Long-Term Results of Percutaneous Balloon Aortic Valvuloplasty in Children With Aortic Stenosis: A Single-Center Experience.

Purpose: Congenital aortic stenosis is a common pathology in the childhood age group and its clinical spectrum varies between asymptomatic and severe heart failure. In our study, we planned to evaluate the long-term results of patients who underwent balloon aortic valvuloplasty (BAV) due to critical aortic valve stenosis in our clinic.

Materials And Methods:  Patients aged 0-18 years who underwent aortic balloon valvuloplasty due to aortic stenosis in our clinic between January 2002 and January 2022 were retrospectively evaluated.

Transcatheter Closure of Secundum Atrial Septal Defects in Pediatric Patients: A 15-Year Single-Center Experience.

Purpose: Transcatheter closure is the first-line treatment option for hemodynamically significant secundum atrial septal defects (ASDs). This study examines our center's experience with this procedure over the last 15 years.

Materials And Methods: Pediatric patients aged 0-18 years with secundum ASDs who were planned for transcatheter closure in our clinic between January 2007 and January 2023 were retrospectively evaluated.

A case-control study of early-stage radiological markers of endothelial dysfunction and cardiovascular findings in patients with osteogenesis imperfecta: genotype-phenotype correlations.

Objectives: Osteogenesis imperfecta (OI) is a disease caused by defective collagen synthesis. Collagen type 1 is found in many structures in the cardiovascular system. Endothelial dysfunction, which develops prior to the emergence of structural and clinical signs of atherosclerosis, is believed to play a key role in atherogenesis.

Hypertrophic cardiomyopathy after Takeuchi procedure for anomalous origin of the left coronary artery from the pulmonary artery syndrome.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare condition. Surgical intervention is indicated in all diagnosed patients. Successful repair is expected to correct left ventricle (LV) functions and heart failure, reduce mitral regurgitation, and resolve infarct patterns observed on electrocardiogram.

Electrocardiographic changes in hospitalised children with COVID-19.

Objectives: Cardiac manifestations of the coronavirus disease 2019 (COVID-19) have mainly been reported in adults. Therefore, we aimed to determine the electrocardiographic abnormalities in hospitalised paediatric patients with COVID-19 and multisystemic inflammatory syndrome in children.

Methods: We retrospectively evaluated hospitalised paediatric patients <18 years of age with a diagnosis of COVID-19 (n = 168) and multisystem inflammatory syndrome in children (n = 48) between March 2021 and December 2021.

Cardiac Assessment in Children with MIS-C: Late Magnetic Resonance Imaging Features.

Multisystem Inflammatory Syndrome (MIS-C) is a new entity that emerges 2-4 weeks after the SARS-CoV-2 infection in children. MIS-C can affect all systems, the most severe of which is cardiac involvement. The duration of the cardiac symptoms is still uncertain and may be persistent or prolonged.

Neurological Recovery with Interferon-gamma Treatment in Friedreich's Ataxia.

Friedreich's ataxia (FA) is a rare, progressive, and degenerative hereditary disorder caused by a deficiency of frataxin protein. This disease is characterised by severe neurological dysfunction and life-threatening cardiomyopathy. Various drugs are used to slow down / stop the neurodegenerative progress.

Low-density lipoprotein and blood pressure are important risk factors for vasculopathy in children on dialysis: A single center study.

Introduction: We searched for risk factors of cardiovascular assessment among children on dialysis.

Methods: This is a cross-sectional study of cardiovascular assessment of all patients on dialysis at Ege University Children's Hospital. Pediatric patients between the ages of 6 and 21 who were on HD and peritoneal dialysis treatment were included in the study.

Rare coexistence of Tay-Sachs disease, coarctation of the aorta and grade V vesicoureteral reflux.

Tay-Sachs disease is a neurodegenerative inherited metabolic disease. There are four forms classified by the time of first clinical symptoms: infantile, late infantile, juvenile and adult. Infantile , Ebru Candab, Ertürk Leventc , The infantile form has the poorest clinical prognosis.

Periodic fever syndromes: a patient diagnosed with recurrent Kawasaki disease.

Kawasaki disease, known as mucocutaneous lymph node syndrome, is a multi-system disease of unknown aetiology that occurs in young children under 5 years of age. The recurrence rate of Kawasaki disease is as rare as 1-3%. Especially in cases with coronary artery involvement, recurrent Kawasaki disease should be investigated in terms of underlying rheumatologic diseases such as periodic fever syndromes, microscopic polyangiitis, polyarteritis nodosa, and systemic-onset juvenile arthritis.

Native valve endocarditis due to Candida albicans in two children: Two new case reports.

Candida endocarditis (CE) is a rare and serious complication of candidemia. Using current diagnostic tools a confirmed diagnosis is often delayed and outcomes remain poor. The majority of new cases occur following cardiac valvular surgery, and/or in patients with the following risk factors: intravenous drug use, cancer chemotherapy, prolonged presence of central venous catheters, and prior history of bacterial endocarditis.

Optogenetic Monitoring of the Glutathione Redox State in Engineered Human Myocardium.

Redox signaling affects all aspects of cardiac function and homeostasis. With the development of genetically encoded fluorescent redox sensors, novel tools for the optogenetic investigation of redox signaling have emerged. Here, we sought to develop a human heart muscle model for in-tissue imaging of redox alterations.

