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There is limited data on the pattern and severity of myocardial injury in patients with COVID-19 vaccination associated myocarditis.We aimed to define the myocardial damage occurring after BNT162b2 vaccination, raise awareness about adverse reactions developing after vaccination, and determine the patterns and scope of Cardiac magnetic resonance imaging (MRI) findings.A total of 9 patients diagnosed with vaccine-associated myopericarditis were followed up.The mean age of the patient at diagnosis was 15.3 ± 1.0 (range: 14-17) years, and all patients were male. Seven patients presented with myocarditis symptoms after their second vaccine dose, one patient presented with pericarditis symptoms after his first dose, and the other patient presented with myocarditis symptoms after his booster dose. The median time at presenting to the hospital was 3 (range: 2-22) days. Seven (77.7%) patients had abnormal electrocardiography (ECG) findings, and the most prevalent finding was diffuse ST-segment elevation. Initial cardiac MRI results were abnormal in all patients, where 8 (88.8%) patients had late gadolinium enhancement, and 5 (55.5%) had myocardial edoema. Three patients showed local left ventricular wall-motion abnormalities. In their follow-up MRIs 3-6 months later, myocardial edoema was present in 2 (28.5%) patients, while late gadolinium enhancement was present in all patients (7/7, 100%, 2 patients did not have control MRI time). Hypokinetic segments were still present in one of the 3 patients. No negative cardiac events were observed in the short-term follow-up of any patient.Further follow-up evaluation and larger multicenter studies are needed to determine the clinical significance of persistent cardiac MRI abnormalities.

Objective: The aim of this study was to present our experiences on transcatheter patent ductus arteriosus (PDA) closure with different devices, mostly the Amplatzer Piccolo Occluder, in infants weighing between 2-10 kg. Material and Methods: In this study, the files of 31 patients who underwent transcatheter PDA closure between December 2019 and August 2022 were reviewed retrospectively. Results: Transcatheter PDA closure was performed on 31(14 female) infants weighing between 2-10 kg. The mean age of patients was 10.7±6.7 months (2-28), and the mean weight was 6.6±2 kg (3-9.9). The median narrowest diameter of the ductus was 2.2 mm (IQR 2-3) and the median ductus length was 6 mm (IQR 4.75-8). The procedural success rate of all interventional procedures was 88% (30 of 34). Complications occurred in a total of four patients including failure of device implantation in one patient, post-procedural device embolization in 2 patients, and the significant residual shunt in one patient. None of the patients required surgery. In 34 interventional procedures, 3 of which were reintervention, 34 devices were used. Twenty-seven (79%) of them were Amplatzer Piccolo Occluder. The median fluoroscopy and procedural times were 10.5 minutes (IQR 7.25-18.5) and 40 minutes (IQR 35-57.5) respectively. The mean duration of follow-up was 10.3±8.8 months (1-32 months). Conclusion: In our experience, transcatheter treatment of PDA with the Amplatzer Piccolo Occluder device which was our first choice for appropriate duct anatomy and size in infants weighing between 2-10 kg, is safe and effective.

Objective: The aim of this study was to evaluate the safety and efficacy of transcatheter atrial septal defect (ASD) closure guided by fluoroscopy and transthoracic echocardiography (TTE) and to present our experiences. Material and Methods: In this study, we evaluated 108 patients’ files taken to the catheter laboratory for transcatheter ASD closure retrospectively. The procedure was abandoned in ten patients because of septum device disproportion (6) and deficient rims (4), mainly inferior vena cava rim. Results: Transcatheter ASD closure guided by TTE was performed in 98 patients (59 female). The mean age of patients was 9.5±6 years (2.6-46), and the mean weight was 30.3±15.3kg (12-80). TTE-guided ASD closure was successfully performed in 92 of 98 (94%) patients. The median largest ASD diameter measured by TTE was 10.75 mm (interquartile range (IQR) 9.12-14. The median stretched balloon diameter measured by fluoroscopy was 14 mm (IQR 12.4-18). The median device waist diameter was 14 mm (IQR 13-18), the median device left atrial (LA) disk diameter was 28 mm (IQR 26-31), and the median ratio of LA disc diameter to total septal diameter was 75% (IQR 68-81). The median fluoroscopy and procedural times were 8 minutes (IQR 5.6-13.75) and 36.5 minutes (IQR 30-49) respectively. Conclusion: Transcatheter ASD closure guided by TTE and fluoroscopy is safe and effective in children, adolescents, and young adults.

A 16-month-old girl was referred for tachycardia and upper respiratory tract infection. Echocardiographic examination revealed pericardial effusion, mild mitral regurgitation, and left ventricle systolic dysfunction. Patient was positive for Parainfluenza type 4 virus. Her laboratory tests revealed increased troponin I level. The patient was treated with intravenous immunoglobulin considering acute viral myopericarditis. Two weeks after treatment, midventricular hypertrophy was detected.

Tetralogy of Fallot with an aortopulmonary window and double aortic arch is very rare. This complex coexistence may be over a wide clinical spectrum. Herein, we present an asymptomatic 8-day-old infant who was diagnosed as having tetralogy of Fallot, double aortic arch, and an aortopulmonary window using transthoracic echocardiography while being examined for microcephaly.

