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Objective: Supraventricular tachycardia, one of the most common conditions requiring emergency cardiac intervention in newborns, is also the most common symptomatic tachyarrhythmia in newborns. Therefore, early diagnosis and treatment approach is important. Method: Demographic findings, clinical findings, and treatment approaches of newborns hospitalized with the diagnosis of supraventricular tachycardia between January 2011 and November 2020 in the Neonatal Intensive Care Unit of our hospital were evaluated retrospectively. Results: Thirty-eight patients without congenital heart disease (except secundum-type atrial septal defect) and diagnosed with supraventricular tachycardia were evaluated retrospectively. The mean week of gestation was 38.2 ± 1.8, the mean age at diagnosis was 10.7 ± 10.1 days, the number of patients with heart failure was 8 (21%), the number of patients with Wolff-Parkinson White syndrome was 8 (21%), the number of patients with fetal arrhythmia was 6 (15.7%) and the mean number of hospitalization days was 15.7±13.5. The patients were given adenosine in the first stage and propranolol, amiodarone, propafenone and flecainide in the second stage as medical treatment. Cardioversion was performed in 5 (13%) patients due to resistant supraventricular tachycardia. Conclusion: Early diagnosis and treatment of supraventricular tachycardia are very important in terms of reducing morbidity and mortality. Therefore, we think that increasing awareness of supraventricular tachycardia among clinicians following newborn babies will enable newborns with supraventricular tachycardia to receive early diagnosis and treatment., Objective: Supraventricular tachycardia, one of the most common conditions requiring emergency cardiac intervention in newborns, is also the most common symptomatic tachyarrhythmia in newborns. Therefore, early diagnosis and treatment approach is important.Method: Demographic findings, clinical findings, and treatment approaches of newborns hospitalized with the diagnosis of supraventricular tachycardia between January 2011 and November 2020 in the Neonatal Intensive Care Unit of our hospital were evaluated retrospectively.Results: Thirty-eight patients without congenital heart disease (except secundum-type atrial septal defect) and diagnosed with supraventricular tachycardia were evaluated retrospectively. The mean week of gestation was 38.2 ± 1.8, the mean age at diagnosis was 10.7 ± 10.1 days, the number of patients with heart failure was 8 (21%), the number of patients with Wolff-Parkinson White syndrome was 8 (21%), the number of patients with fetal arrhythmia was 6 (15.7%) and the mean number of hospitalization days was 15.7±13.5. The patients were given adenosine in the first stage and propranolol, amiodarone, propafenone and flecainide in the second stage as medical treatment. Cardioversion was performed in 5 (13%) patients due to resistant supraventricular tachycardia.Conclusion: Early diagnosis and treatment of supraventricular tachycardia are very important in terms of reducing morbidity and mortality. Therefore, we think that increasing awareness of supraventricular tachycardia among clinicians following newborn babies will enable newborns with supraventricular tachycardia to receive early diagnosis and treatment.

Background Hypertrophic cardiomyopathy (HCM) is the second most common pediatric cardiomyopathy. Although there is a large body of literature about HCM in adults, there is limited information on HCM in childhood. We evaluated various aspects of pediatric HCM patients treated at our center. Methods We identified 152 pediatric patients with HCM between October 2011 and October 2019. Clinical history, invasive (ICD, pacemaker, electrophysiologic study, catheter ablation therapy) and non-invasive (ECG, holter moniterization, echocardiography, cardiac MR, genetic study, medicam treatment) data were collected and evaluated. Results The mean ± standard deviation age of patients was 8.9 ± 5.7 years (1 month-18 years) and 67.8% were male. The most frequent clinical symptoms were murmur and palpitations. Three cases (2%) had aborted sudden death as the first manifestation of HCM. Of these patients, 120 (78.9%) had non-syndromic HCM and 32 (27.2%) had syndromic HCM. Asymmetric septal hypertrophy was common (48.3%) in the non-syndromic group, whereas concentric hypertrophy was common (56.2%) in syndromic group. Left ventricular outflow tract obstruction (LVOTO) occurred in 39 (25.6%) patients. Nine (5.9%) patients underwent electrophysiologic study and/or ablation and 16 patients underwent surgical intervention. Implantable cardioverter defibrillator (ICD) insertion was performed in 38 patients (26 transvenous, 12 epicardial). ICDs were inserted in three (7.9%) patients for secondary prevention; in the remaining patients (92.1%) the devices were placed for primary prevention. Mean SD follow-up time was 27.1 ± 22 months. Five (3.3%) patients died during the follow-up. No patient had heart transplantation or a long-term assistive device. Conclusion The etiology of HCM is heterogeneous and present at any age. It is important to determine the timing of surgery and potential risks for sudden cardiac arrest. As most cases of HCM are familial, evaluation of family members at risk should be a routine component of clinical management.

