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Objective:Laterality anomalies are almost always associated with severe cardiac anomalies. Demographic properties, type of the procedures, associated anomalies, and early and mid-term prognosis of four types of laterality anomalies were analysed.Methods:A total of 64 consecutive patients with laterality anomalies were enrolled between July 2014 and July 2020. We grouped the patients as situs solitus dextrocardia (SSD) (n = 12; 18.7%); situs inversus (SI) (n = 16; 25%); right atrial isomerism (RAI) (n = 29; 45.3%); and left atrial isomerism (LAI) (n = 7; 10.9%). TAPVC was only present in the RAI group (31%). Incidence of mitral or tricuspid atresia was higher in the SSD group (25%). All the patients were followed up with a mean of 19.06 ± 17.6 (0.1–72) months.Results:Early postoperative mortality was 17 patients, among 107 procedures (15.8%). Twelve patients were in the neonatal period. All ten patients survived after isolated ductal stenting. Fourteen of the deaths were in the RAI group (48.3%). The 3-year survival rates were 85% in LAI, 78.7% in SI, 55.8% in SSD, and 38% in RAI groups. According to the multivariable Cox regression model, mechanical ventilation, kidney injury, RAI, and complex surgery in the neonatal period were independent risk factors for early mortality.Conclusion:Laterality anomalies are one of the most challenging patients who commonly had univentricular physiology. The most prevalent anomaly was RAI, and RAI had the worst outcome and survival. Ductal stent is an acceptable first intervention during the neonatal period in suitable patients. Complex procedures may carry a high risk of death in the neonatal period.

Acute rheumatic fever is a disease that is very common in developing countries. Heart involvement is responsible for morbidity and mortality. Mitral and aortic valve insufficiency and stenosis, congestive heart failure, myocarditis and pericarditis can be seen. Even if carditis does not develop during the course of the disease, many rhythm and conduction defects may occur. These include all degrees of atrioventricular block, sinus tachycardia and bradycardia, atrial and ventricular premature beats, accelerated junctional rhythm, supraventricular tachycardia, junctional and ventricular tachycardia. In this review, rhythm and conduction defects that may occur in acute rheumatic fever disease, which continues to be an important public health problem, were discussed in the light of the literature., Akut romatizmal ateş, gelişmekte olan ülkelerde çok sık görülen bir hastalıktır. Morbiditeden ve mortaliteden kalp tutulumu sorumludur. Mitral ve aort kapağında yetmezlik ve darlık, konjestif kalp yetmezliği, miyokardit ve perikardit görülebilir. Hastalığının seyri sırasında kardit gelişmese bile birçok ritim ve ileti kusuru ortaya çıkabilir. Bunlar arasında her dereceden atriyoventriküler blok, sinüs taşikardisi ve bradikardisi, atriyal ve ventriküler erken atımlar, akselere nodal ritim, supraventriküler taşikardi, junctional ve ventriküler taşikardi sayılabilir. Bu derlemede, önemli bir halk sağlığı problemi olmaya devam eden akut romatizmal ateş hastalığında ortaya çıkabilen ritim ve ileti kusurları, literatür bilgileri eşliğinde tartışıldı.

Kawasaki hastalığı, gelişmiş ülkelerde edinsel kap hastalıklarının en sık sebebidir. Hastalık birçok klinik tablo ile ortaya çıkabilmekte olup hastalığa bağlı en önemli komplikasyon, koroner arter anevrizmasıdır. Ne kadar erken tanı konulursa ve tedaviye başlanırsa, bu komplikasyonun riski de o kadar azalır. Atipik Kawasaki hastalığı, klinik olarak hastalık kriterlerini karşılamayan ve normalde sık görülmeyen veya beklenmeyen organ ve sistem tutulumu olmasıyla karakterizedir. Hastaların yaklaşık % 7-10 kadarında atipik tutulum görülmektedir. Atipik hastalarda, tanının geç konulmasına bağlı koroner arter tutulumu daha sık görülür. Bu makalede, atipik Kawasaki hastalığına bağlı karşılaşılabilinen klinik durumlar, literatür bilgileri eşliğinde incelenmiştir.

Hypertrophic cardiomyopathy has the highest incidence rate among genetically inherited cardiac diseases. It develops as a result of mutations in genes in related to the sarcomere protein in cardiac muscle. Generally, this results in asymmetrical hypertrophy. Patients who are symptomatic and have a significantly narrow left ventricular undergo should receive surgical treatment, whereas patients with a sudden cardiac death risk should receive treatment with an implantable cardiac defibrillator. This paper presents an infant with hypertrophic cardiomyopathy who was recently identified as having a mutation that resulted in a deletion-insertion type framework shift in the gene MYBPC3, who had family history of sudden death at a young age, and received myectomy and treatment with an implantable cardiac defibrillator in the same session due to a severely narrowed left ventricular outflow tract.

