckle tracking-derived ejection fraction, global longitudinal strain, and global circumferential strain were significantly lower in cases than controls. Left ventricular mass index displayed a positive correlation with body mass index z score (p = 0.003), fat mass index (p = 0.037), and trunk fat mass (p = 0.021). Global longitudinal strain was negatively correlated with body mass index z score (p = 0.015) and fat mass index (p = 0.016). Serum chemerin was positively correlated with myocardial performance index (p = 0.01). CONCLUSION: Alterations of left ventricular myocardial functions in prepubertal obese children could be detected using different echocardiographic modalities. Chemerin, body mass index z score, fat mass index, and trunk fat mass were correlated with subclinical left ventricular myocardial dysfunction parameters before puberty. Our results reinforce early and strict management of childhood obesity upon detection of changes in anthropometric and body adiposity indices.

Cardiac implications of multisystem inflammatory syndrome associated with COVID-19 in children under the age of 5 years.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is responsible for significant lung disease in adults. Despite mild manifestations in most children, multisystem inflammatory syndrome (MIS-C) associated with COVID-19 is well described in older children with cardiac manifestations. However, MIS-C-related cardiac manifestations are not as well described in younger children. METHODS: The study is a retrospective analysis of MIS-C patients under the age of 5 years admitted between May and November 2020 to a single centre. Included cases fulfilled the case definition of MIS-C according to Royal College of Pediatrics and Child Health criteria with laboratory, electrocardiogram, or echocardiographic evidence of cardiac disease. Collected data included patients' demographics, laboratory results, echocardiographic findings, management, and outcomes. RESULTS: Out of 16 MIS-C cases under 5 years of age, 10 (62.5%) had cardiac manifestations with a median age of 12 months, 9 (90%) were previously healthy. Cardiac manifestations included coronary arterial aneurysms or ectasia in five (50%) cases, two (20%) with isolated myopericarditis, coronary ectasia with myocarditis in two (20%), and supraventricular tachycardia in one (10%). Intravenous immunoglobulins were given in all cases with coronary involvement or myocarditis. The median duration of hospitalisation was 7 (6-14) days; two (20%) cases with cardiac disease were mechanically ventilated and mortality in MIS-C cases below 5 years was 12.5%. Normalisation of systolic function occurred in half of the affected cases within 1 week and reached 100% by 30 days of follow-up. CONCLUSIONS: MIS-C associated with SARS-CoV-2 has a high possibility of serious associated cardiac manifestations in children under the age of 5 years with mortality and/or long-term morbidities such as coronary aneurysms even in previously healthy children.

Evaluation of carotid artery access in comparison with femoral artery access in neonatal percutaneous stenting of ductus arteriosus.

BACKGROUND: Patent ductus arteriosus stenting in duct-dependent pulmonary circulation is a challenging procedure. Percutaneous carotid artery access for ductal stenting has proven to be feasible; however, comparison with femoral artery access in terms of procedure details and complications either immediate or late is scarce. Therefore, we evaluated carotid artery access in comparison with femoral artery for stenting of patent ductus arteriosus. METHODS: Forty neonates were reviewed, 20 were stented via carotid artery access, and 20 via the traditional femoral artery access. Comparison variables were neonatal demographics at the procedure, angiographic ductal anatomy, procedure details, and immediate complications. Follow-up Doppler ultrasound on access site was performed to document late complications. RESULTS: Median age of included cases was 10.5 (3-28) days with complex ductal anatomy more frequently accessed via carotid artery than femoral. Immediate access-related complications were significantly higher with femoral than carotid artery access; 9 (45%) versus 3 (15%) respectively, p = 0.038. With carotid access, we had only one case with small pseudoaneurysm and acute hemiparesis 3 days after the procedure. Delayed local complications were more common with femoral access (15%) than carotid access (5%), mild stenosis in one case, and severe in another with femoral access; while with transcarotid arterial access, only one case had mild narrowing. CONCLUSION: Percutaneous carotid artery access in neonates is a more convenient approach for patent ductus arteriosus stenting especially with complex ductal anatomy. Moreover, local complications are limited and vascular patency is better preserved, in comparison with trans-femoral arterial access. However, the potential for neurological adverse events should not be overlooked.