Objective: The aim of this study is to present electrophysiologic characteristics and catheter ablation results of tachycardia-induced cardiomyopathy (TIC) in children with structurally normal heart. Methods: We performed a single-center retrospective review of all pediatric patients with TIC, who underwent an electrophysiology study and ablation procedure in our clinic between November 2013 and January 2019. Results: A total of 26 patients, 24 patients with single tachyarrhythmia substrates and two patients each with two tachyarrhythmia substrates, resulting with a total of 28 tachyarrhythmia substrates, underwent ablation for TIC. The median age was 60 months (2–214 months). Final diagnoses were supraventricular tachycardia (SVT) in 24 patients and ventricular tachycardia (VT) in two patients. The most common SVT mechanisms were focal atrial tachycardia (31%), atrioventricular reentrant tachycardia (27%), and permanent junctional reciprocating tachycardia (15%). Radiofrequency ablation (RFA) was performed in 15 tachyarrhythmia substrates, and cryoablation was performed in 13 tachyarrhythmia substrates, as the initial ablation method. Acute success in ablation was achieved in 24 out of 26 patients (92%). Tachycardia recurrence was observed in two patients (8%) on follow-up, who were treated successfully with repeated RFA later on. Overall success rates were 92% (24 out of 26) in patients and 93% (26 out of 28) in substrates. On echocardiography controls, the median left ventricular recovery time was 3 months (1–24 months), and median reversible remodeling time was 6 months (3–36 months). Conclusion: TIC should be kept in mind during differential diagnosis of dilated cardiomyopathy. Pediatric TIC patients can be treated successfully and safely with RFA or cryoablation. With an early diagnosis of TIC and quick restoration of the normal sinus rythm, left ventricular recovery, and remodeling may be facilitated. (Anatol J Cardiol 2020; 24: 370-6)

Introduction:Congenital atrioventricular block is diagnosed in uterine life, at birth, or early in life. Atrioventricular blocks can be life threatening immediately at birth so urgent pacemaker implantation techniques are requested. Reasons can be cardiac or non-cardiac, but regardless of the reason, operations are challenging. We aimed to present technical procedure and operative results of pacemaker implantation in neonates.Materials and methods:Between June 2014 and February 2021, 10 neonates who had congenital atrioventricular block underwent surgical operation to implant permanent epicardial pacemaker by using minimally invasive technique. Six of the patients were female and four of them were male. Mean age was 4.3 days (0–11), while three of them were operated on the day of birth. Mean weight was 2533 g (1200–3300).Results:Operations were achieved through subxiphoidal minimally skin incision. Epicardial 25 mm length dual leads were implanted on right ventricular surface and generators were fixed on the right (seven patients) or left (three patients) diaphragmatic surface by incising pleura. There were no complication, morbidity, and mortality related to surgery.Conclusion:Few studies have characterised the surgical outcomes following epicardial permanent pacemaker implantation in neonates. The surgical approach is attractive and compelling among professionals so we aimed to present the techniques and results in patients who required permanent pacemaker implantation in the first month of life.

Congenital atrioventricular block is diagnosed in uterine life, at birth, or early in life. Atrioventricular blocks can be life threatening immediately at birth so urgent pacemaker implantation techniques are requested. Reasons can be cardiac or non-cardiac, but regardless of the reason, operations are challenging. We aimed to present technical procedure and operative results of pacemaker implantation in neonates.Between June 2014 and February 2021, 10 neonates who had congenital atrioventricular block underwent surgical operation to implant permanent epicardial pacemaker by using minimally invasive technique. Six of the patients were female and four of them were male. Mean age was 4.3 days (0-11), while three of them were operated on the day of birth. Mean weight was 2533 g (1200-3300).Operations were achieved through subxiphoidal minimally skin incision. Epicardial 25 mm length dual leads were implanted on right ventricular surface and generators were fixed on the right (seven patients) or left (three patients) diaphragmatic surface by incising pleura. There were no complication, morbidity, and mortality related to surgery.Few studies have characterised the surgical outcomes following epicardial permanent pacemaker implantation in neonates. The surgical approach is attractive and compelling among professionals so we aimed to present the techniques and results in patients who required permanent pacemaker implantation in the first month of life.

Congenital Long QT Syndrome (LQTS) is a dangerous arrhythmic disorder that can be diagnosed in children with bradycardia. It is characterised by a prolonged QT interval and torsades de pointes that may cause sudden death. Long QT syndrome is an ion channelopathy with complex molecular and physiological infrastructure. Unlike the acquired type, congenital LQTS has a genetic inheritance and it may be diagnosed by syncope, stress in activity, cardiac dysfunction, sudden death or sometimes incidentally. Permanent pacemaker implantation is required for LQTS with resistant bradycardia even in children to resolve symptoms and avoid sudden death.

Objective We report a single-institute experience of neonatal transvenous radiofrequency pulmonary valve perforation for pulmonary atresia/intact ventricular septum, with echocardiographic predictors of biventricular circulation. Methods Data were reviewed retrospectively for all neonates who underwent primary transvenous pulmonary valve perforation for pulmonary atresia/intact ventricular septum between January 2008 and November 2018 at our institution. We compared patients who need systemic-to-pulmonary shunt or ductal stenting with patients who did not need. Results During the study period, 31 patients with pulmonary atresia/intact ventricular septum underwent successful radiofrequency pulmonary valve perforation and balloon dilation of the pulmonary valve. There was no procedure-related mortality. Sixteen patients (52%) needed systemic-to-pulmonary shunt or ductal stenting after initial procedure. Among the survivors (follow-up time of 1 to 11.5 years), 15 patients had a biventricular circulation and 6 patients had 1 and 1 forward slash 2 ventricular circulation. Two patients are awaiting for Fontan operation. Both the TV/MV annulus ratio (>0.85) and tricuspid valve z-score (>-1) were found to be a good predictor of a biventricular outcome in our cohort. Conclusions Percutaneous radiofrequency pulmonary valve perforation and balloon valvotomy is an effective and safe primary treatment strategy for neonates with pulmonary atresia/intact ventricular septum. Ductal stenting or systemic-to-pulmonary shunt may be required in the majority of patients who had smaller right heart components. Preselection of patients according to tricuspid valve z-score and TV/MV annulus ratio allows predicting biventricular circulation.