Objective: Childhood obesity which is a predictor of adulthood obesity is associated with type 2 diabetes, metabolic syndrome, dyslipidemia, coronary artery diseases and subclinical inflammation. Growth differentiation factor-15, also known as macrophage inhibitory cytokine-1 (MIC-1/GDF15) is a member of the transforming growth factor- β super family and increases during inflammatory states. Adults with obesity have increased serum concentrations of MIC-1/GDF15. MIC-1/GDF15 is associated with cardio metabolic risk in adults. Design: Identification of obese children at higher risk for cardio metabolic complications is important. The relations of MIC-1/GDF15 to parameters of obesity in children have never been studied. Methods: We measured serum MIC-1/GDF15 concentration in obese children by ELISA and evaluated its relationship with anthropometric, biochemical and echocardiographic parameters. Results: No significant difference in serum MIC-1/GDF15 between normal-weight and obese children was observed. MIC-1/GDF15 was positively correlated with CRP, ALT and aortic dimension when normal-weight and obese children were analyzed together. Conclusions: MIC-1/GDF15 may provide a link between childhood obesity and cardio metabolic complications that will occur in time course. Further work would be required to find out if MIC-1/GDF15 has any role in the early inflammatory, cardiac and metabolic changes in childhood obesity.

Guvenc O, Cimen D. A rare situation in acute rheumatic carditis: Involvement of all four valves. Turk J Pediatr 2017; 59: 497-500. Acute rheumatic fever continues to be an important health problem, especially in countries that are socioeconomically underdeveloped. Carditis, which develops in approximately half of the patients, is responsible for both early-stage mortality as well as late-stage surgical treatment due to heart valve insufficiency or stenosis. The most frequent and severe valve involvement is with the mitral valve, while the aortic valve has the second highest incidence of involvement. Pulmonary and tricuspid valves are rarely involved. The literature cites a few adult cases in which all four valves are affected by rheumatic carditis; however, to the best of our knowledge, there have been no acute-stage rheumatic carditis pediatric cases reported. This article presents a 13-year-old male patient of Syrian origin who escaped to Turkey from the war in his country, and who was in the acute stage of rheumatic carditis in which all four valves were involved.

Siyanoz, redükte hemoglobin konsantrasyonunun 5 gr/dL’den fazla olması sonucu cildin ve muköz membranların mavi mor bir renk almasıdır. Kardiyovasküler, akciğer veya merkezi sinir sistemiyle ilgili önemli bir hastalığın habercisi olabileceği gibi methemoglobinemide de ortaya çıkabilir. Bu derlemede, çocukluk döneminde siyanozla gelen hastalara yaklaşım ve ayırıcı tanının yapılması, literatür bilgileri eşliğinde tartışılmıştır., Cyanosis refers to a condition where the skin and mucous membrane assume a blue-purple color as a result of reduced hemoglobin concentration above 5 g/dL. While it may be the indicator of an important disease related to the cardiovascular, pulmonary or central nervous system, it may also develop with methemoglobinemia. This review discusses the approach and differential diagnosis for patients presenting with cyanosis in the light of literature.

Asymmetric dimethylarginine (ADMA) is an analogue of l -arginine, a naturally occurring product of metabolism found in human circulation. It is an endogenous inhibitor of nitric oxide production. Acute rheumatic fever (ARF) is a delayed immunologically mediated autoimmune sequel of throat infection by group A β-hemolytic streptococci. As serum ADMA levels have not previously been assessed in patients with ARF, we aimed to investigate ADMA levels in patients with ARF during the acute stage and after anti-inflammatory treatment and compared results with healthy control subjects. The study population consisted of 34 children with ARF (30 patients with carditis and 4 patients without carditis) and 31 healthy control subjects. Erythrocyte sedimentation rate and C-reactive protein values were significantly higher and serum ADMA values were lower, but not statistically significant in patients with ARF during the acute stage when compared with controls. Serum C-reactive protein and erythrocyte sedimentation rate values were significantly decreased in patients with ARF after the treatment when compared with baseline and ADMA levels were increased after the treatment compared with baseline, but this change was not statistically significant. Our study has demonstrated that resolution of acute inflammation in patients with ARF may lead to a mild increase in serum concentration of ADMA. Comprehensive prospective and observational studies are required to confirm our findings and to assess potential interactions between ARF and ADMA levels.

Aortopulmonary window (APW) is a rare abnormality in which a pulmonary defect exists between the ascending aorta and the main pulmonary artery. Given that it may result in cardiac failure and pulmonary vascular disease in the early period, treatment needs to be performed without delay. In addition to surgical treatment, transcatheter closure may also be performed for selected patients. This study describes the case of an infant diagnosed with APW and who underwent successful transcatheter closure using a Nit-Occlud® PDA-R device.