Evaluation of the Growth of Central Pulmonary Arteries Following Patent Ductus Arteriosus Stenting in Patients with Duct Dependent Pulmonary Circulation.

Pulmonary arteries' (PAs) growth can be promoted by stenting of patent ductus arteriosus (PDA). This may result in better angle between the PDA and the PAs, allowing improved growth. In this study, we sought to evaluate the effect of PDA stenting on the growth of the pulmonary arteries by comparing their dimensions pre-stenting to their dimensions in the pre-second stage operations in patients with congenital heart diseases-duct-dependent pulmonary (CHD-DDP) circulation. Between January 2015 and December 2016, 58 neonates with CHD-DDP circulation underwent transcatheter PDA stenting and had evaluation of PAs growth before the second stage. Various parameters [Pre-branching right and left pulmonary artery (RPA, LPA) diameters, their Z scores, LPA/RPA ratio, McGoon's ratio and Nakata index] were recorded and compared pre-stenting and pre-second stage. The evaluation was done using catheterization or multislice computed tomography (MSCT). PDA stenting was successful in 49 patients out of 58 (84.5%) patients with an age of 13.5 ± 10.4 days and a weight of 2.9 ± 0.5 kg. Twenty-two (44.9%) patients had complex CHD-DDP, 14 (28.6%) patients had PA/IVS and 13 (26.5%) patients had PA/VSD. Pre-second stage RPA, LPA diameters and their Z scores increased significantly (RPA increased from 0.36 ± 0.05 cm to 0.60 ± 0.11 cm, P < 0.001, RPA Z-score increased from - 1.29 ± 0.91 to 0.81 ± 0.18, P < 0.001; LPA increased from 0.34 ± 0.06 cm to 0.58 ± 0.10 cm, P < 0.001, LPA Z-score increased from - 1.17 ± 0.86 to 0.97 ± 0.48, P < 0.001). McGoon's ratio increased significantly from 1.20 ± 0.11 to 1.61 ± 0.15 (P < 0.001). Nakata index increased from 105.94 ± 33.53 to 183.48 ± 40.58 mm2/m2 (P < 0.001). However, LPA/RPA ratio did not change (0.96 ± 0.05 and 0.98 ± 0.16, P = 0.288). PDA stenting is effective in promoting the global and the individual pulmonary artery growth in congenital heart diseases with duct-dependent pulmonary circulation. In this study, we presented our experience with this approach in 2 tertiary care centers in the DELTA region of Egypt. PDA stenting, generally, showed symmetric growth of the pulmonary arteries with comparable results to the international figures.

Assessment of Myocardial Function in Children before and after Autologous Peripheral Blood Stem Cell Transplantation.

BACKGROUND: Increased interest is focused on the long-term adverse effects of bone marrow transplantation. Subclinical cardiac involvement appears common in adults, but only a few reports have examined pediatric patients. MATERIALS AND METHODS: A prospective case-control study of 19 children with normal cardiac function undergoing autologous hematopoietic stem cell transplantation (HSCT) was performed. Tissue Doppler imaging (TDI) and echocardiographic measurements were obtained according to the guidelines of the American Society of Echocardiography before and 3 months after HSCT. RESULTS: Lateral mitral annulus before HSCT showed significant reduced mitral systolic annular velocity (P < 0.0001), early diastolic annular velocity (P < 0.0001), late diastolic annular velocity (P = 0.02) and prolonged isovolumetric relaxation time (IRT) (P < 0.0001) compared with control. Significant reduced mitral systolic annular velocity (P < 0.0001), early diastolic annular velocity (P = 0.0005) and Em/Am ratio (P = 0.004), with higher late diastolic annular velocity (P = 0.02) and prolonged isovolumetric contraction time (ICT) (P = 0.003) and IRT (P = 0.002) after HSCT, were observed. Investigation of lateral tricuspid annulus showed nearly similar results as the lateral mitral annulus. LV and RV Tei indices were higher before HSCT compared with control and remained high after HSCT. CONCLUSION: TDI detected subtle abnormalities in systolic and diastolic functions before and after HSCT, which suggests that a conditioning regimen may affect cardiac function.