Background Tricuspid annular plane systolic excursion (TAPSE) and mitral annular plane systolic excursion (MAPSE) are two echocardiographic parameters which provide reliable information about the longitudinal myocardial functions of the right and left ventricles in both adult and pediatric population. The aim of this study was to determine the TAPSE and MAPSE reference values in healthy children aged 0-18 years. Methods and results This prospective study included 1300 healthy children evaluated with two-dimensional echocardiography. In addition to routine echocardiographic examination, the right and left ventricular systolic functions were assessed by TAPSE and MAPSE measurements. Statistical analyses were carried out in the groups of subjects stratified according to age and body surface area. The mean and standard deviation values and z-scores of TAPSE and MAPSE were developed in each group. Conclusions The determination of reference values for TAPSE and MAPSE in healthy children will be of guidance in the evaluation of both healthy children and those with congenital or acquired heart diseases in which the right and left ventricular systolic functions are affected. The reference values obtained will contribute to the clinical practice and the future studies.

Aortic cusps might be the source of supraventricular or ventricular arrhythmias. For many years, aortic cusp ablation has been widely used to treat premature ventricular contractions (PVCs) and ventricular tachycardia (VT). However, the data on the outcomes of this procedure in children are limited. The study aimed to convey or describe our own aortic cusp ablation experiences in children and, thus, contribute to the literature. The focus was pediatric cases of ventricular arrhythmia in which the origin of the PVCs was ablated above the Valsalva. The sample comprised patients who underwent aortic cusp ablation between 2013 and 2018. The demographic characteristics, noninvasive test results, procedure details, and follow-up results for the patients were noted. The 3D EnSite Precision cardiac mapping system and limited fluoroscopy were used. A total of 26 procedures were performed on 22 patients. The mean age was 14.4 ± 3.0 (9–19) years, and the mean weight was 57.3 ± 17.5 (27–99) kg. The mean follow-up period after the first presentation was 38.6 ± 22.9 (3–72) months. There were significant differences in the values of the transition index, V2S/V3R, IIR/IIIR, aVRS/aVLS ratio, and QRS polarity in I at various locations. The most common ablation site was the left coronary cusp (LCC). Radio frequency (RF) ablation, cryoablation, and irrigated RF ablation were found to be effective energy sources in 15, 4, and 3 patients, respectively. Patients who underwent ablation at the LCC–right coronary cusp (RCC) commissure were more likely to present with only VT and to experience worsening symptoms. Twelve patients had previously undergone ablation of the right ventricular outflow tract (RVOT). Ablation in the RVOT had been previously performed in all the patients who eventually underwent ablation at the RCC and the LCC–RCC commissure. Aortic cusp ablation can be safely performed in children. The careful evaluation of previous noninvasive tests provides important data for determining the location. There might be significant differences in the signs and requirements on the basis of the locations during mapping and ablation.

Junctional ectopic tachycardia (JET) is a rare form of arrhythmia that is most commonly seen during infancy. JET is continuous and incessant, characterized by persistently high heart rates that may result in impaired cardiac function and tachycardia-induced cardiomyopathy. Despite the availability of multiple antiarrhythmic treatments, including flecainide and amiodarone, management of JET is generally very difficult. Catheter ablation has a high risk of atrioventricular block and it may require the placement of a pacemaker. Ivabradine, also known as a cardiac pacemaker cell inhibitor, is a new-generation antiarrhythmic used to treat sinus tachycardia and angina pectoris in adult patients. In this article, we present three cases of subjects with infantile congenital JET who were admitted to our clinic with a tachycardia-induced cardiomyopathy. The age of the subjects ranged from 52 days to 10 months. Although the cases of tachycardia could not be controlled by multiple antiarrhythmics, including a combination of amiodarone and flecainide combined with either propranolol or digoxin, they were rapidly converted into sinus rhythm with an ivabradine treatment of 0.1-0.2 mg/kg/day. No cardiac or other side effects were observed during ivabradine treatment, and left ventricular functions and rhythms improved within 24 hours. These three cases therefore provide hope that ivabradine may be a suitable standard initial treatment for congenital JET. However, additional research is needed to confirm the validity of these results in other circumstances.

