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.

Can biomarkers predict myocardial iron overload in children with thalassemia major?

Aim: Beta-thalassemia major requires regular blood transfusions throughout life, which in turn leads to iron accumulation in the body. While cardiac T2* MRI is the gold standard in determining cardiac iron accumulation, it is not always feasible, which has led to the search for new biomarkers. Herein, the value of growth differentiation factor-15, galectin-3, and N-terminal pro-B-type natriuretic peptide in predicting cardiac iron accumulation is investigated in asymptomatic children with beta-thalassemia major.

Early and Mid-Term Follow-Up Results After Transcatheter Closure of Secundum Atrial Septal Defect.

Objective: Transcatheter secundum type atrial septal defect closure is an alternative to surgical closure in many cases when conditions are appropriate. In this study, the demographic data and follow-up results of patients with secundum atrial septal defect undergoing transcatheter closure were discussed.

Materials And Methods: Data of patients who underwent transcatheter closure of secundum atrial septal defect between 2004 and 2017 were investigated retrospectively.

Evaluation of left ventricular function and myocardial deformation in children with beta-thalassemia major by real-time three-dimensional (four-dimensional) and speckle tracking echocardiography.

Aim: This study aimed to evaluate the role of real-time three-dimensional (four-dimensional) and speckle tracking echocardiography for early detection of left ventricular systolic dysfunction and also for the relationship between myocardial deformation parameters and myocardial iron load which is measured by cardiac magnetic resonance relaxation time T2* values in asymptomatic children with beta-thalassemia major.

Material And Methods: This multicenter cross-sectional study included 40 patients (mean age 15.4 ± 2.

Evaluation of the left ventricular systolic function and myocardial deformation by real-time three-dimensional (four-dimensional) and speckle-tracking echocardiography in children with type 1 diabetes mellitus.

Aim: This study aimed to evaluate the role of real-time three-dimensional (known as four-dimensional) echocardiography and three-dimensional speckle-tracking echocardiography for the early detection of left ventricular systolic dysfunction in asymptomatic children with type 1 diabetes mellitus.

Materials And Method: This cross-sectional study included 38 patients (mean age 15.4 ± 2.

Evaluation of ventricular function and myocardial deformation in children with repaired tetralogy of Fallot by real-time three-dimensional (four-dimensional) echocardiography.

Aim: The left and right ventricular dysfunction are important clinical course indicators in patients with repaired tetralogy of Fallot. This study aimed to evaluate ventricular volumes, functions, and myocardial deformation in children with repaired tetralogy of Fallot by real-time three-dimensional (four-dimensional) echocardiography and compared with healthy children. It also aimed to investigate the relationships between ventricular volumes, functions, and myocardial deformation parameters in the patients.

Evaluation of the relationship between ventricular function and serum growth differentiation factor-15 levels in patients with operated tetralogy of Fallot.

Aim: Growth differentiation factor-15 is a novel biomarker of increasing importance in cardiovascular diseases. This study aimed to evaluate the relationship between ventricular measurements assessed by cardiac magnetic resonance imaging (MRI) and serum growth differentiation factor-15 levels in children with surgically corrected tetralogy of Fallot.

Materials And Method: Serum growth differentiation factor-15 levels were measured in 40 patients (mean age: 15.

Congenital generalized lipodystrophy: The evaluation of clinical follow-up findings in a series of five patients with type 1 and two patients with type 4.

Congenital generalized lipodystrophy (CGL) is a rare disorder characterized by lipoatrophy affecting the face, limbs and trunk, acromegaloid features, hepatomegaly, hypertriglyceridemia, and insulin resistance. The aim of this study is to evaluate the long-term follow-up findings including gastrointestinal and cardiac manifestations of the patients with CGL1 and CGL4, caused by mutations in the AGPAT2 and CAVIN1 genes, respectively. Two patients aged 2 and 9 years with the same biallelic CAVIN1 mutation and five patients aged between 6 months and 11 years 4 months with AGPAT2 mutations have been followed up for 3-9 years.

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.

Echocardiographic Follow-Up of Congenital Aortic Valvular Stenosis II.

We evaluated the natural course of congenital aortic valvular stenosis (AVS) and factors affecting AVS progression during long-term follow-up with echocardiography. Medical records of 388 patients with AVS were reviewed; patients with concomitant lesions other than aortic regurgitation (AR) were excluded. Trivial AVS was defined as a transvalvular Doppler peak systolic instantaneous gradient of < 25 mmHg; mild stenosis, 25-49 mmHg; moderate stenosis, 50-75 mmHg; and severe stenosis, > 75 mmHg.

Comparison of Balloon Dilatation and Surgical Valvuloplasty in Non-critical Congenital Aortic Valvular Stenosis at Long-Term Follow-Up.

The two main modalities used for congenital aortic valvular stenosis (AVS) treatment are balloon aortic valve dilatation (BAD) and surgical aortic valvuloplasty (SAV). This study evaluates residual and recurrent stenosis, aortic regurgitation (AR) development/progression, reintervention rates, and the risk factors associated with this end point in patients with non-critical congenital AVS who underwent BAD or SAV after up to 18 years of follow-up. From 1990 to 2017, 70 consecutive interventions were performed in patients with AVS, and 61 were included in this study (33 BADs and 28 SAVs).

