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Objective: Cardiac conduction system is affected and heart blocks can be seen in newborns whose mothers have connective tissue disease, especially with systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). Anti-SS-A (Ro), anti-SS-B (La) antibodies in the mother's circulation are responsible for this situation. In this study, it was aimed to evaluate the clinical features and long-term follow-up results of babies born to mothers with connective tissue disease in our hospital. Materials and methods: Patients who were hospitalized in the neonatal intensive care unit between January 2001 and January 2016 due to the diagnosis of SLE, SS or connective tissue disease in their mothers were retrospectively screened, and their demographic and clinical characteristics and electrocardiographic findings were recorded. Results: A total of 49 babies from 48 mothers were included in the study. Mean age of mothers at birth was 30.8±5.0 years (28-41), mean gestational week of patients was 35.8±2.5 weeks (28-41), mean birth weight was 2614±680 g (730-3810 g). Ten newborns (20.4%) had 3rd degree atrioventricular (AV) block, and 1 baby had 1st degree AV block. Permanent pacemaker was implanted in five patients in the neonatal period, two of these patients died in the neonatal period. A pacemaker was inserted in a patient who was followed up with AV block in the 6th month. One baby who had no cardiac conduction problem died due to reasons related to prematurity. The mean follow-up period of 46 living babies was 4.6±3.1 (1.2-10.75) years, and the follow-up period of the patients with complete AV block and without a pacemaker was 5.6±2 years. Conclusion: Complete AV block in the neonatal period is a serious cardiac problem that requires rapid intervention. Expectant mothers with known connective tissue disease should be followed in tertiary care centers and the fetus should be followed closely. It is possible for newborns in need to return to their normal lives by inserting pacemakers.

Introduction:Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein’s anomaly.Methods and results:During the study period, of 89 patients diagnosed with Ebstein’s anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully.Conclusions:Ablation in patients with Ebstein’s anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

Objective We aimed to describe the typical clinical and laboratory features and treatment of children diagnosed with multisystem inflammatory syndrome in children (MIS-C) and to understand the differences as compared to severe/critical pediatric cases with COVID-19 in an eastern Mediterranean country. Methods Children (aged

Although implanted cardiac devices improve patients’ physical health, long-term psychosocial effects especially in the paediatric population are still unknown. The aim of this study was to evaluate the psychosocial effects of cardiac devices in a paediatric population.Pediatric Quality of Life Questionnaire (PedsQoL) was used to evaluate life quality, Connor–Davidson Resilience Scale was used to evaluate resilience and Brief Symptom Inventory was used to evaluate psychiatric symptoms in a paediatric population with cardiac devices.Seventy-one patients were enrolled in the study. Fifty of them (70.5%) had a cardiac pacemaker and 21 of them (29.5%) had implantable cardioverter defibrillator. When compared to the control group both implantable cardioverter defibrillator and pacemaker using patients had lower quality of life (79.5 ± 12.4 versus 86.7 ± 12.1, p = 0.001) but no difference was observed in resilience and psychological pathologies. Age, gender, family size, and education level had no effect on quality of life. Implantable cardioverter defibrillator bearing patients had higher levels of anxiety than pacemaker patients (0.58 versus 0.30 p = 0.045), and implantable cardioverter defibrillator patients who had received shock in the last year had higher levels of somatisation than the group that did not receive device shock (0.17 versus 0.44 p = 0.022).In conclusion study showed that cardiac devices have negative effects on the psychosocial health of children. Cardiologist working with these patients should be aware of these pathologies and monitor not only physical health but also psychosocial health too.

Children with heart diseases have reduced physical activity (PA) levels relative to their peers, which in turn increases cardiovascular risk. To the best of our knowledge, physical fitness and objectively measured PA levels have not been previously studied in children with pacemakers. We evaluated PA levels and physical fitness in pediatric pacemaker patients compared to their healthy peers. Twenty-eight pediatric patients with pacemakers (15 female, 13 male; mean age 13.43 ± 3.68 years) and 24 healthy subjects (14 female, 10 male; mean age 13.08 ± 3.67 years) were included. Physical fitness was assessed using the Munich Fitness Test (MFT). SenseWear Armband metabolic Holter device was used to record the PA for 7 consecutive days. MFT total and sub-parameter scores were significantly lower in the patient group (p 0.05). Patients' total and active energy expenditure, PA level, total distance, number of steps, and vigorous PA were significantly lower than those of healthy children (p 0.05). Sedentary activity and light, moderate, and very vigorous PA durations were similar in both groups (p 0.05). Duration of mean moderate to vigorous PA was higher than 60 min/day recommended in PA guidelines in both patients and healthy subjects. These results provide initial data on PA and fitness in children with pacemakers and suggest that physical fitness and activity levels in children with pacemakers are lower than in healthy peers. Appropriate exercise programs may improve PA levels in pediatric pacemaker patients. Awareness of the importance of PA should be raised among the parents and families of these children.

Necrotizing enterocolitis (NEC) has a multifactorial etiology and predominantly affects preterm infants, with only 13% of cases occurring in term infants. Hypoxia, sudden cardiovascular changes can also affect mesenteric perfusion. It is assumed that decreased mesenteric blood flow, followed by reperfusion, can contribute to intestinal damage, thereby increasing NEC incidence rates in term infants. This report presents a case of NEC development during follow-up for atrial flutter (AF). Repeated cardioversion caused sudden hemodynamic changes, causing NEC.