Pulmonary arteriovenous malformation, which is defined as the presence of an ab-normal connection between the pulmonary artery and pulmonary vein, is rarely seen. Although it generally presents as a congenital condition, it may be accompanied by hereditary hemorrhagic telangiectasia. Clinical signs vary according to the amount of shunt in proportion to the number and size of the fistulae. Patients may present with cyanosis and respiratory trouble. If the disease remains untreated, it may result in cardiac failure and ineffective endocarditis, thereby leading to the rupture of the an-eurysmal fistula. Transcatheter embolization of abnormal vascular connection is the current treatment method in this disease. This article describes the case of an 8½-year-old child. He was presented with the symptom of getting tired quickly. Transcutaneous oxygen saturation of 75%, and pulmonary arteriovenous malfor-mation was detected in his examination. Successful transcatheter fistula embolization was performed.

Costello syndrome is a rare syndrome characterized by failure to thrive, short stature, mental motor retardation, characteristic facial features, macrocephaly, a short neck, loose soft skin with deep palmar and plantar creases, and hypertrichosis. Cardiac involvement is seen in almost two thirds of patients, and is a determinant for the prognosis of Costello syndrome. The most common cardiac anomalies are pulmonary stenosis, hypertrophic cardiomyopathy, atrial septal defect, ventricular septal defect and arrhytmia. In this report, we present a 14-month-old female pediatric patient with hypertrophic cardiomyopathy, clinically and genetically diagnosed with Costello syndrome. The report also contains a review of recent related literature.

Sacral agenesis syndrome (Caudal regression syndrome) is a neural tube defect that is characterized by absence of the vertebral segment that constitutes the sacrum. It is very rarely seen and generally develops sporadically. Its etiology is influenced by maternal diabetes, genetic factors, teratogenic agents and vascular hypo-perfusion. It is important to make a diagnosis in the prenatal period. This paper presents a newborn diagnosed with sacral agenesis as a case and discusses this disease in the light of the latest literature information.

Kısa etkili inhale beta 2 mimetik bir ilaç olan salbutamol, bronkospazm ve solunum sıkıntısı durumlarında çok sık kullanılmaktadır ama ülkemizde intihar amaçlı alımı nadir görülen bir durumdur. Salbutamole bağlı komplikasyonlar iyi bilinmektedir ve terapotik dozlarda da ortaya çıkabilir. İlaca bağlı toksikasyon geliştiğinde hiperaktivite, tremor, konvülzyon, hiperglisemi, hipokalemi, hipomagnezemi, laktik asidoz, hipotansiyon, hipertansiyon, konjestif kalp yetmezliği, miyokard iskemisi, atriyal ve ventriküler ektopik atımlar, atriyal aritmiler, QT uzaması ve ventriküler taşikardi gibi aritmiler ile ani kardiyak ölüm ortaya çıkabilir. Bu makalede, intihar amaçlı olarak salbutamolün tablet formundan 200 mgr kullanan 15 yaşındaki bir hasta sunuldu ve literatür bilgileri eşliğinde çocuklarda salbutamol zehirlenmesine yaklaşım tartışıldı., Salbutamol, a drug with short-acting inhaled beta 2 mimetic effect, is very frequently used in cases of bronchospasm and respiratory distress; however, its intake for suicide purposes is rare in Turkey. Salbutamol-induced complications are well established and may be seen at therapeutic doses. When drug-related intoxication develops, hyperactivity, tremor, convulsion, hyperglycemia, hypokalemia, hypomagnesemia, lactic acidosis, hypotension, hypertension, congestive heart failure, myocardial ischemia, atrial and ventricular ectopic heart beats, atrial arrhythmias, QT prolongation and arrhythmias such as ventricular tachycardia and sudden cardiac death may occur. This paper presents a 15-year-old patient who used 200 mg salbutamol in tablet form for suicide purposes and discusses the approach to salbutamol intoxication with a review of the literature information

Goltz sendromu (Fokal dermal hipoplazi), mezoektodermal dokuyu tutan ve çok nadir görülen bir hastalıktır. Deri lezyonları ile birlikte iskelet sistemi, dental ve oküler anomaliler, hastaların az bir kısmında da kalp ve böbrek anomalileri görülebilmektedir. Makalede, Goltz sendromu tanısı alan, biküspit aort kapağı ve buna bağlı aort kapak darlığı ve yetmezliği olan beş yaşındaki kız hasta olgu olarak sunuldu ve seyrek görülen bu hastalık, son literatür bilgileri eşliğinde tartışıldı., Goltz Syndrome (focal dermal hypoplasia) is a rare disease that is characterized by mesoectodermal tissue abnormalities. In addition to cutaneous lesions, there may be skeletal, dental, and ocular defects as well as cardiac and renal abnormalities in a minority of patients. In this article, a 5-year-old girl with aortic stenosis and regurgitation related to bicuspid aortic valve and diagnosed with Goltz Syndrome is presented. This rare syndrome has been discussed with the help of the recent literature

Major aortopulmonary collateral arteries are abnormal vascular structures that may be seen in cyanotic diseases that progress with reduced pulmonary flow. They occur rather rarely in the absence of cyanotic congenital heart disease. Presently described is the case of an infant who underwent patent ductus arteriosus (PDA) and major aortopulmonary collateral artery occlusion in a single session, without presence of cyanotic congenital heart disease. To the best of our knowledge, this is the first case of congenital aortopulmonary collateral artery to be reported in a symptomatic infant with PDA.

