Examination of cardiac functions during acute attack and remission period in children with familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease characterized by recurring serosal inflammation. Cardiac involvement in FMF commonly manifests as pericarditis and pericardial effusion; however, there is limited research on myocardial function. This study aimed to assess cardiac functions during active inflammation and remission periods of FMF patients and investigate the cardiac effects of inflammation during the attack period.

Unveiling Cardiac Involvement in Juvenile Dermatomyositis Through Speckle-Tracking Echocardiography.

Early detection of cardiac involvement in Juvenile Dermatomyositis (JDM) is difficult due to the absence of clinical signs and symptoms, with systolic dysfunction often emerging in late stages and associated with a poor prognosis. This study aimed to employ two-dimensional speckle-tracking echocardiography (STE) for subclinical assessment of left ventricular (LV) systolic failure in JDM and explore potential associations between impaired LV systolic function (LV-GLS) and disease activity. A prospective study enrolled 20 healthy volunteers and 26 JDM patients (< 18 years old) without cardiac symptoms.

Exploring cardiovascular implications of juvenile dermatomyositis: Insights from aortic stiffness analysis and 3D echocardiography.

Objective: Our goal was to use three dimensional (3D) strain analysis to evaluate myocardial function and ascending aorta elasticity changes in juvenile dermatomyositis (JDM).

Methods: Between 2019 and 2021, 23 JDM patients and 20 healthy volunteers participated. Both groups underwent 2D and 3D strain analysis, assessing aortic stiffness using aortic distensibility, stiffness index, strain, and elastic modulus.

Left atrial and left ventricular strain dynamics with echocardiography in asymptomatic children with mitral regurgitation.

Left atrial (LA) dilatation is a strong predictor of cardiovascular morbidity and mortality. Strain parameters evaluating LA functions have gained importance in assessing cardiovascular health. This study aimed to investigate the relationship between LA and left ventricular (LV) strain, using speckle-tracking echocardiography, and assess the impact of vena contracta on LV functioning in asymptomatic children with mitral regurgitation.

Prenatal Diagnosis of Ductus Arteriosus Anomalies: A Single-Center Study.

To evaluate the fetal ductus arteriosus anomalies diagnosed by fetal echocardiography. The perinatal outcomes and associated cardiac and genetic anomalies are also explored. The fetal echocardiography records of 2366 fetuses were evaluated retrospectively.

Retrospective analysis of children diagnosed with Kawasaki disease.

Background: The aim of our study was to evaluate the long-term impacts of Kawasaki disease on our patients regarding coronary involvement demographic characteristics, treatment regimens, and clinical course.

Methods: Our study included 104 patients diagnosed and hospitalized with Kawasaki disease in our center, from January 2004 to January 2019. In our study, patients were divided into three groups according to coronary artery involvement.

Perinatal Outcomes of Fetuses with Prenatally Diagnosed Atrial Appendage Aneurysm.

To evaluate the characteristics and outcomes of fetuses with atrial appendage aneurysm (AAA) diagnosed by fetal echocardiography. The fetal echocardiography records of 1956 fetuses were evaluated retrospectively. Nine pregnancies who had been diagnosed with fetal AAA prenatally and evaluated after delivery were enrolled in the study.

Congenital Heart Defects and Outcome in a Large Cohort of Down Syndrome: A Single-Center Experience from Turkey.

Objective: Congenital heart defects occur in approximately 50% of children with Down syndrome and they contribute considerably to morbidity and mortality. The aim of this study is to investigate the prevalence, classification, and survival of congenital heart defects in Down syndrome.

Materials And Methods: About 1731 Down syndrome patients who underwent echocardiography between 1986 and 2022 were evaluated.

Cardiometabolic Risk Factors in Pediatric Kidney Transplant Recipients.

Objective: There is an increased risk of obesity and metabolic syndrome among kidney transplant recipients, which adversely affects cardiovascular and renal outcomes in these patients. The present study aims to investigate the prevalence of metabolic syndrome in pediatric kidney transplant recipients and the associations of metabolic syndrome with cardiovascular disease and graft function.

Materials And Methods: This cross-sectional, single-center study included 52 kidney transplant recipients (27 males) transplanted before 18 years of age.

A Large Turkish Cohort of Williams Syndrome: The Evaluation of Facial, Cardiovascular, and Neuropsychiatric Features.

Objective: Williams syndrome is caused by a microdeletion at 7q11.23 and is characterized by a distinctive face, cardiovascular disease, and intellectual disability with a specific cognitive and behavioral profile. This study aims to evaluate the clinical features and obtain important information that can guide early diagnoses and correct follow-up.

Mutation identification and prediction for severe cardiomyopathy in Alström syndrome, and review of the literature for cardiomyopathy.