“Torsades de pointes”, a life-threatening rhythm disorder, is a polymorphic ventricular tachycardia that usually develops in association with a prolonged QT interval. Fluconazole, an anti-fungal drug, may also induce QT prolongation, in some cases subsequent torsades de pointes. Herein, we report a 16-year-old female presenting “torsades de pointes” after administration of fluconazole and rapidly improved upon cessation of the drug.

Coronary sinus abnormalities are usually associated with arrhythmia disorders when symptomatic. We report a 5-year-old 14 kg patient with a giant diverticulum of coronary sinus and Wolff-Parkinson-White syndrome. Catheter ablation therapy was decided during follow-up due to inadequate response to multidrug therapy. Posteroseptal and left posterolateral accessory pathways were established and radiofrequency ablation was performed successfully through coronary sinus.

AIM The aim of our study was to evaluate the safety and efficiency of the 'figure-of-eight' suture among children and young adults with congenital heart defects who underwent interventional procedures, in patients with structurally normal hearts who underwent electrophysiological study and in haemodynamically impaired children and newborns. We also reported a novel femoral haemostasis method in patients with a central catheter by modifying the 'figure-of-eight' suture around the catheter for haemorrhage control. METHOD Between 2015 and 2018, a total of 100 'figure-of-eight' sutures were performed in 90 patients (48 males, 42 females) where the median age was 12.5 years (minimum 3 days, maximum 22 years). The procedures were diagnostic angiography (n = 6), radiofrequency and/or cryoablation (n = 7) and interventional procedures (n = 87). RESULT Haemostasis was achieved in 89 of 90 patients. Haemostasis could not be achieved in one malnourished patient due to lack of subcutaneous tissue. There were no major complications. A bullous skin lesion and minor bleeding were the only complications seen in two patients. A central catheter was inserted in eight patients using the modified 'figure-of-eight' suture technique. CONCLUSION The 'figure-of-eight' suture is a safe and effective method for femoral venous haemostasis in patients who require large sheaths for procedures, in those using high-dose heparin and in haemodynamically unstable children who need cardiac catheterisation.

INTRODUCTION Cardiac sympathetic denervation (CSD) is an effective procedure for the treatment of inherited channelopathies. Left CSD has traditionally been recommended as neuromodulation to prevent arrhythmia episodes; however, it is thought that bilateral sympathectomy in combination with Kuntz ablation may have additional effects effective due to the anatomical variability of preganglionic sympathetic fibers. The aim of the study was to share our single-center clinical experience with bilateral thoracoscopic sympathectomy for cardiac denervation in different groups of pediatric patients with malignant arrhythmias. METHODS Fourteen patients (seven with CPVT, five with LQTS, one with resistant ventricular tachycardia as a sequela of myocarditis, and one with cardiomyopathy and atrial tachycardia) underwent bilateral thoracoscopic sympathectomy for cardiac denervation. RESULTS In all patients, arrhythmia episodes persisted despite medical therapy, and patients with implantable cardioverter-defibrillator received appropriate therapies. The rate of appropriate therapies was 3.25 per year (1-5 per year) in the year before sympathectomy. No major complications related to the procedure were observed in any of the patients. The QTc interval of LQTS patients decreased from 506.2 ± 16.9 ms before the procedure to 476 ± 28.8 ms after the procedure. The mean duration of follow-up after the procedure was 23.3 months (11-47 months). Only two patients received single episodes of therapy 12 and 22 months after CSD, and one patient had arrhythmic events due to noncompliance to medical therapy. CONCLUSION Due to the anatomical variability of preganglionic fibers bilateral CSD with Kuntz nerve ablation effective treatment and is a safe option.

BACKGROUND In pediatric patients with documented narrow QRS tachycardia that is suggestive of atrioventricular nodal reentrant tachycardia (AVNRT) and not inducible in electrophysiological study (EPS), empiric slowpathway ablation (ESPA) may be considered. There is limited data in children about this topic. METHODS Seventy-nine patients who underwent cryoablation and/or radiofrequency ablation (RFA) for presumed AVNRT between January 2010 and January 2020, with no inducible tachycardia and no other tachycardia mechanisms during EPS, were included in this study. RESULTS The age was between 6 and 18 years. All patients had no structural heart disease. Preablation exhibited sustained SP conduction for all patients. In all cases, the ablation end points were prolongation in wenckebach cycle length (WBCL) with loss of cross and/or jump, and/or echo beat. The end points were not achieved in two patients. Overall, the mean basal WBCL increased to 351 ms (240-500 ms) from 301.3 ms (180-420 ms), evident in the non-recurrence group. Nine patients had a transient AV block that improved. We followed the patients without medication for about 46.9 months (8 months to 10 years). Palpitations occurred again in 9 of 77 patients (clinical recurrence rate 9/79 - 11.3%). The documented ECG recurrence rate was 1.2% (1/79). In the non-recurrence group, WBCL prolongation was higher and mean age was lower than in the recurrence group (13.075 vs. 15.33 years). CONCLUSION In cases with presumed AVNRT, ESPA seems to be a reasonable and safe way. In our study, we found our procedural success rate as 97.4% and follow-up recurrence rate as 12.6% (9+1/79).