WOS: 000389599300011, PubMed: 27156243, Purpose: The goal of this study was to investigate the radiation dose and diagnostic efficacy of cardiac computed tomography angiography (CCTA) using prospective ECG-gated high-pitch dual-source computed tomography (DSCT) in the diagnosis of congenital cardiovascular abnormalities in pediatric population. Materials and methods: One hundred five pediatric patients who were clinically diagnosed with congenital heart disease with suspected extracardiac vascular abnormalities were included in the study. All CCTAs were performed on a 128 x 2-section DSCT scanner. CCTA findings were compared with surgical and/or conventional cardiac angiography findings. Dose-length product (DLP) and effective doses (ED) were calculated for each patient. Patients were divided into 4 groups by age, and ED and DLP values were compared among groups. The image quality was evaluated using a five-point scale. Results: CCTA showed 173 abnormalities in 105 patients. There were 2 patients with false positive and 3 with false negative findings. The sensitivity and specificity of CCTA were 98.3% and 99.9%, respectively. The positive predictive value and negative predictive value of CCT were 98.9% and 99.9%, respectively. The average DLP and ED values were 15.6 +/- 9.6 (SD) mGy.cm and 0.34 +/- 0.10 (SD) mSv, respectively. The mean image quality score was 4.8 +/- 0.5 (SD) in all patients. The inter-observer agreement for the image quality scores was good (kappa = 0.80). Conclusion: CCTA is an excellent imaging modality for evaluation of cardiovascular abnormalities and provides excellent image quality with very low radiation exposure when low-dose prospective ECG-triggered high-pitch DSCT is used. (C) 2016 Editions francaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

Patients with wide patent ductus arteriosus and significant pulmonary hypertension not treated in time constitute a significant problem for cardiologists. For these patients, tests that could aid in decision-making for further planning include reversibility and balloon occlusion tests performed in the catheterization laboratory. Devices developed for the closure of ductus as well as different devices with off-label use may be employed in patients scheduled for transcatheter occlusion. When result of reversibility test is borderline positive, the use of fenestrated device may be applicable for selected patients. Presently described is case of a 10-year-old patient with Down syndrome who had a wide ductus and systemic pulmonary hypertension. Transcatheter closure procedure was performed with off-label use of a fenestrated muscular ventricular septal defect occluder device.

WOS: 000382751900015, Acute rheumatic fever (ARF) is a multisystem disease caused by an immunological response to group A streptococcus infection. Its sequel rheumatic heart disease continue to cause a large burden of morbidity and mortality in developing countries. Early detection of ARF is paramount to the prevention of rheumatic heart disease. We report a case of ARF with presenting epistaxis. The variety of clinical manifestations, which may be the presenting signs and symptoms of ARF, are not included in the updated-revised Jones criteria. Therefore, a careful examination and awareness of the disease can play an important role in identifying ARF.

Anomalous origin of the left coronary artery arising from pulmonary artery ALCAPA syndrome is a rare congenital heart disease seen in children. If untreated, it may lead to congestive heart failure, dilated cardiomyopathy (DCM), ischemic and arrhythmic complications may lead to patient’s death. ALCAPA is diagnosed with echocardiography; in the patients of suspected diagnosis, computerized tomography, magnetic resonance imaging and cardiac catheterization are used for further testing. Surgically correctable ALCAPA syndrome must be considered as etiology of DCM in children. In this report, we presented the case of an infant that was referred to our center with the diagnosis of DCM, who was echocardiographically diagnosed with ALCAPA syndrome and successfully treated with surgery, as well as a review of recent literature. Sol koroner arterin pulmoner arterden çıkış anomalisi olarak tanımlanan ALCAPA sendromu, çocuklarda nadir görülen bir konjenital kalp hastalığıdır. Tedavi edilmediği zaman konjestif kalp yetmezliği, dilate kardiyomiyopati (DKM), iskemik ve aritmik komplikasyonlarla hasta kaybedilebilir. Tanı ekokardiyografi bulgularıyla koyulur, tanıdan şüphelenilen olgularda bilgisayarlı tomografi, manyetik rezonans görüntüleme ve kalp kateterizasyonu gibi ileri tetkiklerden faydalanılabilir. Çocukluk çağında DKM tanısı alan hastalarda etiyolojide, cerrahi olarak düzeltme şansı olan ALCAPA sendromu mutlaka düşünülmelidir. Bu yazıda, merkezimize DKM tanısıyla sevk edilen, ekokardiyografi ile ALCAPA sendromu tanısı konulup, başarılı cerrahi tamir yapılan hasta olgu sunumu yapıldı ve son literatür gözden geçirildi.