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

Objective Cardiac dysfunction is a well-known consequence of diabetes mellitus. This study was designed to assess whether type 1 diabetic children and adolescents with good metabolic control have early echocardiographic signs of subclinical left ventricular dysfunction and whether diabetes duration has any influence, using conventional and nonconventional echocardiographic tools. Methods A total of 100 patients with type 1 diabetes mellitus and 80 gender- and age-matched healthy controls were included. The cases underwent standard conventional transthoracic echocardiography, tissue Doppler imaging, and two-dimensional speckle tracking echocardiography. None of the diabetic patients had signs of renal, retinal, or neurological complications of the disease, and all were good metabolic control (mean HbA1c Results There was no difference among groups in relation to age, sex, body mass index, and blood pressure. Conventional echocardiographic parameters were similar between diabetic and nondiabetic subjects except increased mitral valve peak A-wave and significantly lower mitral E/A ratio in diabetics. Diabetic patients had more advanced diastolic dysfunction with TDI analysis. In the diabetic group, left ventricular global longitudinal, circumferential, and radial strain and strain rate were significantly lower compared with the controls. There was a positive correlation between diabetes duration and cardiac dysfunction. Conclusion The results of this study showed that the diabetic children and adolescents with good metabolic control had diastolic dysfunction when assessed with either conventional or tissue Doppler echocardiography. Also diabetic patients had subclinical LV systolic dysfunction with a normal LVEF which can be detected with 2D speckle tracking echocardiography.

Amaç: Yenidoğanlarda kardiyak aritminin bütün şekilleri görülebileceği gibi, en sık görülen aritmi tipi supraventriküler taşikardilerdir (SVT). Bu makalede henüz ortak bir yaklaşım olmayan bu taşiaritmi tipinde güncel tedavi seçeneklerinin tartışılması amaçlanmıştır. Olgular: Hastalarımızda ortalama SVT gelişme yaşı 7,4 gün ve kalp tepe atımı (KTA) 234/dk idi. Tüm olgular medikal tedaviye cevap vermiş olup elektrofizyolojik çalışma gerekmemiştir. Serimizde, tüm olgularda SVT atağında tedavide ilk seçenek olarak adenozin uygulanmış olup tekrarlayan SVT atakları için ek medikal tedavilere gereksinim doğmuştur. Olgularımızdan altı (%85,7) hastaya yineleyen SVT atakları nedeniyle propranolol tedavisi gerekmiştir. Adenozine yanıtsız SVT gelişen bir olgumuzda amiodaron infüzyonunun tedricen azaltılması sırasında propranolol tedavisine ek olarak flekainid eklenmiştir. Tedavi sonrası SVT atağı gözlenmemiştir. Sonuç: Yenidoğanda uzun süre devam eden SVT ataklarının kalp yetmezliği ve multiorgan disfonksiyonuna sebep olabileceği göz önüne alınarak tedavide kullanılan farklı ilaçlar servislerde hazır bulundurulmalı ve vakalar yoğun bakım şartlarında multidisipliner bir yaklaşımla izlenmelidir.

In children with typical atrioventricular nodal reentrant tachycardia (tAVNRT), cryoablation is preferred over radiofrequency ablation (RFA) because of its safety profile and acceptable long-term success rates. In this study, we have assessed the utility of 8-mm tip cryocatheters for tAVNRT ablation in our center. All pediatric AVNRT patients who underwent cryoablation with an 8-mm tip cryocatheter in our center between 2013 and 2018 were included. EnSite™ (St. Jude Medical Inc., St. Paul, MN, USA) was used in all patients. A total of 120 patients (64 females, 53%) were included in this study, and the mean age was 13.9 years with a standard deviation of 2.5 years. Eleven patients (9.1%) had structural heart disease, and 12 patients (10%) had additional arrhythmia substrate. The mean number of effective cryolesions was 8 with a standard deviation of 2.3. Fluoroscopy was used in three patients (2.5%). There were minor complications in only four patients (3.3%)—transient first-degree atrioventricular block or transient incomplete right bundle branch block. Acute success rate of cryoablation was 108/120 (90%). In twelve patients, cryoablation was suboptimal, or it failed. The procedure was completed successfully with RFA in the same session in ten patients. Overall acute success rate of ablation (Cryo ± RFA) was 98.5%. During a mean follow-up period of 24.6 months with a standard deviation of 11.3 months, three patients had recurrence (2.5%). Time between the beginning of the effective cryolesion and termination of AVNRT was found associated with acute success of cryoablation (p = 0.013). Cryoablation of AVNRT with an 8-mm tip catheter in children appears to be safe, with an acceptable acute success rate and a low recurrence rate. A faster termination of AVNRT during the cryolesion, slowing down before ending with atrioventricular block, is a good indicator for acute success

Objective:Anthracycline chemotherapeutic agents carry the well-recognised risk of cardiac toxicity. The aim of this study was to determine the long-term effect of anthracycline chemotherapy on the biventricular function in childhood cancer survivors using tissue Doppler imaging and two-dimensional speckle tracking echocardiography.Patients and Methods:The study included 45 survivors of childhood cancers and 50 healthy age-matched control patients. Cardiac function was prospectively studied with conventional echocardiography, tissue Doppler imaging, and speckle tracking echocardiography after completion of treatment. The same analysis was performed on matched controls.Results:There was no difference in age, gender, height, and weight between the study and control groups. The mean anthracycline dose was 240 ± 106 mg/m2 and the mean remission duration was 8.2 ± 5 years (1–20 years) in the study group. Conventional echocardiography showed similar ejection fraction, shortening fraction, and left ventricle end-diastolic diameter in both groups. Mitral lateral and septal tissue Doppler imaging showed normal but according to control group relatively sub-normal systolic and diastolic function in patient group. The global longitudinal and circumferential strain and strain rates were significantly lower in the patient group compared to control group. Correlation analysis revealed a negative and significant correlation between total anthracycline dose and global longitudinal and circumferential strain and strain rates.Conclusion:Sub-clinical systolic and diastolic dysfunction may not be detected by conventional echocardiographic methods which are frequently used in daily practice. Sub-clinical systolic and diastolic dysfunction may be detected more sensitively by echocardiographic method such as speckle tracking echocardiography in childhood cancer survivors.