Early and mid-term outcomes after surgical repair of congenital supravalvular aortic stenosis with the Doty technique.

Objective: The aim of this study was to evaluate the efficacy and safety of the Doty surgical approach in pediatric patients with congenital supravalvular aortic stenosis (SVAS) by examining early and mid-term outcomes.

Methods: Surgical repair using the Doty technique was performed in a total of 10 pediatric patients with SVAS between January 2005 and July 2015 at this clinic. These patients were evaluated retrospectively.

Defined Engineered Human Myocardium With Advanced Maturation for Applications in Heart Failure Modeling and Repair.

Background: Advancing structural and functional maturation of stem cell-derived cardiomyocytes remains a key challenge for applications in disease modeling, drug screening, and heart repair. Here, we sought to advance cardiomyocyte maturation in engineered human myocardium (EHM) toward an adult phenotype under defined conditions.

Methods: We systematically investigated cell composition, matrix, and media conditions to generate EHM from embryonic and induced pluripotent stem cell-derived cardiomyocytes and fibroblasts with organotypic functionality under serum-free conditions.

Chitotriosidase enzyme activity: is this a possible chronic inflammation marker in children with common variable immunodeficiency and early atherosclerosis?

Background Common variable immunodeficiency is a rare clinically symptomatic primary immunodeficiency disorder which manifests a wide variability of symptoms, complications. Atherosclerosis in common variable immunodeficiency patients has not been investigated yet contrary to other severe clinical complications. We aimed to investigate the chitotriosidase enzyme's role as an inflammation and atherosclerosis marker in paediatric common variable immunodeficiency patients.

Lipoprotein-associated phospholipase A: a new marker to determine cardiovascular risk in hypercholesterolemic dyslipidaemic children.

Background Inflammation and hypercholesterolaemia contribute to atherosclerotic changes which can start in childhood. Children with hyperlipidaemias are at high risk for early coronary atherosclerosis. This study evaluates the relationship between lipoprotein-associated phospholipase A (Lp-PLA), carotid intima-media thickness (CIMT) and flow-mediated dilatation in hypercholesterolaemic dyslipidaemic children.

Cor triatriatum sinister: a case series.

Objective: As a highly rare congenital defect, cor triatriatum sinister represents only 0.1% of congenital cardiac anomalies. Depending on the degree of obstruction and the accompanying symptoms, cor triatriatum can be diagnosed at any age.

Nonsevere acute pulmonary embolism: prognostic CT pulmonary angiography findings.

Purpose: To retrospectively evaluate the prognostic parameters of computed tomography (CT) pulmonary angiographic findings in nonsevere (hemodynamically stable) pulmonary embolism (PE) patients and to assess the predictive value of these parameters for mortality within 1 month of the initial diagnosis.

Materials And Methods: Retrospectively, 67 consecutive patients (28 men, 39 women; mean age, 63.25 ± 18 years) from 2 centers with nonsevere PE diagnosed using CT and a clinical evaluation were included in the current study.

Pre- and post-conditional inhibition of prolyl-4-hydroxylase domain enzymes protects the heart from an ischemic insult.

Several genetically modified mouse models implicated that prolyl-4-hydroxylase domain (PHD) enzymes are critical mediators for protecting tissues from an ischemic insult including myocardial infarction by affecting the stability and activation of hypoxia-inducible factor (HIF)-1 and HIF-2. Thus, the current efforts to develop small-molecule PHD inhibitors open a new therapeutic option for myocardial tissue protection during ischemia. Therefore, we aimed to investigate the applicability and efficacy of pharmacological HIFα stabilization by a small-molecule PHD inhibitor in the heart.

miR-133a enhances the protective capacity of cardiac progenitors cells after myocardial infarction.

miR-133a and miR-1 are known as muscle-specific microRNAs that are involved in cardiac development and pathophysiology. We have shown that both miR-1 and miR-133a are early and progressively upregulated during in vitro cardiac differentiation of adult cardiac progenitor cells (CPCs), but only miR-133a expression was enhanced under in vitro oxidative stress. miR-1 was demonstrated to favor differentiation of CPCs, whereas miR-133a overexpression protected CPCs against cell death, targeting, among others, the proapoptotic genes Bim and Bmf.

Lights on for HIF-1α: genetically enhanced mouse cardiomyocytes for heart tissue imaging.

Background/aims: The hypoxia inducible factor-1 (HIF-1) is a suitable marker for tissue oxygenation. We intended to develop cardiomyocytes (CMs) expressing the oxygen-dependent degradation domain of HIF-1α fused to the firefly luciferase (ODD-Luc) followed by proof-of-concept for its applicability in the assessment of heart muscle oxygenation.

Methods And Results: We first generated embryonic stem cell (ESC) lines (ODD-Luc ESCs) from a Tg ROSA26 ODD-Luc/+ mouse.

Chitotriosidase as a possible marker of clinically evidenced atherosclerosis in dyslipidemic children.

A correlation has been clearly shown between inflammation markers and subclinical atherosclerosis markers in the early stages of atherogenesis in subjects with familial hypercholesterolemia (FH). The aim of this study was to investigate potential inflammation markers in the diagnosis of atherosclerosis in children with FH. A total of 48 dyslipidemic children and 24 healthy age-matched control subjects were taken into study.