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Bicuspid aortic valve (BAV) is the most common congenital heart disease with a prevalence of 0.5%–1.3% of the population. Many children with BAV are asymptomatic. Clinically relevant abnormal valve function usually occurs in adulthood. However, in rare cases, children can fail to thrive which requires valvular intervention. In this review, we will explore in more detail the anatomy of the BAV, clinical presentation of BAV, diagnosis of BAV, and its function by echocardiography, and indications for transcatheter intervention in the pediatric population. [ABSTRACT FROM AUTHOR]

Two newborn infants presented with acute respiratory distress. In both cases, the left lung was opaque, hyperinflated, and associated with a rightward shift of the mediastinum. A diagnosis of retained fetal fluid secondary to vascular compression of the left bronchus by the ductus arteriosus was made by combining various imaging methods including chest radiograph, computed tomography (CT), and echocardiography. Although the initial chest radiographs were similar, the mechanisms of obstruction were different. The imaging emphasizes the importance of CT angiography to understanding the three-dimensional relationships resulting in bronchial compression. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12–19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31–63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10–53 mmHg) pre-procedure to 10 mmHg (IQR 5–16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5–44 min). The large sizes—mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120–300 mL). Patients were followed for a median period of 360 days (IQR 164–525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes. [ABSTRACT FROM AUTHOR]

We describe the successful use of the Impella LP 5.0 intracardiac microaxial pump (Abiomed, Danvers, MA) in a 13-year-old boy with fulminant biopsy-proven viral myocarditis. The patient, who previously was in refractory cardiogenic shock despite increasing inotropic and vasopressor support, immediately stabilized after Impella LP 5.0 implantation and was successfully bridged to a full recovery. Months later, he remains completely well, with no intracardiac or peripheral vascular sequelae of the procedure. In carefully selected pediatric patients the Impella may be a beneficial form of temporary mechanical circulatory support for fulminant cardiogenic shock. [Copyright &y& Elsevier]

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 FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) is one of the most common forms of birth defects worldwide, with a prevalence of 1–2% in newborns. CHD is a multifactorial disease partially caused by genetic defects, including chromosomal abnormalities and single gene mutations. Here, we describe the Sidra Cardiac Registry, which includes 52 families and a total of 178 individuals, and investigate the genetic etiology of CHD in Qatar. We reviewed the results of genetic tests conducted in patients as part of their clinical evaluation, including chromosomal testing. We also performed whole exome sequencing (WES) to identify potential causative variants. Sixteen patients with CHD had chromosomal abnormalities that explained their complex CHD phenotype, including six patients with trisomy 21. Moreover, using exome analysis, we identified potential CHD variants in 24 patients, revealing 65 potential variants in 56 genes. Four variants were classified as pathogenic/likely pathogenic based on the American College of Medical Genetics and Genomics and the Association for Molecular Pathology (ACMG/AMP) classification; these variants were detected in four patients. This study sheds light on several potential genetic variants contributing to the development of CHD. Additional functional studies are needed to better understand the role of the identified variants in the pathogenesis of CHD. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

We sought to determine whether childhood obesity is associated with increased aortic stiffness by measuring the biophysical properties of the aorta in obese children using a noninvasive echocardiographic Doppler method. Increased aortic stiffness is a strong predictor of future cardiovascular events and mortality in adults. Obesity is known to be associated with increased aortic stiffness and arterial disease in adults. We prospectively evaluated a cohort of obese children (n = 61) and compared them to normal-weight controls (n = 55). The anthropometric data were recorded. The pulsewave velocity (PWV), aortic input impedance (Zi), characteristic impedance (Zc), arterial pressure-strain elastic modulus (Ep), arterial wall stiffness index (B index), and peak aortic velocity were calculated. We correlated our echocardiographic Doppler findings with the lipid levels. We assessed the left ventricular (LV) dimensions and standard measures of cardiac function. Cardiopulmonary exercise testing was performed on all obese children. Compared to normal-weight children, obese children had a greater PWV, Zc, B index, Ep, and peak aortic velocity. Obese children had greater systolic blood pressure than normal-weight children but no difference in diastolic blood pressure. The LV dimensions and standard measures of cardiac systolic function were similar in the 2 groups, but the obese children had altered diastolic properties. The LV mass was greater in the obese children. No association was found between the lipid levels and the biophysical properties of the aorta. The relative oxygen consumption was 68% predicted in obese children. In conclusion, measures of the biophysical properties of the aorta are already abnormal in obese children, reflecting increased aortic stiffness at this early stage of disease. Obese children also had an increased LV mass, altered diastolic properties, and an abnormal exercise capacity. PWV might be useful in monitoring the progression of arterial disease or the effect of therapeutic interventions. [ABSTRACT FROM AUTHOR]