Objective Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial genetic disease that occurs primarily in the right ventricle. Patients with ARVD may present with severe ventricular arrhythmias, syncope, and cardiac arrest. The purpose of this study is to evaluate the clinical features and arrhythmic complications of patients with pediatric-onset ARVD. Methods Patients diagnosed with ARVD between January 2010 and January 2019 were included in this study. Results A total of 19 patients with ARVD were evaluated. Of them, 15 patients were male, and their mean age was 12±4 years. The most common symptoms were palpitations (n=6), syncope (n=4), and heart failure symptoms (n=2). Five patients were asymptomatic. Thirteen patients had an epsilon wave; all patients ≥14 years had a T wave inversion in V1-3. Premature ventricular contractions (PVCs) were observed in 15 patients, and ventricular tachycardia (VT) was observed in 9 patients. All patients underwent cardiac magnetic resonance imaging (MRI). Echocardiography and cardiac MRI of two patients were normal at the time of admission; patients were in the concealed phase, and the diagnosis was made by ECG, Holter monitoring, and genetic findings. We administered a beta-blocker in all patients. Two patients underwent an electrophysiological study and ablation because of PVC/VT. An implantable cardiac defibrillator was implanted in 8 patients. The mean follow-up period was 21.5±11 months. Two patients were deceased with incessant VT and heart failure, and one patient was deceased with multiorgan dysfunction after biventricular assist device implantation (n=3). Conclusion Diagnosis of pediatric-onset ARVD might be much more difficult in children. Sudden cardiac death might be prevented in the early period by raising the awareness of physicians about the disorder. Prevention of sudden death with implantable cardiac defibrillators is crucial in the management of these patients. It should be kept in mind that children with structurally normal hearts may present with an earlier concealed phase and can be diagnosed with ARVD.

Catheter ablation via the femoral vein has been widely used in children. However, in certain conditions, an alternative vascular access is required for a successful ablation. Herein we reported that, accessory pathways (APs) and ectopic foci which reside right anterior and anterolateral to tricuspid valve orifice can be safely and effectively ablated with transjugular venous approach. Eleven procedures performed via the transjugular venous approach were reviewed retrospectively from the 355 electrophysiological procedures performed between March 2016 and November 2017. EnSite 3D electro-anatomic mapping and limited flouroscopy was used in all patients. The mean age of patients was 14.4 ± 2.9 years (12–18) and 6/11 (54.5%) were males. Seven of the patients had previous ablation procedures via the femoral vein approach and due to failure or recurrence the procedures had to be repeated. In these patients mean procedure time shortened from 196 ± 80 (105–280) to 111 ± 13 (96–125) min with the transjugular approach. 10/11 patients had APs, and one patient had focal atrial tachycardia. One of the APs was a Mahaim pathway. Ablation localisations were right anterior and right anterolateral in 8/11 and 3/11 patients respectively. The average fluoroscopy time was 3.38 ± 6.5 (0–15) min. All of the RF ablations via transjugular approach were successful (100%, 11/11). The mean follow-up period was 10.6 ± 1.1 (9–12) months. So far no recurrence has been observed. Right anterior and anterolateral regions are the most challenging areas in terms of catheter stabilization even when long sheaths are used during femoral route ablation. Ablation attempts in these regions have partial success, frequent recurrence, and high complication rates. This study revealed that transjugular approach seems as an effective alternative for those cases where transfemoral approach is unsuccessful for the ablation targets located in the right anterior and antero-lateral regions.

Background: The aim of this study was to determine the incidence of acute rheumatic fever (ARF) in Turkish children. Material and Methods: Our data was collected from the population and hospital based studies in Turkey between 1981 and 2012. We examined 12 studies reported from Turkey between these dates. Results: Between 1981-1990, 1991-2000, 2001-2012; 573, 1677, 1688 patients had been followed up, respectively. In these three periods of follow up time carditis was observed in (51, 62 and 71% of patients), arthritis in (67, 68, 56% of patients), Sydenham's chorea in (13, 13 and 13% of patients) and erythema marginatum in (2, 0.1, 1% of patients), respectively. Between 1981-1990, 1991-2000 and 2001-2012 the incidence of ARF was detected 1.1/100.000, 2.6/100.000 and 2.3/100.000, respectively. Conclusion: While ARF incidence in Turkey had remained at a constant rate until 1980, a rapid decline occurred after this time, and this decline remained almost unchanged until 2012.

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.