Anomalous systemic venous return (AVSR) may sometimes present as pathologic entities itself, or associated with other congenital heart defects. The presence of AVSR may require significant changes in surgical technique during the repair of congenital heart defects. The aim of our study was to assess the prevalence of anomalous systemic venous return and to determine the congenital heart anomalies accompanying AVSR in children. This study is based on a retrospective review of the medical records of 175 children who consecutively underwent cardiac catheterisation because that congenital heart disease. The most extensively diagnosed cardiac malformation was VSD (26.8%). ASVR was prevalent in 4.5% of patients. The most commonly diagnosed ASVR was persistent left superior vena cava (3.4%) and a second ASVR type was associated interruption of the inferior vena cava (IVC) with azygos vein continuation (1.1%). A systematic study of systemic venous connections should be performed in all those undergoing cardiac catheterisation as a prelude to open heart repair because it may require significant changes in surgical technique during the repair., Anormal sistemik venöz dönüş (AVSR) bazen tek başına patolojik bir durum veya diğer doğumsal kalp hastalıkları ile birlikte görülebilir. AVSR mevcudiyeti doğuştan kalp kusurlarının onarımı sırasında cerrahi teknikte önemli değişiklikler gerektirebilir. Çalışmamızın amacı, doğuştan kalp kusurları olan hastalarda anormal sistemik venöz dönüş sıklığını değerlendirmek ve AVSR’ye eşlik eden konjenital kalp anomalilerini belirlemekti. Bu çalışma doğuştan kalp kusurları nedeniyle kardiyak kateterizasyon uygulanan ardışık 175 çocuğun tıbbi kayıtlarının geriye dönük yorumuna dayanmaktadır. En yaygın kardiyak malformasyon tanısı ventriküler septal defekt idi (26.8%). Anormal sistemik venöz dönüş hastaların % 4.5’inde görüldü. En sık görülen ASVR sol persistan superior vena kava (% 3.4) ve ikinci sıradaki ASVR tipi azygos ven devamlılığı ve kesintili inferior vena kava (% 1.1) idi. AVSR doğuştan kalp kusurlarının onarımı sırasında cerrahi teknikte önemli değişiklikler gerektirebilir bu yüzden açık kalp cerrahisi uygulanması için kardiyak kateterizasyon yapılan tüm hastalara sistemik venöz bağlantı için sistematik bir çalışma yapılmalıdır

Diyabetes mellitus, gebelik boyunca fetal gelişim üzerine olumsuzetkileri olan, yenidoğanlarda metabolik bozukluklara ve doğumsal anomalilereyol açan bir durumdur. En sık doğumsalanomaliler, kardiyovasküler, gastrointestinal, genitoüriner sistemde ve santralsinir sisteminde görülür. Oluşabilen kardiyovasküler anomaliler arasındaventriküler ve atriyal septal defekt, patent duktus arteriyozus, asimetrikseptal hipertrofi, büyük arter transpozisyonu, triküspit atrezisi ve aortkoarktasyonu sayılabilir. Diyabetik anne bebeklerinin intrauterin dönemdenitibaren doğumsal kalp hastalıkları yönünden de takip edilmesi gereklidir. Bu yazıda, diyabetik anne bebeklerinin kardiyovaskülersistem sorunlarının değerlendirilmesi ve son literatür bilgileri eşliğindegözden geçirilmesi amaçlanmıştır., Diabetes mellitus is a condition that has negative effects on fetal development throughout pregnancy and causes metabolic disorders and congenital abnormalities in neonates. The most frequent congenital abnormalities are seen in cardiovascular, gastrointestinal, genitourinary system and central nervous system. The cardiac abnormalities that may develop include ventricular and atrial septal defect, patent ductus arteriosus, asymmetrical septal hypertrophy, large artery transposition, tricuspid atresia and aortic contraction. The infants of diabetic mothers need to be followed up also in respect of congenital heart diseases as of the intrauterine period. In this study, we aimed to evaluate the problems of infants of diabetic mothers and to determine risk factors of mothers during pregnancy.

In this report we persent a rare complication of Amplatzer septal occluder; associated with ‘atrial rim’ erosion during atrial septal defect closure. In our case report, we also present a brief review of literature of complications associated with transcatheter closure of atrial septal defects

WOS: 000343068800007, PubMed: 25539541, Aim: Evaluation of cardiac structures requires a large study group for accurate data on normal values. The aim of the study was to obtain normal M mode echocardiographic values in a substantial sample of healthy term neonates and children to develop centile charts. Materials and methods: Data were obtained over 2 years from a single center in Turkey, from 1200 healthy infants and children aged 1 day to 17 years. Using echocardiographic investigation, measurements were obtained of the following: left ventricular dimension at end diastole and end systole; thickness of interventricular septum and posterior wall of the left ventricle; aortic and pulmonary root diameter; and left atrial dimension. The influence of systematic errors as statistical noise in this large sample was decreased using third-degree polynomial curves. Results: Measurements are presented graphically as curved lines of centiles with respect to body weight for healthy term neonates and children. The values showed a good correlation with body weight and allowed the construction of percentile curves (5%, 25%, 50%, 75%, and 95%). Higher values were observed in boys during adolescence. Conclusion: The presented charts and tables make it possible to judge the echocardiographic measurements of a particular patient as normal or abnormal.