Early Effects of Renal Replacement Therapy on Cardiovascular Comorbidity in Children With End-Stage Kidney Disease: Findings From the 4C-T Study.

Background: The early impact of renal transplantation on subclinical cardiovascular measures in pediatric patients has not been widely investigated. This analysis is performed for pediatric patients participating in the prospective cardiovascular comorbidity in children with chronic kidney disease study and focuses on the early effects of renal replacement therapy (RRT) modality on cardiovascular comorbidity in patients receiving a preemptive transplant or started on dialysis.

Methods: We compared measures indicating subclinical cardiovascular organ damage (aortal pulse wave velocity, carotid intima media thickness, left ventricular mass index) and evaluated cardiovascular risk factors in 166 pediatric patients before and 6 to 18 months after start of RRT (n = 76 transplantation, n = 90 dialysis).

Evaluation of Ventricular Septal Defect with Special Reference to the Spontaneous Closure Rate, Subaortic Ridge, and Aortic Valve Prolapse II.

The medical records of 2283 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. One thousand eight hundred and twenty-three patients had been followed 1 month to 26 years (median 4 years) by echocardiography. Most of 460 patients could not be followed due to transportation of the institution.

Antegrade transvenous balloon angioplasty for coarctation of the aorta in infants with ventricular septal defect.

For a newborn, surgical correction has been the primary treatment of native coarctation at most centers; however, there has been an increased use of balloon angioplasty (BA). The anterograde transvenous (AT) technique is another alternative way for coarctation (AoC) angioplasty in low weight patients with large ventricular septal defect (VSD). Four, 5-day-old to 7-month-old, infants weighing 2500, 2700, 2800, and 3400g, respectively presented to emergency unit (EU) with cyanosis, tachypnea, and loss of weight.

QT dispersion and ventricular arrhythmias in children with primary mitral valve prolapse.

Aim: To investigate ventricular arrhythmias in children with primary mitral valve prolapse and to evaluate its relation with QT length, QT dispersion, autonomic function tests and heart rate variability measurements.

Material And Methods: Fourty two children with mitral valve prolapse and 32 healthy children were enrolled into the study. Twelve-lead electrocardiograms, autonomic function tests, echocardiography and 24-hour rhythm Holter tests were performed.

Update on diagnosis of acute rheumatic fever: 2015 Jones criteria.

In the final Jones criteria, different diagnostic criteria were established for the diagnosis of acute rheumatic fever for low risk and moderate-high risk populations. Turkey was found to be compatible with moderate-high risk populations as a result of regional screenings performed in terms of acute rheumatic fever and rheumatic heart disease. The changes in the diagnostic criteria for low-risk populations include subclinical carditis found on echocardiogram as a major criterion in addition to carditis found clinically and a body temperature of 38.

Cardiovascular alterations do exist in children with stage-2 chronic kidney disease.

Background: Cardiovascular disease (CVD) is an important complication of chronic kidney disease (CKD) in children. However, it is not well known when and how cardiovascular alterations start.

Methods: This cross-sectional, controlled study consisted of 25 patients and 28 healthy controls.

Percutaneous transcatheter snare vegetectomy in a child.

Surgical vegetectomy may be indicated in patients with unresolving sepsis, heart failure, recurrent embolism, or the presence of large vegetations >10 mm in size. Percutaneous vegetectomy using a snare may be a reasonable option instead of open-heart surgery in selected patients. We describe the case of a patient with operated tetralogy of Fallot and infective endocarditis who underwent vegetectomy via a percutaneous approach.

Cardiovascular side effects related with use of synthetic cannabinoids "bonzai" : two case reports.

Information about the effects of synthetic cannabinoids "bonzai" on the cardiovascular system is limited. In this article, two patients in whom different cardiological side effects were observed following use of synthetic cannabinoids 'bonzai' were presented. Our first patient who was a 16-year old boy presented to pediatric emergency department with severe chest pain which had started one hour before.

Evaluation of right ventricular function in patients with tetralogy of Fallot using the myocardial performance index and isovolumic acceleration: a comparison with cardiac magnetic resonance imaging.

Background: Assessment of right ventricular function is a key point in the follow-up of operated patients with tetralogy of Fallot. Cardiac magnetic resonance assessment of right ventricular function is considered the gold standard. However, this technique is expensive, has limited availability, and requires significant expertise to acquire and interpret the images.

Voriconazole induced bradycardia.

Voriconazole is a triazole antifungal drug that is used to treat invasive fungal infections, especially aspergillus. Here, we report two children who had severe bradycardia associated with voriconazole at a dose of 12 mg/kg per day. Bradycardia resolved in 24 hours in both after decreasing the dose to 10 mg/kg per day.

P wave dispersion in juvenile idiopathic arthritis patients with diastolic dysfunction.

Objective: Cardiac involvement as pericarditis, myocarditis and valvular disease is common in juvenile idiopathic arthritis (JIA). However, there are few studies concerning systolic and diastolic functions of the left ventricle in children with JIA. P wave dispersion is a sign for the prediction of atrial fibrillation.