Congenital multiple obstructive lesions of the left heart Objective: To investigate the localization, type, associations, other accompanying abnormalities and treatment methods. Material and Methods: The study included 24 cases presenting to the Pediatric Cardiology Unit of our Hospital with more than one left heart lesion, evaluated with two-dimensional color Doppler echocardiography (Vivid 7 Pro); 18 underwent cardiac catheterization and angiography. Complaints at admission, lesion site, type and associations, additional abnormalities and treatment were evaluated. Results: Multiple obstructive left heart lesions were more frequent (79%) in males, at an average age of 6.3. Most frequent symptoms at admission were dyspnea, fatigability and cyanosis. The most frequent associations of obstructive abnormalities were: mitral and aortic stenosis in 29%, mitral stenosis with aortic coarctation in 17% and aortic valve stenosis with aortic coarctation in 37%. Mitral stenosis associated to both aortic valve stenosis and aortic coarctation was seen in 17%. The most frequent associated non-obstructive abnormality was a bicuspid aortic valve (62%). Following diagnosis, 5 of the patients did not show up for follow-up, while the decision for one patient was observation. The remaining 18 cases underwent cardiac catheterization and angiography. Six patients had successful coarctation angioplasty and one angioplasty plus aortic valvuloplasty. We lost 2 of the patients who had undergone coarctation angioplasty. Conclusion: It was observed that the lesion most significantly contributing to mortality due to congenital obstructive lesions of left heart outflow are mitral valve obstruction and pulmonary hypertension, and that early invasive intervention is life-saving.

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

Rhabdomyomas are the most common primary cardiac tumors, especially seen during early periods of childhood. Fetaltype rhabdomyoma is a benign tumor described almost always in extracardiac locations. Although the natural history of the cardiac rhabdomyoma is to regress, the behaviour of the fetal-type rhabdomyomas when present in the heart is unknown with respect to its infrequency. Herein, we report a hemodynamically unstable female neonate with a single large intra-cardiac mass unresponsive to medical treatment, who underwent surgery. The neonate could not survive the operation due to ventricular fibrillation. The mass was diagnosed as fetal-type cardiac rhabdomyoma on autopsy.

Amac: Bu calismada aort koarktasyonlu yenidoganlarda balon anjiyoplasti ile ilgili klinik deneyimlerimiz, mevcut verilerimiz ve kisa ve orta donem sonuclarimiz incelendi. Ca­lis­ma­ pla­ni:­Aralik 2004 Mart 2010 tarihleri arasinda aort koarktasyonu nedeniyle balon anjiyoplasti uygulanan 51 yenidogana (41 erkek, 10 kiz; ort. yas 13±9 gun; dagilim 1-30 gun) ait veriler retrospektif olarak incelendi. Bul gu lar: Olgularin 13’unde (%25) izole koarktasyon, 38’inde (%75) kompleks koarktasyon tespit edildi. On sekiz hastada (%35.3) sol ventrikul disfonksiyonu, 17 hastada (%33.3) isthmus hipoplazisi goruldu. En sik eslik eden anomali, ventrikuler septal defekt (VSD) (%49) idi. Yirmi dokuz hastada (%56.9) pulmoner hipertansiyon vardi. Koarktasyon bolgesinde ortalama sistolik basinc gradiyenti dilatasyon oncesi 36±20 mmHg iken, dilatasyon sonrasi 8.6±7.0 mmHg’ye dustu. Bir hastada femoral arter trombozu gelisti. Ortalama izlem suresi 8.7±9.6 ay (dagilim 1-46 ay, ortanca 6 ay) idi. Islem sonrasi ortalama 3.2±3.1 ay sonra 20 hastada (%39.2) rekoarktasyon gelisti. Rekoarktasyon gelisen hastalarin dokuzuna (%45) tekrar anjiyoplasti, 11’ine (%55) cerrahi islem uygulandi. Bir-yedi aylik izlemde yedi hasta (%13.7) kaybedildi. So­nuc:­Kisa ve orta izlem sonuclarina gore balon anjiyoplasti, cerrahi isleme gore yuksek rekoarktasyon oranina sahiptir. Bu nedenle balon anjiyoplasti, kompleks kardiyak hastaligi olan ve genel durumu kotu olan hastalarda tam duzeltme ameliyati yapilana dek sagkalim icin uygulanmalidir. Anah tar soz cuk ler: Aort koarktasyonu; balon anjiyoplasti; yenidogan. Background:­In this study, we reviewed our clinical experience along with the existing data and shortand midterm results of balloon angioplasty for coarctation of aorta in neonates. Methods: The data of 51 neonates (41 boys and 10 girls; mean age 13±9 days; range 1 to 30 days) who underwent balloon angioplasty for aortic coarctation between December 2004 and March 2010 were retrospectively analyzed. Results:­Isolated coarctation was found in 13 patients (25%) and complex coarctation in 38 (75%). The left ventricular dysfunction was seen in 18 patients (35.3%), while 17 patients (33.3%) had isthmus hypoplasia. The most common concomitant abnormality was ventricular septal defect (VSD) (49%). Twenty-nine patients (56.9%) had pulmonary hypertension. The mean systolic pressure gradient across the coarctation site fell from 36±20 mmHg before dilatation to 8.6±7.0 mmHg following the intervention. One patient developed femoral artery thrombosis. The mean follow-up was 8.7±9.6 months (range 1-46, median 6 months). Recoarctation developed in 20 patients (39.2%) after an average 3.2±3.1 months. Of these, nine (45%) with recoarctation underwent repeated angioplasty and 11 (55%) surgical repair. During follow-up of 1-7 months, seven (13.7%) patients died. Conclusion:­ Our shortand midterm results suggest that balloon angioplasty has a higher recoarctation rate than surgery. Therefore, balloon angioplasty should be done to ensure survival until full corrective surgery is performed in patients with complex cardiac disease and poor overall condition.

