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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 Congenital heart diseases (CHD) are the most common birth defects. Necrotizing enterocolitis (NEC) is an important cause of morbidity and mortality in premature infants, and probiotics can be used to protect NEC. CASE REPORT We present a term infant with aortic coarctation who developed sepsis with Lactobacillus rhamnosus GG after probiotic use, successfully treated with ampicillin. The baby unfortunately died of acute cardiac arrest on the 90th day of life. CONCLUSION Probiotic-associated sepsis may develop in infants with various risk factors such as central catheterization, long-term mechanical ventilation and in those at risk for NEC.

Pulmonary artery sling is a rare congenital vascular abnormality, where the left pulmonary artery originates from the right pulmonary artery, passes between trachea, and esophagus and reaching the left hilum. Cough, wheezing, and difficulty in feeding are three major symptoms. Untreated pulmonary sling carries high morbidity and mortality, most of which is due to the airway and other associated anomalies. Herein, we reported a 40-day-old male infant who admitted to the pediatric intensive care unit with progressive respiratory distress and diagnosed with left pulmonary sling with tracheal stenosis. We discussed the diagnosis and management of pulmonary artery sling and present the successful use of laryngeal mask in difficult airway management.

Introduction: This study aimed to investigate the complication frequency and the changes in right heart geometry with different access types in the pediatric population. Methods: We included 32 consecutive patients aged between 10 and 19 and who underwent hemodialysis sessions via permanent hemodialysis catheter (nHC = 18) or arterio-venous fistula (nAVF = 14) between January 2013 and March 2018. We recorded and compared the complication frequency and the changes in echocardiography findings with different access types. Findings: Demographic data were similar in both groups. Number of new access creation (nHC = 15 vs nAVF = 1) and all complications (nHC = 19 vs nAVF = 6) were significantly higher in hemodialysis catheter group and the statistical analysis showed the superiority of arterio-venous fistula group in comparison of event-free survival (event-free patients; nAVF = 8 (57%), nHC = 3 (16%); p = 0.02). Control echocardiography showed impressive delta-change in right atrium diameter (p = 0.04), right ventricular end-diastolic volume (p = 0.004), right ventricular end-systolic volume (p Conclusion: Arterio-venous fistula has lower risk of complications, but overloading stress on right heart chambers triggers remodeling process and geometrical changes, which can be early pieces of evidence of delayed right heart dysfunction in pediatric hemodialysis patients.

Objective Vasoactive-inotropic Score (VIS) was developed to quantify the amount of inotropic support provided in the postoperative period. We investigated the predictive value of (VIS) for mortality in neonates with congenital heart disease (CHD). Study design Prospective cohort. Patients 119 newborns who underwent cardiac surgery. Setting Tertiary NICU-CHD center of Ankara from November 2016 to January 2019. Intervention/Measurement VIS values were calculated by a standard formula for the first 72 postoperative hours, and the maximum score was recorded. Primary outcomes Duration of mechanical ventilation, NICU length of stay, and mortality. Results At surgery, the median (IQR) age was 15 d (9-31). The patients were divided into two groups according to mortality; Group 1 (Non-survivors) (n=36) and Group 2 (Survivors) (n=83). Higher VIS score was correlated to longer duration of mechanical ventilation (P=0.009, r=0.33), and was higher among patients who died (P=0.003). Area under the curve (AUC) was 0,83 (P15.5) was independently associated with increased odds for mortality (OR: 8.1, 95% CI: 1.8-35.7, P=0.005). Conclusions In newborns with CHD, a higher VIS within 72 hours after cardiac surgery is associated with increased duration of mechanical ventilation, and mortality. VIS may be useful for prediction of mortality at early postoperative period.

