Advancing Kawasaki Disease Research in the Arab World: Scoping Literature Review Analysis with Emphasis on Giant Coronary Aneurysms.

To evaluate giant aneurysms (GiAn) prevalence in Arab countries and examine contributing factors; and to review Kawasaki disease (KD) publication trends and collaborations among Arab nations. A scoping literature review was conducted to analyze the publications across the Arab world, spanning 16 countries from 1978 to 2023. The collected articles were a combination of database search with a call on Kawasaki Disease Arab Initiative (Kawarabi) members to share non-PubMed publications. Over 45 years, 50 articles originated from the Arab Countries with a 30% average annual growth rate in KD research output. Publications were evenly split between case reports (42%) and institutional series (52%). Research productivity lagged in developing nations with UAE, KSA and Egypt, contributed to 64% of total publications. Among 26 institutional series, 256 coronary artery aneurysms (CAA) from a total of 1264 KD cases were reported. Of those, 25 CAA were GiAn (prevalence 1.43% [range 0-12.5%]). The initial KD misdiagnosis rate was 4%, and incomplete KD (iKD) averaged 10.6%. Series (38.5%) that did not report iKD correlated with a higher prevalence of CAA, but not of GiAn. Longer fever duration emerged as a pivotal factor for GiAn (OR 5.06, 95%CI 1.51-17). This review unveils the research landscape of KD in the Arab world over 45 years. Initial misdiagnosis, untreated cases, delayed diagnosis and underreporting of iKD are contributing factors for an underestimated epidemiology, explaining the higher GiAn prevalence. This calls for strategic interventions to enhance KD research in these countries, aligning with Kawarabi's mission.

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.

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.

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.

Predictors of Neurological Complications of Pediatric Post-Cardiotomy Extracorporeal Life Support.

Background: Post-cardiotomy extracorporeal membrane oxygenation (ECMO) was associated with significant neurological complications affecting the overall outcome. The aim of the work is to determine the incidence and the predictors of neurological events during pediatric extracorporeal life support after cardiac surgery. Patients & Methods: This is a retrospective study that encompassed all neonates, infants, and children (<18 years of age) who need extracorporeal life support following cardiac surgery between January 2015 and December 2018 at San Donato Hospital, Italy. Data as regards surgical procedure of congenital heart disease, in-hospital mortality, length of ECMO, hospital stay durations, short-term neurological ECMO complications and outcome were analyzed. Results: The sixty-three patients who received post-cardiotomy ECMO, Neurological complications were evident in 31.7% in the form of ischemic stroke in 17.5% and hemorrhagic stroke in 11.1%. By multivariable analysis, the older age of cyanotic cases, the need for a venting cannula, and the rapid CO2 drop in the first 24 h were the most independent risk factors for neurological complications. Prolonged ECMO support and hospital stay duration were associated with neurological sequelae. Conclusion: Neurological complications either ischemic or hemorrhagic strokes were common during pediatric post-cardiotomy ECMO and were significantly related to prolonged ECMO support and hospital stay. Predictors of these neurological sequelae are the older cyanotic cases, the need for a venting cannula, the oxygenator thrombosis, and the rapid CO2 drop in the first 24 h of ECMO.

Early onset left ventricular remodeling in juvenile systemic lupus erythematosus; Insight from 3-dimensional speckle tracking echocardiography.

BACKGROUND: Early diagnosis and treatment of myocardial affection in patients with systemic lupus erythematosus (SLE) are crucial. OBJECTIVES: To evaluate the ventricular systolic function in juvenile-onset systemic lupus erythematosus (j-SLE) patients by 3-D speckle tracking echocardiography (3D-STE) and to determine the predictors of left ventricular (LV) dysfunction if present. METHODS: Twenty-six SLE patients without heart failure and 21 healthy controls were studied by standard echocardiogram and 3D-STE. Conventional parameters included LV ejection fraction (EF), fractional shortening (FS), and mitral annular plane systolic excursion (MAPSE). Global LV strain (GLS) and global area strain (GAS) were obtained by 3D-STE. Medical records, including diagnosis criteria, duration of disease, and SLE disease activity index (SLEDAI) were evaluated. RESULTS: The mean age was similar in patients and controls 11.42 vs 11.48 years p = 0.93. The mean duration of the disease was 1.87 ± 1.02 years and SLEDAI ranged from 0 to 9. By conventional and tissue Doppler imaging echocardiography, only MAPSE was significantly lower in SLE patients compared to controls (14.56 vs 18.46 mm, p < 0.001). By 3D speckle tracking echocardiography, GLS and GAS were significantly reduced in SLE patients compared to controls (-15.07 vs -19.9.4%, -34.6% vs -39.7%, respectively, p < 0.001). Multiple linear regression and ROC analyses indicated that the SLEDAI score was the only predictive factor for the left ventricular remodeling. CONCLUSIONS: These results indicate that early subclinical LV dysfunction occur in jSLE patients even with normal EF and SLE disease activity might be a potential driver for LV deformation.

Left and right ventricular speckle tracking study before and after percutaneous atrial septal defect closure in children.