Turner syndrome (TS) is one of the most common malformation syndromes in females. A total number of 107 TS patients, diagnosed between 2000 and 2018, were evaluated for their phenotypic features, and cardiac and renal findings. The mean age of patients at admission was 10.08 ± 4.9 years (range, newborn to 18 years). Four different karyotype groups were encountered, and the most common findings in all groups were short stature, followed by cubitus valgus. Echocardiographic findings of 85 patients were available among which 63 (n = 63/85, 74.1%) were found to be normal. The most common cardiac anomaly was left ventricular outflow tract/aortic arch pathology detected in 9 patients (n = 9/22, 40.9%). Renal malformations were detected in 15 patients (n = 15/84, 17.9%) by renal ultrasonography, and horseshoe kidney was the most common renal malformation, followed by left multicystic dysplastic kidney. There was no significant difference in the frequency of renal malformation and cardiac anomalies among the 4different karyotype groups (χ2 exact test, p > 0.05). Compared with the literature, the frequency of renal anomalies was detected at a lower rate. Karyotype analysis should be carried out in all female patients with short stature, even if there are no associated phenotypic findings suggestive of TS. Since cardiac anomalies are frequently seen in TS patients and they represent a common cause of mortality, echocardiography should be carried out as soon as the definite diagnosis is established. Renal anomalies may be less frequent than cardiac anomalies; however, evaluation of TS patients with renal ultrasonography should be done at the time of diagnosis. Although renal ultrasonography can be used as the initial renal screening in TS patients, it may underestimate the frequency of renal malformation; hence, further management may be required.

Andersen-Tawil syndrome is a rare autosomal dominant genetic or sporadic disorder characterized by periodic paralysis, ventricular arrhythmias and dysmorphic features. Ventricular arrhythmias can include frequent premature ventricular complex, polymorphic ventricular tachycardia, and less frequently bidirectional ventricular tachycardia. Left ventricle function has been reported in only a few individual cases of Andersen-Tawil syndrome. A 14-year-old female patient was referred to our clinic from another center with documented arrhythmia and left ventricular systolic dysfunction. Andersen-Tawil syndrome was suspected and the diagnosis was confirmed after detection of a previously unreported mutation in children. We report the successful use of flecainide in bidirectional ventricular tachycardia and tachycardia-induced cardiomyopathy in a case of Andersen-Tawil syndrome associated with a novel mutation.

The coexistence of different mechanisms of arrhythmia and multiple accessory pathways (MAPs) leading to multiple ablation targets is rarely seen in children, and data regarding these patients in the literature are limited. Herein, we aimed to evaluate patients who required multiple ablation applications, focusing on different targets during the procedures in children, and evaluating the characteristics of coexistent arrhythmia and MAPs, and the results of these procedures in children. Ablation procedures conducted between March 2009 and December 2018 were evaluated retrospectively, and patients with MAPs and/or coexistent arrhythmia who had undergone ablation procedures were included in the study. Among the 1210 patients who underwent ablation procedures, 52 patients (26 male, 26 female) were ablated for multiple targets. Of the 456 patients with APs, 21 had MAPs (4.6%) and of the 1210 patients who underwent ablation procedures, 31 patients had coexistent arrhythmia (2.5%). The patients had a mean age of 12.24 ± 3.4 (4–18) years and mean body weight of 45.17 ± 14.12 (17–74) kg. A total of 110 APs or foci were identified as quaternary in one patient, while it was triple in four patients. The procedures were unsuccessful in six targets of six patients. Although recurrence was observed in four patients, none were ablated for MAPs. Two complications were encountered, comprising ST segment depression that developed in one patient with Wolf-Parkinson-White syndrome, atrioventricular nodal re-entry tachycardia, and a temporary atrioventricular block during atrioventricular nodal re-entrant tachycardia ablation. The overall success rate according to the pathway/foci number was 94.5% (104/110), with a recurrence rate of 4.5% (5/110), and a complication rate of 1.8% (2/110). The patient success, recurrence, and complication rates were 88.4% (46/52), 7.6% (4/52), and 3.8% (2/52), respectively. In conclusion, the incidence of multiple arrhythmogenic foci and MAPs were not as low as expected in children. A structured and stepwise approach is mandatory for the diagnosis of the different mechanisms of tachycardia, even after successful ablation procedures. The success, recurrence, and complication rates were comparable with those of patients who had a solitary arrhythmogenic focus or solitary AP.