For the small for gestational age (SGA) infants born at the normal birth time but whose birth weights were below the 10th percentile and for the appropriate for gestational age (AGA) infants born in normal birth weights according to gestational week, the diameters of right and left coronary arteries were echocardiographically measured in three separate time intervals with their body weights, heights and body surface area parameters. The aim of this study is to compare the progression of coronary artery diameters of SGA infants with AGA infants over time and the effects of coronary artery diameters on the clinical and hemodynamic parameters of SGA babies.Term 55 SGA babies were compared with 200 AGA infants at birth, first and sixth months of ages for both growth parameters and coronary artery diameters measurements.In comparison of SGA and AGA groups at birth, first and sixth months of ages on body weight, height and body surface area, aortic annulus and left coronary artery and right coronary artery diameters, it was seen that SGA group could not catch up the AGA group in all time periods on both anthropometric and coronary artery diameters data even if they have an increased growth (In the evaluation of growth indices and coronary artery diameters at birth, first and sixth months of ages in SGA babies, it is clear that impaired fetal growth of these babies has lasting-effects on coronary artery diameters and cardiac structure.

Objective:Non-cardiac chest pain is a common and persistent problem for children; yet, typically, there is no clear medical cause. To date, no behavioural and/or psychological factors have been studied to explain chest pain in a pre-school paediatric sample. We hypothesized that pre-school children with medically unexplained chest pain would have higher rates of behavioural problems compared to healthy controls.Methods:We assessed 41 pre-school children with non-cardiac chest pain and 68 age matched children with benign heart murmurs as the control group using the Child Behaviour Check List-1 1/2–5 to evaluate emotional and behavioural problems.Results:Internalizing problem scores comprising emotionally reactive, anxiety/depression, and somatic complaints were higher in children with non-cardiac chest pain than in the control group. Among the possible factors, the factor that is related to behaviour problem scores, in univariate analysis, was a significant and inverse correlation between maternal education and behaviour problem scores. Also, maternal employment status was associated with behavioural problems. Children with a housewife mother were more susceptible to having such behavioural problems. Based on multiple regression analyses, being in the non-cardiac chest pain group was found to be significantly related to internalizing problems in our total sample.Conclusions:These results suggest that pre-school children with non-cardiac chest pain may experience increased levels of certain behavioural comorbidities. Systematic behavioural screening could increase the detection of behavioural problems and improve care for this population. Future studies of non-cardiac chest pain in pre-school children should include larger samples and comprehensive diagnostic assessments as well as long-term follow-up evaluations.

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.

Hypotonia-cystinuria syndrome is a very rare autosomal recessive contiguous gene deletion syndrome of PREPL and SLC3A1 at 2p21 with neuromuscular and neuroendocrinologic presentation. We report a two-year-six-month-old affected female infant and her five-month-old affected brother with a novel homozygous deletion in SLC3A1 and PREPL gene. Both of siblings had mild facial dysmorphism, hypotonia, feeding problems, failure to thrive, developmental delay. She also had dilated cardiomyopathy which differ from other reported patients. Therefore cardiomyopathy may also be considered one of the features of hypotonia-cystinuria syndrome. With this case report, we present cardiac manifestation of hypotonia-cystinuria syndrome for the first time. Because of two siblings had hyperechogenic bowel in prenatal sonography, it might be a prenatal marker for HCS.

Balloon atrial septostomy is a palliative procedure that is performed in D-transposition of great arteries when surgery is not immediately available. Although D-TGA and left isomerism association are rare, it is an important condition as the BAS procedure approach is unique. In this case report, we present two cases of D-TGA with left isomerism where BAS was performed due to restrictive atrial septal defect and lack of immediate availability of the paediatric cardiac surgeon.

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.

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.

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.

Introduction:Chest pain is the second most common reason for referral to paediatric cardiologists after benign heart murmurs. Aetiology frequently depends on non-cardiac reasons. In addition, individuals may experience non-cardiac chest pain which is idiopathic or of unknown origin. The aim of this study is to examine psychological symptoms in children and adolescents with medically unexplained chest pain.Methods:A total of 76 patients (ages 8–18 years) were included in the study, who were referred to the paediatric cardiology department with the complaint of chest pain but did not have any detected cardiac aetiology or any other organic causes of chest pain. The control group was composed of 51 healthy volunteers. Self-evaluation scales were given to both groups which included Beck Anxiety Inventory and Children’s Depression Inventory. Also parents of both groups completed the Conner’s Parent Rating Scale for assessment of Attention-deficit/hyperactivity disorder.Results:Anxiety scores of the non-cardiac chest pain group were significantly higher compared to controls. No significant differences were found between patients and controls in terms of attention-deficit/hyperactivity disorder and depression scores. In patient group, patterns were similar for boys and girls and for children and adolescents; except girls scored significantly higher than boys in children’s depression inventory.Conclusions:In children and adolescents, non-cardiac chest pain is associated with increased levels of anxiety. These results show the importance of psychiatric evaluation in non-cardiac chest pain patients. Larger controlled studies are needed to determine the prevalence and impact of attention-deficit/hyperactivity disorder and depression in children and adolescents with non-cardiac chest pain.