OBJECTIVES: To analyze the acute and short-term deformation changes of both right (RV) and left (LV) ventricular wall before and after transcatheter closure of atrial septal defect (ASD) secundum in children. OUTCOME MEASURES: To determine the feasibility of tissue Doppler and myocardial deformation imaging for evaluating RV and LV functions in children undergoing transcatheter ASD closure. PATIENTS AND METHODS: A prospective study was performed for 32 children with hemodynamic significant ASD secundum before and 6 months after percutaneous ASD closure in the Pediatric Cardiology Division of Specialized Pediatric Hospital, Cairo University. Speckle tracking echocardiography (STE) of LV and RV global analysis (longitudinal and circumferential strain) before and after ASD transcatheter closure was performed. RESULTS: The mean age of the patients was 6.01 ± 3.19 (range: 3-9) years with a female to male ratio of 1.3:1. There was an improvement in the RV and LV myocardial performance index (MPI) 6 months post-ASD closure (RVMPI = 0.46 ± 0.069 vs. 0.38 ± 0.05, p < 0.0001; LVMPI = 0.49 ± 0.12 vs. 0.38 ± 0.08, p < 0.0001, respectively). By 2D STE, there was a significant improvement in the RV global longitudinal strain (GLS) 6 months post-ASD closure (-20.17 ± 3.14% vs. -25.86 ± 5.02%, p < 0.0001). There was a significant increase in the LV end-diastolic volume (EDV) and LV end-systolic volume (ESV) using 4D STE after device closure (LVEDV = 32.96 ± 10.99 mL vs. 44.024 ± 14.9017 mL, p < 0.0001; LVESV = 15.16 ± 6.08 mL vs. 21.76 ± 8.34 mL, p < 0.0001, respectively). Additionally, there was a significant improvement in the LV GLS after device occlusion (-19.17 ± 3.67% vs. -22.36 ± 4.72%, p = 0.009) using 4D TomTec software. There was a significant decrease in the RVEDV (54.65 ± 10.05 mL vs. 15.73 ± 8.67 mL) and RV stroke volume (25.15 ± 6.36 vs. 20.06 ± 7.2) after device occlusion using 4D TomTec software. CONCLUSION: By using 4D STE, the LV GLS was significantly improved; in contrast, by 2D STE, the RV volume overload decreased and the RV GLS was improved on short term after transcatheter ASD secundum closure in children.

Left ventricular rotational deformation changes by speckle tracking imaging before and 24 hours after transcatheter closure of large secundum atrial septal defects in children.

BACKGROUND: Large atrial septal defects (ASDs) in children cause increased volume overload of the right side of the heart which in turn lead to impairment of left ventricular (LV) performance. AIM: The aim of this study was to evaluate immediate LV rotational deformation changes in children with large ASDs post-device closure and removal of right ventricle (RV) volume overload. PATIENTS AND METHODS: Twenty children who underwent transcatheter closure (TCC) of large secundum ASDs were included in the study. LV rotational deformation was assessed pre- and 24 hours post-device closure using speckle tracking imaging (STI). RESULTS: 55% were females with mean age 6.1 ± 3.5 years. LV peak basal clockwise rotation improved significantly (-6.9 ± 2.6° before vs -10.3 ± 4.1° after TCC, P = .005), and time to peak clockwise rotation (345.1 ± 124.7 milliseconds (ms) before vs 282.2 ± 82.9 ms after closure, P = .02). There was no significant difference in apical rotational parameters including peak counterclockwise rotation (P > .05 for both). LV twist (11.3 ± 3.8° before vs 17.5 ± 7.1° after closure, P = .001) and torsion (2.1 ± 0.7°/cm before vs 3.1 ± 1.2°/cm after closure, P = .01) were significantly improved, mainly as the result of improvement of LV basal rotation. LV revealed a significant increase in LV end-diastolic volumes (P = .02) 24 hour after TCC with no significant change (P > .05) in end-systolic volumes after closure. CONCLUSION: Increased peak LV twisting and torsion were attributed to the improved peak systolic clockwise basal rotation after TCC of large ASDs in children.

Experience with balloon pulmonary valvuloplasty and predictors of outcome: a ten-year study.

BACKGROUND: Balloon pulmonary valvuloplasty is the treatment of choice for patients with moderate to severe pulmonary valve stenosis. METHODS: An observational retrospective cross-sectional study including neonates, small infants, and children who underwent balloon pulmonary valvuloplasty in the period from 2007 to 2016 in the cardiac catheterisation unit of the paediatric cardiology department in Cairo University. Multivariable models were built to report the predictors of the outcome of balloon pulmonary valvuloplasty and its complications. RESULTS: A total of 1200 patients were included in the study and divided according to age into 3 groups: neonates and early infants (n = 282), infants (n = 362), and children (n = 556). Procedural success, defined as a drop pressure gradient across the pulmonary valve to less than or equal to 50% of the baseline measurements, was achieved in 82.7% of the patients. Multivariate analysis revealed that only infundibular pulmonary stenosis (p value 0.032), supravalvular in association with valvular pulmonary stenosis (p value <0.001), and pulmonary valve diameter by angiogram (p value <0.001) were significant predictors of success. The presence of supravalvular in association with valvular pulmonary stenosis (p value <0.001) was associated with a lower weight (p value 0.007) and higher right ventricular pressure before the intervention (p value <0.001), and a minor immediate drop in the pressure gradient post-intervention (p value <0.001) was found to be the most significant predictor of the occurrence of complications. CONCLUSION: The absence of infundibular and supravalvular stenosis and a large pulmonary valve diameter were the most significant predictors of success.

