A Quite Rare Association: Levo-Malposition of the Great Arteries with Left Juxtaposition of the Atrial Appendages in "Double Outlet Right Ventricle".

Although many anatomical variations may be encountered in children with double outlet right ventricle, coexistence of levo-malposed great vessels and left juxtaposed atrial appendages is uncommonly observed. This case report underlines the rarity of this anatomical combination and its clinical significance along with the surgical management in an infant.

A Rare Associated Anomaly in Tetralogy of Fallot: Scimitar Syndrome in an Infant.

Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture.

Successful radiofrequency ablation of accessory pathway associated with left atrial appendage aneurysm in a low birthweight premature patient.

Ergül Y, Öztürk E, Özgür S. Successful radiofrequency ablation of accessory pathway associated with left atrial appendage aneurysm in a low birthweight premature patient. Turk J Pediatr 2019; 61: 142-146.

The role of intraoperative epicardial echocardiography in pediatric cardiac surgery.

Aim: Our aim was to evaluate the findings and the role of intraoperative epicardial echocardiography (IEE) in the management of pediatric cardiac surgery patients.

Methods: Patients evaluated with IEE between December 2015 and December 2017 were analyzed retrospectively. Demographic data, preoperative transthoracic echocardiography (TTE), and IEE reports were evaluated.

Sustained tachyarrhythmia in children younger than 1 year of age: Six year single-center experience.

Background: The aim of this study was to evaluate the results of non-operational sustained tachyarrhythmia in patients <1 year of age at the present center.

Methods: Between November 2010 and November 2016, the demographic characteristics, type and localization of the tachyarrhythmia, echocardiographic findings, and medical and/or ablation therapy for patients <1 year of age with sustained tachyarrhythmia were evaluated.

Results: Of 99 patients, 91 had sustained supraventricular tachycardia, and eight had sustained ventricular tachycardia.

Spontaneous Remission of Congenital Complete Atrioventricular Block in Anti-Ro/La Antibody-Negative Monozygotic Twins: Case Report.

Background: Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins.

Case Report: The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35.

Junctional ectopic tachycardia in late period after early postoperative complete atrioventricular block: Messenger of return to normal sinus rhythm? : Explanation with four case series.

Junctional ectopic tachycardia(JET) is a rare childhood arrhythmia originating from the area adjacent to the atrioventricular(AV) node. It often occurs after surgical procedures like repair of Tetralogy of Fallot, atrioventricular septal defect and ventricular septal defect, which are all performed in that area. While AV block (AVB) can occur after JET, it is very rare for late JET occurring after early postoperative AVB to be followed by normal sinus rhythm (NSR).

Comparison of Transcatheter Atrial Septal Defect Closure in Children, Adolescents and adults: Differences, Challenges and Short-, Mid- and Long-Term Results.

Background And Objectives: This study aims to compare the characteristics, effectiveness and results of transcatheter closure of atrial septal defect between children, adolescents, and adults.

Subjects And Methods: In this study, 683 patients who underwent atrial septal defect closure in the last 10 years were divided into three groups: children (age <12), adolescents (age 12 to 16), and adults (age >16) as group 1, group 2 and group 3, respectively.

Results: The average defect size and incidence of complex atrial septal defect were higher in group 3 (p=0.

Cryoablation with an 8-mm-Tip Catheter for Right-Sided Accessory Pathways in Children.

Background: Cryoablation is increasingly utilized in children because of its safety profile. Recently, larger catheter tips have been more widely used to improve long-term success rates. The aim of this study was to assess the safety and efficacy of 8-mm-tip catheters for cryoablation of right-sided accessory pathways (APs) in children.

Spectrum of clinical manifestations in two young Turkish patients with congenital generalized lipodystrophy type 4.

Congenital generalized lipodystrophy type 4 is an extremely rare autosomal recessive disorder. We report our clinical experience on two unrelated Turkish patients with congenital generalized lipodystrophy type 4. A 13-year-old girl (patient-1) presented with generalized lipodystrophy and myopathy.

Fondaparinux Treatment in a Neonate with Heparin Induced Thrombocytopenia during Extracorporeal Life Support.

The utilization of mechanical life support due to respiratory insufficiency and serious cardiac pathologies have been increasing. Anticoagulation to prevent clot formation is mandatory and unfractioned heparin is the standard therapy in use. But rarely heparin induced thrombocytopenia (HIT) might develop as an immunologic side effect of heparin.