Background:Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term study.Aim:We have recently reported that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension in a 24-week prospective study and well tolerated without adverse events. We now aimed to evaluate clinical efficacy, safety of switch in a larger patient population, in a 24-month prospective study.Methods:This is a single-institution, 24-month prospective study. Patients ≥12 years with idiopathic/heritable, pulmonary arterial hypertension, or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan treatment were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors/inhaled prostanoids was allowed. Outcome measures included change from baseline to 24 months, in the 6-minute walk distance, functional class, oxygen saturation at rest/after walk distance test, and natriuretic peptide levels. Safety end points included adverse events, laboratory abnormalities.Results:Twenty-seven patients (19 adults/8 children, mean age: 21.1 ± 6.3 years (12–36), weight: 53.1 ± 15.7 kgs (26–87)) were included. Mean duration of macitentan treatment: 22.3 ± 3.9 months (9–24). Six-minute walk distance significantly improved from baseline (mean: 458 ± 79 m (300–620)) at 6 months (mean: 501 ± 73 m (325–616) + 43 m) (p < 0.05), at 12 months (mean: 514 ± 82 m (330–626) + 56 m) (p < 0.05), and at 24 months (mean: 532 ± 85 m (330–682) + 74 m) (p < 0.05). We observed a significant improvement during the first 6 months but no incremental improvement after 6 months (p > 0.05). Macitentan did not significantly change functional class, oxygen saturation, and natriuretic levels (p > 0.05). None of the patients had anaemia, hepatotoxicity, and peripheral edema.Conclusions:Our study is the first study which showed that switch from bosentan to macitentan improved exercise capacity in children and young adults with pulmonary arterial hypertension significantly in the first 6 months and compared to baseline in 24 months and well tolerated without adverse events.

Background: Myocarditis is an inflammatory condition located mainly in the myocardium. It is caused by a variety of bacterial and viral infections. Influenza is one of the most common relevant viruses that cause myocarditis. Objectives: We attempted to share our experiences about clinical and laboratory findings, cardiac evaluation, and treatment of children with influenza myocarditis. Methods: This retrospective study was performed by the Department of Pediatric Infectious Diseases at the Faculty of Medicine, Hacettepe University in Turkey. The medical records of patients diagnosed with myocarditis associated with an influenza infection between January 2014 and January 2017 were systematically reviewed. Results: Vaccination seems likely to be an important protection strategy for both influenza infections and complications. (C) 2018 The Authors. Published by Elsevier Limited on behalf of King Saud Bin Abdulaziz University for Health Sciences.

To analyze the role of sensitive cardiac troponin I (scTnI) and high-sensitive troponin T (hscTnT) in the determination of myocardial injury caused by volume and pressure load due to pulmonary hypertension (PH) and to investigate if these markers may be useful in the management of PH in childhood. Twenty-eight patients with congenital heart disease (CHD) with left to right shunt and PH, 29 patients with CHD with left to right shunt but without PH, and 18 healthy children, in total 75 individuals, were included in the study. All cases were aged between 4 and 36 months. Echocardiographic evaluation was performed in all cases, and invasive hemodynamic investigation was performed in 33 cases. Blood samples were obtained from all cases, for the measurement of brain natriuretic peptide (BNP), pro-brain natriuretic peptide (pro-BNP), sensitive cardiac troponin I (scTnI), and high-sensitive troponin T (hscTnT) levels. The mean BNP, pro-BNP, scTnI, and hsTnT levels were statistically significantly higher in patients with PH than in the patients without PH (p

BackgroundMacitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term event-driven study.AimFew studies compared the clinical efficacy and safety of switch from bosentan to macitentan only in adult patients with pulmonary arterial hypertension. We aimed to evaluate the clinical efficacy and safety of switch from bosentan to macitentan in children and young adults.MethodsThis is a single-institution, 24-week prospective study. Patients ⩾12 years of age with idiopathic/heritable pulmonary arterial hypertension or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan therapy were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors and inhaled prostanoids was allowed. Outcome measures included change from baseline to week 24, in the 6-minute walk distance, functional class, oxygen saturation at rest/after 6-minute walk distance test, systolic pulmonary artery pressure estimated by echocardiography, and brain natriuretic peptide levels. Safety end points included adverse events laboratory abnormalities.ResultsA total of 13 patients – 5 male and 8 female – completed the study. The mean age was 20.3±6.5 years (12–35) and weight was 54.0±14.5 kg (27–75). Five patients were ⩽18 years of age. Macitentan improved 6-minute walk distance from baseline (mean: 466±35 m (300–590)), at 12 weeks (mean: 494±78 m (325–590), +28 m) (p0.05). None of the patients had anaemia, hepatotoxicity, and peripheral oedema.ConclusionsOur study is the first study that showed that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension and is well tolerated without any adverse events.

Cardiac involvement is a rare initial presentation of familial Mediterranean fever (FMF). We described 2 children with massive pericardial effusion and cardiac tamponade, who were later diagnosed as having FMF based on clinical and laboratory findings. Therefore, in children presenting with massive pericardial effusion, FMF should be considered as one of the differential diagnoses. In addition, massive pericardial effusion is a serious clinical condition that requires emergency therapeutic approach including pericardiocenthesis. For medical treatment, colchicine is the first line therapy, but in resistant cases, other anti-inflammatory drugs can be used for extra anti-inflammatory effect.