Ergul Y, Ozturk E, Ozgur 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. Tachyarrhythmias are common cardiac arrhythmias in newborns and infants. We present a premature case with a 2.4-kg birth weight, with non-immune hydrops due to supraventricular tachycardia. Despite the combination of amiodarone, esmolol and flecainide, tachyarrhythmia could not be controlled and radiofrequency ablation was applied due to left ventricular dysfunction. After the procedure the rhythm returned to normal sinus and left ventricular functions improved rapidly. The follow-up of the case continues without any problems. In this presentation, successful ablation of the accessory pathway associated with structural heart disease have been covered in the youngest and smallest patient to date.

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.

WOS: 000350022000007, PubMed ID: 25250785, …

Background/aim: This study aimed to describe the spectrum and frequency of cardiovascular abnormalities in pediatric and young adult patients with Turner syndrome (TS) using cardiac MRI and MR angiography. Materials and methods: This prospective study consisted of 47 female patients of pediatric age and young adults with a karyotypically confirmed diagnosis of TS. All patients underwent cardiac MRI and contrast-enhanced MR angiography. A second examination after 9-26 months was performed for 28 of these patients. Results: Elongation of the transverse aortic arch (ETA) was the most frequent abnormality with a rate of 37%. The rate of partial anomalous pulmonary venous connection (PAPVC) was 21.7%, bicuspid aortic valve (BAV) was 19.6%, coarctation was 6.5%, ascending aorta dilatation was 28.3%, and descending aorta dilatation was 15.2%. The diameters of the aorta and the rate of aortic dilatation per unit of time was greater in the patients with BAV (P < 0.05). ETA was less observed in the patients who were receiving growth hormone therapy (P < 0.05). Conclusion: The most common cardiovascular abnormalities in TS patients are aortic arch anomalies such as ETA and coarctation, aortic dilatation, PAPVCs, and BAV. The presence of BAV is an important risk factor for the aortic dilatation.

Objectives: The aim of our study is to investigate the frequency of structural heart diseases in patients with Marfan syndrome (MS) and to reveal the importance of clinical follow-up in MS. Materials and methods: Study population consisted of 17 patients admitted to the Pediatric Cardiology department between January 2005 and March 2010 with the diagnosis of MS according to the Ghent criteria. Patients were evaluated for the eye, genetic and the cardiovascular system abnormalities. Physical examination findings, echocardiographic, and radiological examinations of the patients were evaluated retrospectively. Results: Of the 17 cases, 9 were girls and 8 were males, ages ranged from 1 month to 17 years (mean 9.7 years). There was a second degree of kinship between mothers and fathers in 5 patients. Respiratory distress, syncope, chest pain and palpitation were the most seen in the presentation complaint of the patients. Skeletal findings observed in 13 patients, 4 patients had subluxation of the lens. Two patients had positive family history. When cardiovascular findings were examined, there were aortic root dilatation with mitral valve prolapse and/or tricuspid valve prolapse in 8 patients, mitral valve prolapse in 3 patients only aortic root dilatation in 3 patients and mitral valve prolapse and tricuspid valve prolapse in 3 patients. Mean follow-up period was 2.6 years, aneurysm and rupture of the aorta wasn\'t observed during this period. Conclusion: In patients with Marfan syndrome, regular follow-up and cardiological evaluation should be done because significant structural heart diseases can be seen in these patients. J Clin Exp Invest 2012; 3(2): 199-201, Amaç: Bu çalışmamızın amacı, Marfan sendromlu (MS) olgularda yapısal kalp hastalıklarının sıklığını araştırmak ve klinik izlemin önemini ortaya koymaktır. Gereç ve yöntem: Ocak 2005-Mart 2010 tarihleri arasında Çocuk Kardiyolojisi bölümüne başvuran ve Ghent kriterlerine göre MS tanısı konan 17 hasta çalışmaya alındı. Hastalar kardiovasküler sistem muayenelerinin yanı sıra göz ve genetik bakımından da değerlendirildi. Hastaların fizik inceleme bulguları, ekokardiyografik ve radyolojik incelemeleri geriye dönük olarak irdelendi. Bulgular: Çalışmaya alınan 17 olgunun 9'u kız, 8'i erkek olup, yaşları 1 ay-17 yıl (ort. 9.7 yıl) arasında değişmekte idi. Hastaların 5'inde anne ve baba arasında 2. derecede akrabalık vardı. Hastaların başvuru yakınmaları arasında en fazla solunum sıkıntısı, bayılma, göğüs ağrısı ve çarpıntı görüldü. Hastaların 13'ünde iskelet sistemine ait bulgular görülürken, 4 hastada lens subluksasyonu saptandı. İki hastada aile anamnezi pozitifti. Kardiyovasküler bulgular incelendiğinde, hastaların 8'inde aort kökü dilatasyonu ile birlikte mitral valv prolapsusu ve/veya triküspit valv prolapsusu, 3'ünde mitral valv prolapsusu, 3'ünde sadece aort kökü dilatasyonu, 3'ünde mitral valv prolapsusu ve triküspit valv prolapsusu vardı. İzlem süresi ort. 2.6 yıl olup bu süre içinde aorta anevrizması ve rüptürü gözlenmedi. Sonuç: Marfan sendromlu hastalarda önemli yapısal kalp hastalıklarının görülmesi nedeniyle kardiyolojik olarak değerlendirilmesi ve düzenli izlemleri yapılmalıdır.