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.

Troponin is a marker that displays cardiac injury quickly and accurately. In adults, troponin elevation is usually associated with coronary artery disease and requires urgent cardiac catheterization. In healthy children, myocardial injury is rare and may develop due to many different causes. Therefore, troponin elevation in children and adolescents does not usually require emergency cardiac catheterization. The aim of this study is to assess the most common causes of troponin elevation in children and adolescents and to show which diagnostic tests are helpful in assessing pediatric patients with elevated troponin. Patients who had been diagnosed with troponin I elevation (> 0.06 ng/ml) at Dr. Sami Ulus Maternity, Children's Health and Disease Training and Research Hospital between 2007 and 2018 were retrospectively evaluated. Patients undergoing cardiac surgery and those with severe congenital heart disease were excluded from the study. The medical records of the patients were examined and age, gender, diagnostic tests, and diagnosis were evaluated. During the study period, the records of 972 patients were obtained. 213 patients were excluded from the study because of heart surgery, congenital heart disease, and neonatal asphyxia or sepsis. Of the remaining 759 patients, 58% were male, 42% were female, and the median age was 4 years (3 days to 17 years). The most frequent causes are myopericarditis (n: 164), drug intoxications (n: 85), carbon monoxide poisoning (n: 74), perimyocarditis (n: 65), and intensive inhalation β agonist use in acute asthma and lower respiratory tract infections (n: 70). Patients diagnosed with myocarditis and myopericarditis were admitted with a complaint of chest pain, and the diagnosis was made by history, physical examination, ECG, and echocardiographic findings. Unlike adults, troponin I elevation may be associated with many cardiac and non-cardiac pathologies in children. The most common pathologies in cardiac etiology are myopericarditis and perimyocarditis and can be diagnosed by history, physical examination, ECG, and echocardiography. Cardiac catheterization is not necessary except for rare cardiac pathologies and does not alter the prognosis.

ObjectiveThe aim of the present study was to determine the role of toxic elements and trace elements in the pathogenesis of conotruncal heart defects by measuring their concentrations in the first meconium specimens of the affected newborns.MethodsConcentrations of lead, cadmium, iron, zinc, and copper were measured in 1st-day meconium specimens that were collected from 60 newborns with conotruncal heart defects (Group I) and 72 healthy newborns (Group II).ResultsThe newborns with conotruncal defects and the healthy newborns had statistically similar demographic and clinical characteristics. When compared with healthy newborns, mean concentrations of lead, cadmium, iron, zinc, and copper were significantly higher in newborns with conotruncal heart defects (p=0.001 for each). In total, 51 newborns with conotruncal heart defects had normal karyotype. These newborns had significantly higher concentrations of lead, cadmium, iron, zinc, and copper when compared with healthy newborns. There were significant and positive correlations between the concentrations of lead and cadmium (r=0.618, p=0.001), lead and iron (r=0.368, p=0.001), lead and zinc (r=0.245, p=0.005), lead and copper (r=0.291, p=0.001), cadmium and iron (r=0.485, p=0.001), cadmium and zinc (r=0.386, p=0.001), and cadmium and copper (r=0.329, p=0.001).ConclusionToxic metals and trace elements may disturb DNA repair mechanisms by impairing DNA methylation profiles, and thus have a role in the pathogenesis of conotruncal heart defects.