The aim of this study was to review the characteristics of patients with Kawasaki disease (KD) from Turkey and to assess the performance of the Kobayashi score (KS), Harada score (HS), Formosa score (FS), Egami score (ES) and other parameters in predicting intravenous immunoglobulin (IVIG) resistance and coronary artery involvement (CAI) in the Turkish population. Patients who were diagnosed as being in the acute phase of KD at Hacettepe University Faculty of Medicine (Ankara, Turkey) between June 2007 and January 2016 reviewed retrospectively, and those between January 2016 and February 2018 reviewed prospectively, were included in this cohort study. A total of 100 patients with KD were included in this study. Statistical Package for Social Sciences for Windows 22.0 (SPSS Inc, Chicago, IL, USA) was used for statistical analysis. Eighty-five patients (85%) responded to IVIG treatment, whereas 15 (5 female, 10 male) were IVIG resistant. CAI was detected in echocardiography at diagnosis in 31 (31%) (9 female; 22 male) patients. For predicting IVIG resistance, KS, ES, FS, and HS had sensitivity of 82.1%, 26.7%, 30.8%, 69.2% and specificity of 35.7%, 94%, 51.2%, 45.8%, respectively. For the association with CAI occurrence, the sensitivities were 17.2%, 3.3%, 35.7%, 70.4% and the specificities were 78.5%, 88.4%, 49.3%, 49.3% for the aforementioned scores, respectively. The multivariate analysis showed white blood cell (WBC) count [Odd’s ratio (OR) 4.1; 95% confidence interval (CI) 1.26–13.23; p = 0.019] and hematocrit (OR 3.8; 95% CI 1.15–12.4; p = 0.028), as independent predictors of CAI while gamma-glutamyl transferase (GGT) level (OR 5.7; 95% CI 1.73–27.51; p = 0.018) was detected as the only independent predictor of IVIG resistance. This is the first study from Turkey in KD to evaluate the association of the scoring systems for IVIG resistance and CAI. The risk scoring systems in KD did not predict the risk for IVIG resistance and were not associated with CAI in Turkish population.

Background Despite concerns about complications with the implantable cardioverter defibrillator (ICD), it is effective for the prevention of sudden cardiac death (SCD). We aimed to analyze our midterm experience with ICD in children and young adults. Methods This retrospective study included patients who were implanted with an ICD between 2001 and 2014. Demographic characteristics, clinical information, shock features, and complications for all patients with ICD were analyzed. The study population was divided into two groups: early-era patients implanted before 2008, and late-era patients implanted after 2008. Results Sixty-nine patients (median age: 12 years, median follow-up: 52 months) were implanted with an ICD. Diagnostic categories were channelopathy (56.6%), cardiomyopathy (36.2%), congenital heart disease (5.8%), and other (1.4%). We performed implantation for primary prevention in 66.6% (39.3% in early-era patients and 85.4% in late-era patients). Thirty-one (44.9%) received 139 appropriate shocks (66% of total shocks) while 14 (20.2%) received 71 inappropriate shocks. However, there was no statistically significant difference in the use of appropriate shocks in the primary (66.7%) versus the secondary (72.2%) prevention groups. The incidence of appropriate and inappropriate shock was 66.7% and 33.3% in the primary prevention group, and 72.2% and 27.8% in the secondary prevention group, respectively. Two patients died, although only one death was the result of a lead problem. Conclusions Although lead integrity problems, inappropriate shocks, and infections are significant issues, ICD therapy appears to be a safe, effective, and necessary option for the prevention of SCD in both children and young adults.

Perkutan yerleştirilen santral venöz kateterler premature yenidoğanlara parenteral beslenme sıvıları sağlamak için sıklıkla kullanılmaktadır. Bu çalışma hiperosmolar parenteral beslenmeye bağlı olarak perikardiyal tamponad gelişen iki preterm yenidoğan vakasını sunmaktadır. Perkutan yerleştirilmiş santral venöz kateteri bulunan bir yenidoğanda klinik durum kötüleştiğinde perikardiyal efüzyon tanısı koymak için yüksek klinik şüphe ve hızlı tedavi hayat kurtarıcı olabilir., Percutaneously inserted central venous catheters are commonly used to provide parenteral nutrition for premature neonates. This study presents report of two preterm neonates who developed pericardial effusion resulting from osmotic damage of parenteral nutrition. When a neonate with percutaneously inserted central venous catheter clinical status worsens a high index of clinical suspicion for diagnosing pericardial effusion and immediate treatment might be life saving.

Objective This study aims to determine efficacy and safety of rapid left ventricular pacing (RLVP) during balloon aortic valvuloplasty and effect on development of postprocedural aortic insufficiency (AI) in children. Design This is a retrospective comparison of 56 children (mean age 18.3 month; 1 day–15 years of age) who underwent valvuloplasty by using RLVP with standard method (without pacing) during same time period (74 children; mean age 12.6 month; 1 day–18 years of age). Results The systolic valvular gradient decreased from a mean 67 ± 20.4 mm Hg (26–120 mm Hg) before the procedure to a mean 27.6 ± 17.8 mm Hg (0–120 mm Hg) after the procedure. Procedure failed in two of the patients (3.6%) who received the RLVP; only two patients developed severe AI. Among those patients who underwent the standard method procedure failed in 6 patients (8.3%), whereas severe AI was seen in 15 (20.2%). RLVP decreased the incidence of postprocedure severe AI significantly (P .05). RLVP did not change the procedure duration, but decreased the duration of fluoroscopy significantly (P