Objective: Alström syndrome (ALMS) is a rare autosomal recessive genetic disorder that is caused by homozygous or compound heterozygous mutation in the ALMS1 gene. Dilated cardiomyopathy (DCM) is one of the well-recognized features of the syndrome ranging from sudden-onset infantile DCM to adult-onset cardiomyopathy, sometimes of the restrictive hypertrophic form with a poor prognosis. We aimed to evaluate severe cardiomyopathy in Alström syndrome in infancy and display susceptible specific mutations of the disease, which may be linked to severe DCM.

Clinical features and outcomes of 76 patients with COVID-19-related multi-system inflammatory syndrome in children.

Objectives: Multi-system inflammatory syndrome in children (MIS-C) is a less understood and a rare complication of coronavirus disease-2019 (COVID-19). Given the scarce data regarding this novel disease, we aimed to describe the clinical features and outcomes of our patients with MIS-C and to evaluate the associated factors for the pediatric intensive care unit (PICU) admission.

Methods: The MIS-C patients under 18 years old diagnosed and treated in three referral centers between July 2020 and March 2021 were included.

Systolic and Diastolic Cardiac Functions in Juvenile Spondyloarthropathies.

Background/objective: Juvenile spondyloarthropathies (JSpAs) are a group of inflammatory diseases characterized by asymmetric peripheral arthritis (especially in lower extremities), axial skeleton involvement, and enthesitis. Although cardiovascular findings of inflammatory diseases such as juvenile systemic lupus erythematosus (SLE) and juvenile scleroderma (SD) are well documented, there are only a few studies assessing the cardiovascular consequences of JSpA in the literature.

Methods: Forty patients with JSpA and 20 healthy controls were included into this cross-sectional study.

Unexpected increase of aortic stiffness in juvenile Spondyloarthropathies.

Juvenile spondyloarthropathy is an umbrella term for a group of childhood rheumatic diseases that can cause chronic arthritis extending to the axial skeleton before the age of 16. Although ankylosing spondylitis has aortic involvement as one of its most important effects, this relationship has not been extensively studied in children with juvenile spondyloarthropathy. Here, a cross-sectional study of the elastic properties of the aorta of 43 patients with juvenile spondyloarthropathy and 19 healthy controls is reported.

A rare cause of sudden cardiac arrest: Catecholaminergic polymorphic ventricular tachycardia.

Catecholaminergic polymorphic ventricular tachycardia is a rhythm disorder that develops due to genetic reasons in the absence of structural cardiac abnormalities. Ventricular tachycardia, ventricular fibrillation, cardiac arrest, and death may occur. Two-year-old patient presented to the Emergency Department with sudden cardiac arrest.

Aortic arch anomalies detected in foetal life by echocardiography.

Aortic arch anomalies refer to congenital malformations of position or branching pattern of the aortic arch. To-date, only a few small studies have documented prenatal detection of aortic arch anomalies. In this article, we share our experience in detecting aortic arch anomalies.

Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission.

Aghdasi-Bornaun H, Kutluk G, Keskindemirci G, Öztarhan K, Dedeoğlu R, Yılmaz N, Tosun Ö. Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission. Turk J Pediatr 2018; 60: 407-414.

New Insights into Cardiac Involvement in Juvenile Scleroderma: A Three-Dimensional Echocardiographic Assessment Unveils Subclinical Ventricle Dysfunction.

Cardiac manifestations in juvenile scleroderma or systemic sclerosis (JSSc) have poor prognosis, begin in early stages of the disease, and remain clinically asymptomatic. New echocardiography modalities, such as 2D/3D speckle tracking (STE, strain analysis for regional and global ventricular functions), can detect cardiac involvement in early stages. We assessed 21 JSSc patients and 19 controls using 2D/3D STE.

Evaluation of pulmonary artery pressure in patients with juvenile systemic lupus erythematosus (jSLE).

Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Previous studies among adults have shown impaired right ventricular (RV) function in patients with SLE. Also, these patients may develop pulmonary artery hypertension (PAH), which is one of the most threatening complications of SLE.

[Our experience in the diagnosis and treatment of postural orthostatic tachycardia syndrome, vasovagal syncope, and inappropriate sinus tachycardia in children].

Objectives: The aim of this study was to share our experience in the diagnosis and treatment of patients who presented at our clinic with syncope, pre-syncope, dizziness, and palpitations.

Study Design: Patients who were treated at pediatric cardiology clinic for complaints of syncope, dizziness, and palpitations between 2014 and 2016 were enrolled in the study. Detailed history of the patients, physical examination findings, laboratory and electrocardiogram results were recorded.

Evaluation of myocardial deformation in patients with Kawasaki disease using speckle-tracking echocardiography during mid-term follow-up.

Unlabelled: Speckle-tracking echocardiography is a recently developed technique for the evaluation of myocardial deformation or strain. Our objective was to examine strain through a mid-term follow-up of patients with Kawasaki disease.

Methods: We explored left ventricular mechanics using speckle-tracking echocardiography in 35 patients with a history of Kawasaki disease at least 6 months after the acute phase.