Valved bovine jugular vein conduit is considered a suitable choice for paediatric population with congenital heart defect requiring right ventricle to main pulmonary artery connection. However, complications related to the use of this device have been reported, with conduit failure occurring mainly as a consequence of stenosis, conduit thrombosis, and valve regurgitation. We present a case of aneurysmal conduit failure of a valved bovine jugular vein conduit used to reconstruct the right ventricular outflow tract.

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

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.

The aim of this study was to determine accurate and validated Z-score equations for the normal values of coronary diameters adjusted with growth changes of the neonates by testing three different time periods.Echocardiography was performed on 200 healthy neonates at birth, 1 and 6 months of ages. Several regression models for the left (LCA) and right coronary artery (RCA) diameters were tested with weight, height, body surface area and aortic annulus diameter. Reliable standards for the coronary artery diameters for healthy newborns by determining Z-score equations with appropriate statistical validations were established.The coronary diameters on birth measurements were strongly correlated with birth weight, height, surface area and the diameter of the aortic annulus (Pearson's R0.8, all p0.01). There were significant associations between the mean data of weight, height, body surface area, aortic annulus and the LCA and RCA diameters measurements of study subjects at birth, 1 and 6 months of ages (p0.05). Z-score graphs of coronary artery diameters and weight of the subjects at three different time (LCA vs. weight at birth, R = 0.82, p0.001; LCA vs. weight at 1 month, R = 0.64, p0.001; LCA vs. weight at 6 months, R = 0.55, p0.001; RCA vs. weight at birth, R = 0.80, p0.001; RCA vs. weight at 1 month, R = 0.59, p0.001; RCA vs. weight at 6 months, R = 0.49, p0.001) were constructed.We present a new set of equations for neonatal Z-score calculation on the basis of a large number of healthy neonates on three different time period consisting of birth, 1 and 6 months of ages. It is clear with this study that the growth in caliber of the coronary arteries is definite and progressive during postnatal time.

Amaç: Hodgkin lenfoma hastalarında tedavi ile ilişkili kardiyomiyopati yakınmasız da olabilen bir sorundur. Bu çalışmanın amacı Hodgkin lenfoma hastalarında potansiyel olarak var olan tedavi ile ilişkili kardiyomiyopatiye tanı konmada doku Doppler ekokardiyografi tekniğinin geleneksel ekokardiyografi tekniğine oranla bir üstünlüğünün olup olmadığının araştırılmasıdır. Gereç ve Yöntem: En az dört yıl süreyle hastalıksız izlenen ve kalp yakınması olmayan 17 Hodgkin lenfoma hastası (12 erkek, 5 kız) ve kontrol grubu olarak 14 sağlıklı kişi (8 kız, 6 erkek) çalışmaya alındı. Hastaların kalp işlevleri M-mod, 2 boyutlu, renkli Doppler, CW Doppler ve pulse Doppler teknikleri ile değerlendirildi. Veri analizleri Statistical Package for Social Science for Windows 11,5 paket programı ve sırasıyla Shapiro Wilk, Student’s t testi, Mann Whitney U, Fisher’in kesin sonuçlu ki-kare testleriyle yapıldı. Çalışma için hastane yerel etik kurul onayı alındı (07.05.2007/no:5639). Bulgular: Her iki grup geleneksel ekokardiyografi yöntemleri ile ejeksiyon fraksiyonu ve kısalma fraksiyonu açısından değerlendirildiğinde gruplar arasında fark gözlenmedi (sırasıyla p=0,302 ve p=0,860). Her iki grup sol ventrikül izovolemik ivmelenme zamanı açısından değerlendirildiğinde ölçümler hasta grubunda kontrol grubuna oranla istatiksel olarak anlamlı biçimde kısa bulundu (p:0,038). Miyokardiyal performans indeksi ölçümleri ve sol ventrikül izovolemik kasılma zamanı ölçümleri açısından değerlendirildiklerinde ise yapılan ölçümler hasta grubunda kontrol grubuna oranla istatistiksel olarak anlamlı biçimde uzamış olarak bulundu (sırasıyla p=0,029 ve p=0,049). Çıkarımlar: Sol ventrikül izovolemik ivmelenme zamanı, miyokardiyal performans indeksi ve sol ventrikül izovolemik kasılma zamanlarının tedavi ile ilişkili kardiyomiyopatiyi erken belirlemede önemli ölçümler olduğu sonucuna varılmıştır., Aim: Treatment associated cardiomyopathy which is one of the side effects of treatment can be asymptomatic in Hodgkin lymphoma patients nbsp; The aim of this study was to find out whether tissue Doppler echocardiography was superior to conventional echocardiography in determining potential cardiac problems in the patients diagnosed with Hodgkin lymphoma Material and Method: A total of 17 Hodgkin lymphoma patients 12 males 5 females who were being followed up with disease free status and without cardiac symptoms and whose treatment had been stopped for at least 4 years and a control group of 14 healthy persons 8 males 6 females were included in the study nbsp; The cardiac functions of the patients were evaluated by M mode 2 dimentional colour Doppler CW Doppler and pulse Doppler techniques nbsp; Data analyzes were evaluated by program of Statistical Package for Social Science for Windows 11 5 and Shapiro Wilk Student rsquo;s t testi Mann Whitney U Fisher rsquo;s exact chi square tests respectively nbsp; Hospital ethics committee consent was received for the study 07 05 2007 no:5639 Results: There was no statistical difference between the two groups p: 0 302 and p: 0 860 respectively when both groups were evaluated in terms of ejection fraction and fraction shortening by conventional echocardiography technique Evaluation of both groups for the left ventricular isovolumic contraction time revealed a statistically significant longer time in the patient group compared to the control group p: 0 038 The results were found longer in the patient group compared with the control group when both groups were evaluated in terms of myocardial performance index parameters and the left ventricular isovolumic contraction time parameters p: 0 029 and p: 0 049 respectively Conclusions: We concluded that the left ventricular isovolumic contraction time the nbsp; left ventricular isovolumic acceleration time and myocardial performance index tests are important parameters for the early detection of cardiac pathologies that may develop in relation to chemotherapy Turk Arch Ped 2011; 46: 220 5