Objectives: With the advancements in neonatal and pediatric intensive care services and technology, the number of children requiring long term mechanical ventilation support is increasing. Home mechanical ventilators enable discharge of patients with chronic respiratory failure from hospital and allows children to grow in natural environment. The objective of this study was to define clinical characteristics and follow up results of pediatric patients who are dependent on invasive home mechanical ventilation support. Methods: This retrospective study included a total of 61 patients discharged from pediatric intensive care unit while on mechanical ventilation (HMV). Results: The median age of patients was 8.5 (2 - 196) months with 60% of them being younger than 12 months. The decision for HMV was made after median 38 ± 37.2 days of mechanical ventilation in hospital. The most common reason of dependency on invasive HMV was found as central nervous system and neuromuscular diseases by 94%. The median duration of follow up was 11 months (4 days-7.5 years). HMV was no longer necessary in 12% of patients ventilated for neurological reasons and 50% for chronic pulmonary diseases. Anthropometric measures were observed to be improved in 69% of the patients. Mortality rate attributed to complications of tracheostomy was 15% and overall mortality was found as 46.7%. Conclusions: HMV enables ventilator dependent patients to be discharged and allows patients to develop and grow in their natural environment which helps them to catch up their growth potentials. The survival and quality of life (QoL) of these patients can be improved by family education and with the support by nutrition and care rehabilitation programs.

Amaç: Respiratuvar sinsityal virüs (RSV), bebeklik döneminde görülen bronşiolit ve pnömoninin en sık nedenidir. Prematürite, doğuştan kalp hastalığı, kronik akciğer hastalığı ya da immün yetmezlik gibi altta yatan hastalığı olanlarda RSV’ye bağlı komplikasyon ve ölüm riski yüksektir. Çalışmamızda hastaneye yatırılarak izlenen alt solunum yolu enfeksiyonlarında RSV sıklığını ve risk faktörlerini belirlemeyi amaçladık. Gereç ve Yöntem: Ekim 2012 –Mart 2013 tarihleri arasında alt solunum yolu enfeksiyonu nedeni ile hastanemizdeyatırılan 2 yaşın altındaki 257 olgu prospektif olarak değerlendirildi. Hastaların klinik ve laboratuvar bulguları incelendi, risk faktörleri belirlendi. Hastalardan nazofaringeal fırça ile nazofaringeal sürüntü örneği alınarak hızlı antijen testi ile RSV antijeni tarandı. Bulgular: Hastaların yaşları 1-24 ay (ort.6.67 ± 5.93 ay) arasında olup; %40’ı kız, %60’ı erkek idi. Hastalarda RSV sıklığı %38.1 olarak bulundu. RSV pozitif hastalarınen sık ocak ve şubat aylarında, yakınmaları başladıktan sonra ilk 5 günde başvurduğu gözlendi (p

UNLABELLED Cardiac rhabdomyoma is the most common primary cardiac tumor, is considered to be a hamartoma of developing cardiac myocytes. Cardiac rhabdomyoma is associated with tuberous sclerosis complex (TSC) in 50-86% of cases. Mutations in TSC-1/TSC-2 genes result in increased mammalian target of rapamycin (mTOR) pathway activation responsible for the hamartomatous lesions of tuberous sclerosis complex. Therapy with mTOR inhibitors is currently under investigation as a treatment option for tumors associated with TSC. In this report we present a case with multiple symptomatic rhabdomyomas associated with tuberous sclerosis complex, deemed to be ineligible for surgical removal, treated with everolimus (mTOR inhibitor). CONCLUSION As we observed in our patient, in cases with inoperable symptomatic rhabdomyomas associated with TSC, everolimus, an mTOR inhibitor, may be the treatment of choice, which should be confirmed with additional studies.

Numerous investigations have demonstrated that phototherapy (PT) directly or indirectly causes ductal patency by photorelaxation effect. In this observational study, we aimed to assess the effect of PT on the incidence of patent ductus arteriosus (PDA) together with prostaglandins (PGE2) and (PGI2) levels in preterm infants.Preterm infants whose gestational age34 weeks and who required PT in the first 3 d of life were enrolled in this prospective study. The clinical signs of PDA, the data of detailed echocardiographic study were recorded and plasma PGE2 and PGI2 levels were measured before and after PT. The outcome measures were the status of ductus arteriosus and alterations of PGE2 and PGI2 levels under the effect of PT.A total of 44 preterm infants were enrolled in the study, of these 21 (47.7%) were in Group 1 (Non-PDA Group) and 23 (52.3%) were in Group 2 (PDA Group). After PT, ductal reopening occurred in three infants (14.3%) in Group 1, while ductus closed in four infants in Group 2 (17.3%). PT does not seem to effect ductal patency for both groups (p=0.250 and p=0.125, respectively). PGE2 levels were not different before and after PT for both groups (p=0.087, p=0.408, respectively). However, PGI2 levels were significantly decreased after PT in both groups (p=0.006, and p=0.003, respectively).There was no effect of PT on ductal patency. We can conclude that PGs were eliminated simultaneously with ductal closure and photorelaxation effect did not influence PG levels.

Background Catheter cryoablation of supraventricular tachycardias involving the perinodal regions is considered to be a safer alternative compared to radiofrequency ablation. Limited information is available for efficacy, midterm outcomes, and complications regarding the ablation of parahissian accessory pathways (APs) in pediatric patients. Methods A retrospective review of all pediatric patients who underwent cryoablation for treatment of a parahissian AP was performed. Results Twenty-five patients (median age 13 years and weight 45.6 kg) underwent cryoablation of a parahissian AP. Median number of cryolesions applied was four (range: 3–6). Initial procedural success was achieved in 23 patients (23/25, 92%). Transient third-degree atrioventricular (AV) block was noted in two patients. There was no permanent AV block. Transient right bundle branch block (RBBB) was observed in one patient and permanent RBBB occurred in two patients. Of the patients successfully ablated with cryo, there was only one recurrence (1/23, 4.3%) over a follow-up of 17.5 months (range 6–34 months). Conclusion Cryoablation of parahissian APs is both safe and effective with a low risk of recurrence in pediatric patients.