ASD device closure in pediatrics: 3-Dimensional transthoracic echocardiography perspective.

OBJECTIVE: Real-time three-dimensional echocardiography, using both reconstruction methods and RT3D, has been used as an extra helping tool in several forms of congenital heart diseases. Our aim was to understand the relation of the ASD device to all surrounding structures by 3-dimensional echocardiography (3D). METHODS: This prospective study included 37 patients diagnosed as ASD secundum by transthoracic (TTE) and transesophageal echocardiography (TEE) referred for transcatheter closure from October 2013 to July 2016. Follow-up for 1 year using 2D and 3D-echocardiography was performed to assess the relations of the device to the surrounding structures. RESULTS: Transcatheter ASD closure and echocardiographic examinations were successfully performed for all patients. By 3D echocardiography, 16 patients (43.24%) had their ASD device close to the aortico-mitral continuity plane without apparent regurgitation, while the rest of our patients (56.75%) the devices were away from this plane. The following variables were significantly different between the two groups; body surface area, atrioventricular rim (AV), device size, left disc size and ratio of left disc to interatrial septum. A cut-off AV rim length not less than 8 mm was found optimal to avoid device encroachment on the sensitive surrounding structures. New Formula was constructed to aid in device choice. CONCLUSION: Use of 3D before and after ASD closure is of value to determine the device relation to the surrounding structures. AV rim by TEE is an important rim to avoid eventual encroachment on the mitral valve and aorta.

Acute Kawasaki disease with emphasis on the echocardiographic profile: A single center experience.

Background: Echocardiographic features of acute Kawasaki disease (KD) have not been well characterized in Egyptian children. This study aimed to provide insight into the pattern of cardiac involvement in Egyptian children with Kawasaki disease, focusing on echocardiographic coronary abnormalities and their associated risk predictors. Methods and Results: Medical records of 64 KD patients from 2012 to 2016 were retrospectively analyzed with recalculation of coronary artery z-scores during the first eight weeks after fever onset. All patients received intravenous immunoglobulin (IVIG) and 57.8% were treated within 10 days of illness onset. Coronary abnormalities were found in 53.1% of all patients, and in 43.2% of those who received IVIG within 10 days. Giant aneurysms (z-score>10) comprised 23.5% of all coronary abnormalities. Coronary thrombosis occurred in two patients (5%), both of whom developed myocardial infarction, and one succumbed to heart failure with eventual in-hospital death. Overall, 7% of patients had mitral regurgitation (n = 5), 1.5% had aortic regurgitation (n = 1), and 7.8% had pericardial effusion (n = 5). Among a number of laboratory and clinical predictors, platelet count had the strongest association with coronary abnormalities (Area under Receiver-operating characteristic (ROC) curve: 0.794; 95% confidence interval 0.678-0.910; P < 0.001). Conclusion: Coronary abnormalities occur in a substantial percentage of KD in Egypt, with associated evidence of severe inflammation. Further efforts are required to increase awareness of the disease and to emphasize the importance of early IVIG administration. Future studies should also be undertaken to characterize the long term progression profile of the disease as well as the possible genetic background of the disease in Egypt.

Incomplete RV Remodeling After Transcatheter ASD Closure in Pediatric Age.

Published data showing the intermediate effect of transcatheter device closure of atrial septal defect (ASD) in the pediatric age-group are scarce. The objective of the study was to assess the effects of transcatheter ASD closure on right and left ventricular functions by tissue Doppler imaging (TDI). The study included 37 consecutive patients diagnosed as ASD secundum by transthoracic echocardiography and TEE and referred for transcatheter closure at Cairo University Specialized Pediatric Hospital, Egypt, from October 2010 to July 2013. Thirty-seven age- and sex-matched controls were selected. TDI was obtained using the pulsed Doppler mode, interrogating the right cardiac border (the tricuspid annulus) and lateral mitral annulus, and myocardial performance index (MPI) was calculated at 1-, 3-, 6- and 12-month post-device closure. Transcatheter closure of ASD and echocardiographic examinations were successfully performed in all patients. There were no significant differences between two groups as regards the age, gender, weight or BSA. TDI showed that patients with ASD had significantly prolonged isovolumetric contraction, relaxation time and MPI compared with control group. Decreased tissue Doppler velocities of RV and LV began at one-month post-closure compared with the controls. Improvement in RVMPI and LVMPI began at 1-month post-closure, but they are still prolonged till 1 year. Reverse remodeling of right and left ventricles began 1 month after transcatheter ASD closure, but did not completely normalize even after 1 year of follow-up by tissue Doppler imaging.