Amaç: Bu çalışmada, çocuk kardiyoloji kliniğimizde üç yıl boyunca pulmoner balon valvüloplasti işlemi yapılan çocuklarla ilgili klinik deneyimlerimizin paylaşılması ve sonuçlarının değerlendirilmesi amaçlandı . Gereç ve yöntem: Bu retrospektif çalışmaya, Kasım 2010- Kasım 2013 tarihleri arasında pulmoner balon valvüloplasti işlemi uygulanmış olan çocuklar dahil edildi. Hastalardan sendromik olan yoktu. Pulmoner balon valvüloplasti işlemi, hastalar kateterizasyon laboratuarında genel anestezi altında entübe edilerek gerçekleştirildi. Bulgular: Çalışmaya yedisi kız, altısı erkek 13 hasta dahil edildi. Hastaların ortalama yaşı 2.93.74 yıl, yaş aralığı dört günlük-10 yaş arasında idi. Pulmoner darlık 10 hastada valvüler tipte olmasına karşılık, üç hastada valvüler ve infundibuler darlık birlikteydi. İşlemde monofoil kateterler kullanıldı, kateterlerin ortalama çapı 12.2 mm (6-20 mm) idi. Balon/anülüs çap oranı ortalaması 1.21 (1.1-1.33) olarak hesaplandı. Kalp kateterizasyonu sırasında pulmoner kapak üzerinde ölçülen basınç gradiyenti, işlem öncesinde ortalama 75 mmHg (40-120 mmHg), işlem sonrasında ise ortalama 36.4 mmHg (0-95 mmHg) olarak ölçüldü. Hastalara ortalama üç kere dilatasyon yapıldı. Hastalardan dördünde darlık gradiyentinde anlamlı bir düşüş olmadığı için hastalar operasyona verildi, bir hastaya ise takiplerinde rezidü darlık geliştiğinden dolayı ikinci kere balon valvüloplasti işlemi yapıldı. Dört hastada (% 30) hafif pulmoner yetmezlik tespit edildi, başka majör veya minör bir komplikasyon gelişmedi. Sonuç: Pulmoner balon valvüloplasti işlemi, pulmoner kapak darlıklarında, her yaştaki hastada ilk tedavi seçeneğidir. Güvenli ve etkili bir işlemdir. Balon/anülüs çap oranı 1.2-1.4 arasında olursa; başarı şansı daha yüksek ve komplikasyon gelişme ihtimali daha düşüktür, Objectives: In this study, we aimed to share our clinical experience and evaluate the results of the pulmonary balloon valvuloplasty procedure in patients who attended our pediatric cardiology department fot the last three years. Matherial and methods: The patients who underwent pulmonary balloon valvuloplasty procedure between November 2010 and November 2013 have been included this retrospective study. There were no syndromic patients. The pulmonary balloon valvuloplasty procedure was performed by intubating the patients under general anesthesia in the catheterization laboratory. Results: Seven girls and six boys have been included this study. The mean age was was 2.9±3.74 years and the patients aged between four days and 10 years-old. There were valvular type pulmonary stenosis in ten patients, valvular and infundibular type pulmonary stenosis in three patients. Monofoil catheters were used for the procedure and the average diameter of the catheter was 12.2 mm (6-20 mm). Balloon/anulus diameter ratio was 1.21 (1.1-1.33). Du- ring cardiac catheterization pulmonary valve pressure gradient was measured as 75 mmHg (40-120 mmHg) before the procedure. The gradient was measured as 36.4 mmHg (0-95 mmHg) after the procedure. Dilatation procedure was performed 3 times on patients. Four patients were given to operation because of the non effective reduction in the stenosis gradient. Second pulmonary balloon valvuloplasty procedure was performed for a patient because of the residual stenosis observed during follow-up. Mild pul- monary failure was observed in four patients (% 30). There was no major or minor complication. Conclusion: Pulmonary balloon valvuloplasty procedure is the first treatment option in patients of all ages for pulmonary valve stenosis.The procedure is known as safe and effective. If balloon/ anulus diameter ratio is 1.2-1.4, complications are less likely and the success of the procedure is higher