Background This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). Methods Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients’ records were reviewed. Results The median age of the patients was 9.5 months, and 46 of the 88 (52.3%) patients were female. The most common concomitant CHD were ventricular septal defect, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF). When the patients were compared according to their CHD, persistent left SVC frequency was significantly higher in those with DORV (P

INTRODUCTION Systemic lupus erythematosus (SLE) is a multisystemic autoinflammatory disease that can involve any organ system; therefore, diagnosis can be challenging. Hereby, we present 4 cases that presented to pediatric emergency department with unusual clinical pictures of SLE. CASES Case 1 presented with inability to walk or talk for the last 1 week as well as intermittent pain and swelling in her joints. Case 2 presented with generalized edema and severe dyspnea. Case 3 and 4 presented to pediatric emergency department with rashes on the legs. DISCUSSION Systemic lupus erythematosus may mimic many clinical entities, and differential diagnosis may be difficult, especially if presentation is atypical. In every emergency physician, right diagnosis and prompt treatment are very important especially in life-threatening conditions such as cardiac involvement in SLE.

Heart transplantation indications in pediatric population include congenital heart diseases, cardiomyopathies and retransplants. Cardiomyopathy is the primary indication for 11 to 17 years of age. The surveillance after transplantation is a very important issue because of both the rejection risk and the adverse effects due to medications after transplantation. Immunosuppressive agents that are commonly used after heart transplantations have several toxicities. Here we present an adolescent patient diagnosed with dilated cardiomyopathy, performed heart transplantation, treated with tacrolimus and suffered from diabetic ketoacidosis due to tacrolimus. After the diagnosis was made the appropriate fluid and insulin therapy was started immediately and ketoacidosis resolved in the first 24 hours of the therapy. The diagnosis revised as new onset diabetes mellitus after transplantation and the tacrolimus dosage titrated to therapeutic level. After glycemic control the patient discharged with rapid acting insulin, three times daily, before meals; and long acting insulin once daily at night. In ten month follow up time the insulin dosages were progressively reduced.

Objective: Although sudden cardiac death is rare in children, an intracardiac defibrillator system is indicated in children with various types of cardiomyopathy, primary electrical diseases, and after surgical repair of congenital heart defects. The use of transvenous defibrillator lead systems is limited in pediatric patients because of a small body size and/or limited vascular access. Subcutaneous array leads combined with an abdominally placed generator can enable implantation. Method: This is a retrospective study of 13 patients who underwent subcutaneous defibrillator implantation between September 2010 and March 2015. The subcutaneous system was preferred because patients were not amenable to transvenous lead placement. Results: The median patient age was 4.1 years, and the median patient weight was 12.1 kg. Diagnoses of patients were long-QT syndrome in 6, aborted cardiac arrest with left ventricular non-compaction in 3, hypertrophic cardiomyopathy with sustained ventricular tachycardia in 3, and arrythmogenic right ventricular cardiomyopathy in 1. Revision of the subcutaneous lead was required in 5 patients 2–26 months after the implantation. Appropriate shocks were observed in three patients. Inappropriate shock and lead fractures were observed in one patient during the follow-up period. The failure of therapy was observed in one patient. There were no perioperative complications and no early or late deaths. Conclusion: Subcutaneous defibrillator systems are safe and effective in pediatric patients when the transvenous method is risky and contraindicated. Because the high growth rate in this population leads to lead failures, a close follow-up of this population is essential. (Anatol J Cardiol 2016; 16: 630-4)

Subkutanöz port kateter sistemleri uzun sureli vaya aralıklı infüzyon tedavi gereksinimi olan hastalarda giderek artan sıklıkta uygulanmaya başlamıştır. Farklı endikasyonlarla port sistemi kullanımının, aynı zamanda potansiyel olarak ciddi olabilecek birçok komplikasyona yol açtığı bildirilmiştir. Kateterin ayrılması ve embolizasyonu nadir görülen bir komplikasyon olmakla birlikte, tanısı konulduğunda kateterin çıkarılması gerekmektedir. Perkutanöz yaklaşım, minimal invavif, basit, güvenli ve konvansiyonel cerrahi seçeneklere gore daha düşük komplikasyon oranlarına sahip olduğundan altın standard tedavi yöntemi olarak kabul edilmektedir. Bu makalede tekrarlayan dirençli nöbetleri olması nedeniyle port kateteri takılan, takibinde kateterde ayrılma ve embolizasyon gelişmesi nedeniyle kateteri transvenöz yolla çıkarılan 2 yaşında bir kız hasta sunulmuştur., Subcutaneous port catheter systems are widely used with increasing frequency in patients requiring long-term or intermittant infusion therapy. Usage of port systems for a wide variety of indications also leads to well-documented wide spectrum of complications that can be potentially serious. Venous catheter dislodgement and migration is one of the rare complications of venous port implantation, however once diagnosed it must be removal of the catheter is indicated. Percutaneous approach for removal is considered a gold standard treatment because it is a minimally invasive, relatively simple, safe procedure, with low complication rates compared to conventional surgical treatment. In this report we present a 2-year-old girl who had a port catheter implanted because of recurrent intractable seizures and subsequently dislodgement and embolizing of entire catheter, which was removed with a snare-loop catheter via transvenous approach.