Unilateral pulmonary artery agenesis is a rare congenital anomaly caused by a backward displacement of the conical artery of the truncus arteriosus. It is commonly associated with additional cardiovascular abnormalities. A 7-year-old girl was admitted to our clinic with the complaint of shortness of breath upon exertion. Chest radiography revealed a hypoplastic right lung. Absence of the right pulmonary artery with atrial septal defect and pulmonary hypertension was demonstrated by echocardiography, computed tomography, and cardiac catheterization. Bosentan is effectively used to treat pulmonary arterial hypertension.

To investigate the effect of pseudoephedrine on heart rhythm of children with rhinitis. The study included 25 children diagnosed with rhinitis from March 2009 through February 2010 in the Department of Pediatrics. Holter records were obtained for 24 h before and at the fourth day of pseudoephedrine treatments. Study group consisted of 18 girls (72%) and 7 boys (28%) with a mean age of 8.7 ± 3.4 (4–17.9 years). Common complaints of the patients were rhinorrhea (100%), cough (68%) fatigue (48%), sore throat (36%), and headache (28%). Of the 25 patients whose Holter recordings were evaluated, rare supraventricular extrasystoles were observed in one prior to the administration of pseudoephedrine, which were not repeated on this patient’s follow-up recording on day four. There were two ventricular extrasystoles in the day four Holter recording of another patient. None of the patients complained of chest pain or palpitation. There were no observations of supraventricular tachycardia, ventricular tachycardia or ventricular fibrillation. No statistical differences could be found (p > 0.05) in the values before treatment and those on day four of treatment of either the time-dependent Heart rate variability (HRV) parameters SDNN, SDNN index, SDANN and RMSSD, or the frequency-dependent parameters (TP, HF, LF). No statistical difference could be determined between heart rate values of the patients before treatment and those on day four of treatment (p > 0.05). This study has established that therapeutic doses of pseudoephedrine do not cause an additional dysrhythmia risk for children with no health problem except rhinitis.

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.