Myocardial hypertrophy and cardiac dysfunction frequently occur in newborns of diabetic mothers. The authors hypothesized that wall hypertrophy or disproportionate left ventricular wall thickness in newborns of diabetic mothers may affect both QT and QTc dispersion. This study aimed to assess whether left ventricular hypertrophy affects the QT variables of infants born to diabetic mothers. This prospective cross-sectional study was conducted with 47 consecutively selected neonates of gestational diabetic mothers and 30 healthy neonates born to healthy mothers. All the subjects were evaluated during the neonatal period. Electrocardiography with echocardiography was performed for the patients and the control subjects. The newborns of the diabetic mothers were classified according to septal thickness as group 1 (16 newborns with septal hypertrophy) or group 2 (31 newborns without septal hypertrophy). The study group consisted of three cohorts: groups 1, 2, and 3 (control group). Both QT and QTc dispersion were computed from a randomly selected beat as well as from an average beat derived from 12 beats included in a 10-s electrocardiography. A total of 16 infants (34 %) had a septal thickness of 6 mm or greater. The left ventricular end-systolic diameter in group 1 was smaller (p = 0.0029) than in groups 2 and 3 (p = 0.003). The interventricular septal thickness at end diastole (IVSTd) and the left ventricular posterior wall thickness at end diastole in group 1 were higher than in of groups 2 and 3. The QT and QTc dispersion intervals were longer in group 1 than in groups 2 and 3 (p < 0.001), and a highly significant positive correlation was detected between IVSTd and QT dispersion (r = 0.514, p = 0.042). Elevated QT and QTc dispersions may be risk factors for the development of arrhythmias in newborns of diabetic mothers. These patients may critically need systematic cardiac screening.

BackgroundThis was a prospective controlled study to determine the P-wave duration and P-wave dispersion in patients with atrial septal aneurysm.MethodsA total of 41 children with atrial septal aneurysm, including 21 boys and 20 girls (mean age 11.85 ± 3.8 years), and 32 controls, including 17 boys and 15 girls (mean age 12.3 ± 2.9 years), were included. P-wave dispersion was calculated from the 12-lead electrocardiogram. Cardiac functions, morphology of the aneurysm, and left atrial diameter were measured using conventional echocardiography. The diagnosis of atrial septal aneurysm was made when the base of the aneurysms with an excursion ratio ≥25% was found on echocardiography.ResultsThere was no significant difference between the patient and control groups in demographic, clinical findings, and M-mode echocardiographic parameters. The P-wave dispersion in patients with atrial septal aneurysm was significantly longer compared with the control group (64.4 ± 13.4 ms; p < 0.0001). Similarly, the the maximum duration of the P wave in the patient group was significantly longer compared with the control group (106.1 ± 13.3 ms; p < 0.001). The P-wave duration and dispersion were not correlated with age, gender, systolic and diastolic blood pressure, or m-mode echocardiographic parameters.ConclusionsThis study shows that P-wave dispersion is delayed in atrial septal aneurysm patients. Prolonged P-wave dispersion was determined to indicate electrical disturbance, and therefore it has an increased electrocardiographic risk of atrial arrhythmia in children with atrial septal aneurysm.

Objective: The aim of this study was to examine whether asymmetric dimethylarginine (ADMA) concentrations are associated with ventricular function in the infants of mothers with gestational diabetes. Method: Twenty-five term newborns of mothers with gestational diabetes and term newborns as the control group (n ¼ 25) with normal general health status were evaluated at two time points, on the 3rd postnatal day, at the 3th months. Echocardiographic evaluations of all participants were performed and ADMA level was measured. Results: In the first analysis, 10 patients (40%) had a septal thickness of 6 mm or more, indicating septal hypertrophy. In the first and second analysis, interventricular septum end-diastolic thickness (IVSTd) and the left ventricular posterior wall end-diastolic thickness (LVPWTd) in the patient group were higher than the control group. ADMA level measurement was not significantly different between the groups the first and second analysis. There was no difference in ADMA levels of the group with septal thickness � 6 mm and the group with56 mm. Conclusion: Newborn cardiac wall thickness was increased in pregnancies complicated by Gestational diabetes mellitus (GDM), and the increase was independent of glycemic control. Diastolic newborn cardiac function was impaired in GDM, and this effect was independent of septal thickness. We found no association between ADMA levels and cardiac systolic, diastolic functions or septum thickness in the GDM newborn.

The majority of patients with congenital heart disease (CHD), particularly those with relevant systemic-to-pulmonary shunts, if left untreated, will experience the development of pulmonary arterial hypertension (PAH). Previous studies have shown that platelet activation occurs in patients with PAH. In response, this study aimed to investigate the platelet indices, including platelet distribution width (PDW) and mean platelet volume (MPV), in patients with PAH. The study was conducted in the pediatrics cardiology unit of Selcuk University Medical Faculty between July 2010 and January 2012. The patients' clinical and laboratory data were obtained retrospectively from hospital recordings. The study enrolled 57 children with CHD (all with left-to-right shunting). The patients who had undergone diagnostic cardiac catheterization were analyzed according to the presence or absence of PAH. Group 1 had CHD with PAH, and group 2 had CHD without PAH. Compared with the group 2 patients, a significant decrease in PDW (p < 0.0001) was noted in the group 1 patients (with PAH). Likewise, a significant difference was found in the MPV of group 1 (p < 0.0001). Statistically, groups 1 and 2 did not differ in terms of platelet count (p = 0.3). Patients who had pulmonary hypertension secondary to CHD with left-to-right shunting exhibited a lower PDW and MPV.