Background:: Tracheostomy was first observed in Egyptian drawings in 3600 BC and performed frequently during the 1800’s diphtheria epidemic. Objectives:: The aim of this study was to elucidate the indications, complications, mortality rate, and the effect of pediatric tracheostomy on length of PICU or hospital stay. Materials and Methods:: Demographic characteristics, diagnosis at admission, duration of ventilation of 152 patients were analyzed retrospectively. Results:: The most common tracheostomy indication was prolonged intubation. The mean duration of mechanical ventilation before tracheostomy was 23.8 days. Forty five percent of the tracheostomy procedures were performed at bedside. Neither the place nor the age had any effect on the development of complications (P = 0.701, P = 0.622). The procedure enabled 62% of the patients to be discharged from hospital. Conclusions:: Tracheostomy facilitates discharge and weaning of mechanical ventilation. Although the timing of tracheostomy has to be determined for each individual patient, three weeks of ventilation seems to be a suitable period for tracheostomy. Tracheostomy can be performed at bedside safely but patient selection should be made carefully.

Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive cardioauditory ion channel disorder characterized by congenital bilateral sensorineural deafness and long QT interval. JLNS is a ventricular repolarization abnormality and is caused by mutations in the KCNQ1 or KCNE1 gene. It has a high mortality rate in childhood due to ventricular tachyarrhythmias, episodes of torsade de pointes which may cause syncope or sudden cardiac death. Here, we present a 4.5-year-old female patient who had a history of syncope and congenital sensorineural deafness. She had a cochlear implant operation at 15 months of age and received an implantable cardioverter defibrillator (ICD) at 3 years of age because of recurrent syncope attacks. Five months after cochlear implant placement, she could say her first words and is now able to speak. With β-blocker therapy and ICD, she has remained syncope-free for a year. On the current admission, the family visited the genetics department to learn about the possibility of prenatal diagnosis of sensorineural deafness, as the mother was 9 weeks pregnant. A diagnosis of JLNS was established for the first time, and a homozygous missense mutation in the KCNQ1 gene (c.128 GA, p.R243H) was detected. Heterozygous mutations of KCNQ1 were identified in both parents, thereby allowing future prenatal diagnoses. The family obtained prenatal diagnosis for the current pregnancy, and fetal KCNQ1 analysis revealed the same homozygous mutation. The pregnancy was terminated at the 12th week of gestation. The case presented here is the third molecularly confirmed Turkish JLNS case; it emphasizes the importance of timely genetic diagnosis, which allows appropriate genetic counseling and prenatal diagnosis, as well as proper management of the condition.

Studies have shown that a prolonged P-wave dispersion is a risk factor for the development of atrial fibrillation. The aim of this study was to evaluate P-wave dispersion in adolescents with anorexia nervosa at diagnosis. We evaluated electrocardiographic findings, particularly the P-wave dispersion, at initial assessment in 47 adolescents with anorexia nervosa. Comparison of P-wave dispersion between adolescents with anorexia nervosa and controls showed a statistically significant higher P-wave dispersion in patients with anorexia nervosa (72 ± 16.3 msec) when compared to the control group (43.8 ± 9.5 msec). Percent of body weight lost, lower body mass index, and higher weight loss rate in the patients with anorexia nervosa had no effect on P-wave dispersion. Due to the fact that anorexia nervosa has a high mortality rate we believe that cardiac pathologies such as atrial fibrillation must also be considered in the medical evaluation.

Noonan sendromu farklı kardiyakanomalilerin de eşlik ettiği nadir görülen bir hastalıktır. Sunulan olgudaNoonan sendromlu bir hastada kardiyak anomalilerin daha önce bildirilmemiş birkombinasyonu bildirilmiştir. Eşlik eden kardiyak anomaliler atriyal septaldefekt, atriyal fibrilasyon ve ventrikül miyokardında noncompaction idi. Atriyalseptal defekt cerrahi olarak fenestre biçimde kapatılırken maze prosedürüuygulandı. Sendromlu hastalarda saptanan konjenital kalp defektlerinin cerrahitedavisinde sendromu olmayan hastalara göre farklı güçlükler ilekarşılaşılmaktadır. Bu nedenle sendromlu vakalarda özellikli bir yaklaşımihtiyacı vardır., Noonan syndrome is a raredisorder in which different cardiac abnormalities can exist. This reportdescribes the combination of cardiac abnormalities in a patient with Noonansyndrome, which has not been reported previously. Coexisting cardiacabnormalities were: atrial septal defect, atrial fibrillation and noncompactionof the ventricular myocardium. Surgical fenestrated closure of atrial septaldefect and concomitant maze procedure were performed. Surgery of congenitalheart defects in syndromic patients has different difficulties than those innonsyndromic patients. Thus, specific approach should be taken in thesepatients.