Heart rate variability improvement in children using transcatheter atrial septal defect closure.

Objective: We evaluated autonomic behavior by examining heart rate variability (HRV) in the time domain and frequency domain in pediatric patients who underwent transcatheter closure of atrial septal defect (ASD).

Methods: A prospective study design was used. Holter ECG was performed in a control group of 30 healthy subjects and a group of 47 patients who underwent transcatheter ASD closure.

The value of coronary artery calcium score in the early diagnosis of coronary artery disease in patients with stable chronic obstructive pulmonary disease.

Objective: Our aim was to assess the value of coronary artery calcium score (CACS) in the early diagnosis of coronary artery disease in Global Initiative for Chronic Obstructive Lung Disease (GOLD) stage II chronic obstructive pulmonary disease (COPD) patients and to identify high-risk patients.

Methods: Forty-two patients with GOLD stage II COPD and 31 healthy control subjects were enrolled in the study. This study was designed as a prospective observational cross-sectional study.

Serum NGAL, cystatin C and urinary NAG measurements for early diagnosis of contrast-induced nephropathy in children.

Aim: The study investigated a number of biomarkers for the early diagnosis of contrast-induced nephropathy (CIN), which is an important cause of acute kidney injury (AKI).

Material And Methods: The study included 91 children scheduled for elective cardiac angiography and 50 healthy controls. Biomarkers including serum (s) and urinary (u) sodium, serum and u-creatinine, s-cystatin-C, serum neutrophil gelatinase-associated lipocalin (NGAL) and urinary N-acetyl beta glucosaminidase (u-NAG)/creatinine ratio were measured 4 times sequentially in the patients and once in the controls.

Effects of atomoxetine on heart rhythm in children and adolescents.

Background: The aim of this study was to examine the effects of atomoxetine on heart rhythm using 12-lead electrocardiography (ECG) and 24 h Holter monitoring.

Methods: Children and adolescents who were diagnosed with attention deficit-hyperactivity disorder according to DSM-IV-TR were referred to a pediatric cardiology clinic for cardiologic examination before and after 4 or 5 weeks of atomoxetine treatment. Cardiac examination, complete blood count, biochemistry, thyroid function tests, 12-lead ECG and 24 h Holter monitoring were performed routinely in all patients.

Extracorporeal cardiopulmonary resuscitation for breath-holding spells followed by cardiac arrest due to left main coronary artery stenosis.

Non-syndromic congenital supravalvular aortic stenosis (SVAS) leads to ventricular hypertrophy and increased oxygen consumption, and when combined with other factors reduces coronary blood flow, potentially resulting in myocardial ischemia and sudden cardiac death. While the anatomic obstruction of coronary circulation is as common in non-syndromic SVAS as in Williams syndrome, it often remains unacknowledged. Extracorporeal membrane oxygenation (ECMO) is an elective procedure that can be used to support patients with cardiac arrest during diagnosis as a way to reduce cardiopulmonary load in preparation for surgery or further treatment.

Utility of Exercise Testing and Adenosine Response for Risk Assessment in Children with Wolff-Parkinson-White Syndrome.

Background: We aimed to determine the correlation between noninvasive testing (exercise stress testing [EST] and adenosine responsiveness of accessory pathway [AP] ) and invasive electrophysiology study (EPS) for assessment antegrade conduction of the AP in Wolff-Parkinson-White syndrome.

Patients And Method: This prospective, observational study enrolled 40 children (58% male children, median age of 13 years, and median weight of 47.5 kg) with Wolff-Parkinson-White syndrome.

Electrocardiographic and electrophysiologic effects of dexmedetomidine on children.

Background: Dexmedetomidine (DEX) is a highly selective alpha-2-adrenergic agonist approved for short-term sedation and monitored anesthesia care in adults. Its effects on the electrocardiography and cardiac conduction tissue are not well described in the literature. Therefore, we aimed to characterize the electrocardiographic and electrophysiologic effects of DEX in children.

Successful transvenous lead extraction using the Evolution System in a 17-kg child.

Despite technological advancements in the field of pacemaker lead extraction, available data on pediatric patients is limited, and risk of failure and severe complications remains high. In this report, we present the case of a male patient who, at five months old, had been transvenously implanted with a single-chamber ventricular pacemaker due to complete atrioventricular block. At 7-year of age, the patient was referred to us with growth-related lead tension, severe tricuspid insufficiency, and weak battery.