Paediatric exercise testing laboratories should accommodate subjects of various sizes and ages. To this day, games and conventional treadmill exercise tests have not been carried out in pre-pubertal children. Children cannot easily adapt to the treadmill or cycle ergometer. We therefore aim to the use of video games as a form of exercise tests in pre-pubertal children. Twenty healthy children (10 girls and 10 boys, aged 5-11 years old) were enrolled in the study. The physical examination, 12-lead electrocardiography and echocardiography were performed. The heart rate and respiratory rate were measured with systolic blood pressures. Treadmill exercise testing was performed, and electrocardiographic changes were studied during both the treadmill exercise test with Bruce protocol and an activity-promoting video game (Nintendo Wii Boxing), in different time periods. Exercises were stopped at the target heart rate.The mean duration of exercise was 15 minutes in both the Bruce protocol and the activity-promoting video game. Although the data of healthy children (including heart rate and blood pressure responses to exercise) were consistent with the results from several countries using the Bruce protocol, the mean maximal heart rates for all groups were slightly lower than those obtained with the video game. The data obtained from this new exercise test may be used to determine the diagnosis and activation of cardiovascular disease in pre-pubertal children. It can be used as an exercise test especially in young children who are unable to use the treadmill or cycle ergometer

Atriyal septal defekt (ASD), en sik gorulen dogustan kalp hastaliklarindan birisidir (1). Bu defekt, uzun yillar cerrahi yontemle kapatilmaktaydi. Son yillarda ise cerrahi tedavi; torakotomi gerekmesi, skar olusmasi, hastanede kalis suresinin uzun olmasi, reziduel sant gorulebilmesi ve ameliyata bagli gelisen diger komplikasyonlardan dolayi tercih edilen bir secenek olarak gorulmemektedir. Sekundum ASD’ler, cerrahi tedaviye alternatif olarak perkutan yontemle tikayici cihazlarla kapatilmaktadir (2). Belirgin soldan saga dogru sant olusturan ve sag ventrikulun hacim yukunu artiran defektlerin transkateter yolla kapatma endikasyonu vardir (1). Bu yontemle tedavi edilen hastalarda hastanede kalis suresi cok daha kisadir, mortalitesi ve morbiditesi cerrahi tedaviye gore cok dusuktur. Bu makalede, merkezimizde sekundum ASD’lerin perkutan yolla kapatilmasina ait deneyimlerimizi ve takip sonuclarimizi paylasmayi planladik. Gerec ve yontemler

Aneurysm of the ascending aorta represents a rather rare disease condition. It may remain silent over the course of seve­ ral years, without symptoms or physical examination signs. While the principal causes in adults of what is generally an isolated aneurysm are degenerative processes and atherosclerosis, in children the presence of systemic disease must be carefully investigated. A 7­year­old male patient was consulted for a swelling in the right chest. While asymptomatic, he had a 1/6 early diastolic murmur at the left midsternal border and a right parasternal swelling on physical examination. Chest X­ray showed car­ diomegaly and an enlarged mediastinum (Fig. 1). Echocardiography found an aneurysmal dilatation of the ascending aorta immediately past the aortic valve, measuring 85 mm (z­score: +13.9) at its widest (Fig. 2) and mild aortic insufficiency was noted. Computerised tomographic angiography of the chest confirmed the presence of an aneu­ rysmal dilatation measuring 88 mm at its widest point, extending from the aortic root to the left subclavian artery ostium (Fig. 3). Genetic studies performed by cDNA array analysis in polymerase chain reaction showed the presence of an exon 10 deletion. This result was diagnostic for Marfan syndrome. Aortic valve was preserved during the operation and graft replacements of the ascend­ ing and transverse aorta were performed. No postoperative complications were seen. Even in the absence of other findings suggestive of Marfan syndrome, in particular with large aneurysms, this should be kept in mind in investigating a patient. Dissection and rupture of the aortic root is the most frequent cause of death in these cases, with a peak incidence in the third and fourth decades of life. The substantial mortality and morbidity of acute aortic events contrast with the important increase in life expectancy achieved by preven­ tative interventions. Composite valve graft and valve­sparing aortic root replacement procedures are performed under elective conditions with low morbidity and mortality.

Truncus arteriosus is an uncommon congenital cardiac abnormality which is characterized by a single arterial trunk origin from the heart that supplies both the systemic, pulmonary and coronary circulation. We present a preterm newborn female patient with type 2 truncusarteriosus, left superior vena cava and aberrant subclavian artery diagnosed with low dose dual-source cardiac computed tomography (CT). We discuss that low dose dual-source cardiac CT has more advantages than other imaging methods and it is an important modality for assessment of patients with conotruncal anomalies such as truncusarteriosus.