Q fever is a zoonotic infection caused by Coxiella Burnetii Although it is usually asymptomatic acute form of disease may change from flu like illness to severe pneumonia Chronic form may present with vascular disease or endocarditis A 6 year old female patient from Sudan followed with the diagnosis of pulmonary atresia and ventricular septal defect was referred to our department because of stenosis in the conduit placed to right ventricular outflow during Rastelli operation performed in Sudan Six months ago in Jordan stenting was performed for conduit stenosis but was unsuccessful Because of long lasting fever she was treated for culture negative endocarditis in Sudan In physical examination fever and hepatosplenomegaly were present Laboratory findings revealed anemia leukopenia Coombs rsquo; positivity hypergamaglobulinemia and positive anticardiolipin antibodies but blood cultures were negative Different antibiotic protocols were administered for culture negative endocarditis for 2 months but fever persisted On 67 day of hospitalization positive antibody titers for Coxiella Burnetii were detected and doxycycline and hydroxychloroquine were started Here a case of chronic Q fever due to Coxiella burnetii which is a rare cause of culture negative endocarditis is presented Turk Arch Ped 2013; 48: 339 41

In the present study, it was aimed to investigate the concomitance of additional cardiac problems, mainly mitral valve prolapse, in adolescents and pediatric patients with Hashimoto’s thyroiditis, by screening autoimmune markers.Fifty-seven euthyroid patients, who applied to the Pediatric Endocrinology clinic at our institution with marked symptoms of hypothyroidism at the time of diagnosis, and were diagnosed and treated for Hashimoto’s thyroiditis, were included in the present study. All patients were evaluated by performing non-organ specific autoantibodies which could be tested at our institution, thyroid ultrasonography, two-dimensional echocardiography, and 24-h holter monitorization.Of the 57 cases with Hashimoto’s thyroiditis, 48 (84.2%) were female, and nine (15.8%) were male. In the echocardiographic evaluation, mitral valve problems were detected in 10 (17.5%) of all cases; mitral valve prolapse was diagnosed in eight (seven females and one male) cases, and mitral insufficiency was diagnosed in two female cases. First-degree atrioventricular block was observed in only two patients during 24-h holter monitorization. Different non-organ specific autoantibody positivity was distributed as antinuclear antibody in 15 (26.3%) cases, anticardiolipin IgG in two cases, anticardiolipin IgM in three cases, tissue transglutaminase IgA in one, glutamic acid decarboxylase in one, anti-insulin antibody in four cases, antiphospholipid IgG in one, and antiphospholipid IgM in one case.It should be underlined that patients with Hashimoto’s thyroiditis should to be followed up closely for mitral valve prolapse and accompanying autoimmune diseases.

Dogumsal kalp hastaliklari siklik acisindan yenidogan doneminde gorulen konjenital problemler arasinda ilk sirada yer almaktadir. Hastaliklardan bazilari belirgin klinik bulgular verse de; nadir olmayarak bulgularin silik olabilecegini gormekteyiz. Insan gozunun yanilabilecegi noktalarda cesitli standardizasyon yontemleri arastirilmis; ve pulse oksimetre ile oksijen saturasyonu taranmasinin iyi bir alternatif olabilecegi uzerinde durulmustur. Cesitli calismalarda, klinik olarak farkedilemeyen bazi dogumsal kalp hastaliklarina saturasyon taramasi ile tani konuldugundan bahsedilmektedir. Ancak bu yarar yontemin yalanci negatiflik ve yalanci pozitifligi oranlari ile golgelenmektedir. Bu nedenle Amerikan Pediatri Akademisi 2011'de saturasyon taramasi algoritmini guncellemistir. Guncelleme yenidoganlarda saturasyon olcum zamanlari ve elde edilen saturasyonlarin degerlendirilmesinde standardizasyon saglanmasini hedeflemektedir. Tum cabalara ragmen halen bir kisim konjenital kalp hastalikli yenidogan tani alamadan taburcu olmaktadir. Bu baglamda, pratikte cok kullanilmasina ragmen; ayrintilari iyi bilinmeyen saturasyon olcumunun onemsenmesi gerektigini dusunmekteyiz.

ÖzetAmaç: Çalışmamızda konvansiyonel ekokardiyografi ile kalp fonksiyonlarınormal bulunan Duchenne Musküler Distrofili (DMD) hastaların miyokardfonksiyonlarının pulse wave doku dopplergörüntüleme (PWDDG) ve strain/strain rate (S/SR) ekokardiyografi aracılığıyla ölçülmesi ve kontrol grubu ilekarşılaştırılması amaçlandı. Materyal ve Metod: Bu çalışmada;ortalama yaşı 85,2 ± 38,4 ay olan 32 DMD’li erkek hasta ve 89 ± 38,9 ay olan 31sağlıklı çocuk değerlendirildi. Demografik özellikler olgular ve kontrolgrubunda değerlendirildi. Cinsiyet, yaş, vücut kitle indeksi, konvansiyonel ekokardiyografi verileri, pulse wave doku Dopplergörüntüleme (PW-TDI) verileri ve iki boyutlu (2D) longitudinal strain (LS) / longitudinalstrain rate (LSR) ekokardiyografi değerleri karşılaştırıldı.Bulgular: Hasta ve kontrol grupları arasında kalp hızı bakımından istatistikselolarak anlamlı fark saptandı (p