Objective: β-thalassemia major is an inherited hemoglobin disorder resulting in chronic hemolytic anemia. Cardiac involvement is the main cause of death in patients. Speckle-tracking echocardiography is feasible method for the evaluation of cardiac function via an assessment of the longitudinal deformation of the myocardium through the cardiac cycle. The aim of our study is to evaluate the association of vitamin D deficiency and deformation of the left ventricular myocardium measured by speckle-tracking echocardiography in children with thalassemia major.Methods: In this prospective study, 33 thalassemic patients with vitamin D deficiency were enrolled. Cardiac magnetic resonance T2* value, conventional echocardiography and speckle tracking, left ventricle longitudinal and circumferential strain values were measured. Vitamin D level 30 ng/ml considered sufficient. Myocardial functions of patients with vitamin D deficiency or insufficiency were evaluated by speckle-tracking echocardiography before and after vitamin D replacement.Results: The mean age of our patients was 15.4 ± 3.09 years, male / female ratio was 18/15. Vitamin D level was deficient in 30 (90%) and insufficient in 3 (10%) of our patients. Speckle-tracking analysis showed a significantly decreased absolute value of left ventricle global longitudinal strain before vitamin D replacement. A significant improvement in the global longitudinal strain of left ventricular after vitamin D replacement was detected (pConclusion: Vitamin D deficiency are frequently observed and vitamin D deficiency causes decreased contractility and leads to an increase in cardiac iron in thalassemic patients. In our study, we observed that our patients cardiac functions had improved after vitamin D replacement therapy. Further prospective studies are needed to evaluate the relationship between vitamin D levels and cardiac function in patients with these patients.

Coronavirus disease of 2019 (COVID-19) is a cause of significant morbidity and mortality worldwide. Although COVID-19 clinical manifestations are mainly respiratory, major cardiac complications are being reported. The mechanism of cardiac injury and arrhythmias is unclear. Also, drugs currently used to treat the COVID-19 may prolong the QT interval and may have a proarrhythmic propensity. The study aims to investigate the effects of COVID-19 infection with asymptomatic and mild symptoms on trans-myocardial repolarization parameters in children without treatment. A total of 105 COVID-19 patients were compared with 40 healthy children. The patient and control group data were compared by calculating the QT interval, corrected QT (QTc), QT dispersion (QTd), QTc dispersion (QTcd), Tp-e, Tp-e dispersion, Tp-e/QT ratio, and Tp-e/QTc ratio on the 12-lead surface electrocardiogram. The mean age was determined as 11.2 ± 0.3 years in the patient group, and 10.8 ± 2.1 years in the control group. In the COVID-19 group, QTd, QTcd, Tp-e, Tp-e dispersion, Tp-e/QT ratio and Tp-e/QTc ratio were statistically higher than the control group. The ventricular repolarization was impaired even in asymptomatic children with COVID-19 infection. These results suggest the need to further assess the long terms risks of prolonged QT dispersion in the setting of COVID-19 infection.

OBJECTIVE: Anthracyclines are widely used in the treatment of acute lymphoblastic leukemia (ALL). However, cardiotoxicity is the most critical side effect that requires dose limitation. It is thought to occur at first exposure, but the clinical presentation may occur years later. In this study, we aimed to determine the time of initial damage and cardiotoxicity that develops in children with ALL. METHODS: In this prospective study, 13 patients with newly diagnosed intermediate-risk precursor B cell ALL treated with the ALL-IC BFM 2009 protocol were included. Conventional echocardiography, tissue Doppler imaging (TDI), and speckle-tracking echocardiography (STE) were performed in all the patients before chemotherapy, after completing the induction phase, and at the end of the reinduction phase. RESULTS: The mean age of the patients was 7.8±4.6 (3.1–16.3) years. Myocardial velocity during systole (S(m)) determined by TDI at the interventricular septum significantly decreased during the induction phase. Despite a decrease in STE parameters, a statistically significant reduction was determined in the global longitudinal strain rate at both left and right ventricles at the end of the induction. Nevertheless, a statistically significant increase was observed among the conventional echocardiographic findings in the left ventricular end-diastolic diameter at the end of the reinduction. CONCLUSION: During the treatment of ALL, subclinical anthracycline-associated cardiotoxicity develops in the early stages of treatment. The findings detected by TDI and STE could be missed by conventional echocardiography. We recommend evaluating patients with these newly developed techniques to detect subclinical cardiotoxicity at an early stage and starting appropriate therapy on time.

Cocukluk cagi kanserlerinde sag-kalim sonrasi kardiyovaskuler hastalik riski kontrol gruplarina gore 8-10 kat daha fazla bulunmustur. Bu konuda yapilan calismalar kardiyovaskuler hastaliklardan olum riskinin tedavi tamamlandiktan 5-10 yil sonra belirgin arttigini, tani uzerinden 30 yildan uzun sure gecen hastalarda kardiyak toksisiteye bagli hastaliklarin en onemli mortalite nedeni oldugunu gostermistir. Bu nedenle tedavi sirasinda ve sonrasinda hastalarin duzenli takibi gerekmektedir. Ornegin, antrasiklin tedavisi verilen ve tedavi uzerinden 10 yildan uzun sure gecen hastalarin yaklasik yarisinda, hicbir semptom bulunmasa bile subklinik kardiyak hastalik bulunmaktadir. Sag-kalim sonrasi tum hastalar dikkate alindiginda bu rakam %25’in uzerindedir. Bu sonuclar subklinik bulgularin yillar icerisinde kotulesecegini gostermektedir. Cocuklarda kemoterapiye bagli toksisiteyi ve uzun donemde toksisitenin nasil sonuclanacagini tahmin etmek her zaman mumkun degildir. Direkt miyokard hasari yaninda miyokardiyal fonksiyon kaybi yada vaskuler hemodinaminin bozulmasi gibi kemoterapiye bagli degisiklikler kardiyovaskuler sistemi etkileyebilir. Kardiyovaskuler sistem ayrica radyasyon hasarina da aciktir. Temel mekanizma interstisyel fibrozis gelismesidir. Tedavisi devam eden hastalarda kardiyovaskuler sistem ile ilgili bazal degerlerin elde edilmesi ve erken bulgularin saptanabilmesi amaciyla duzenli aralarla tanisal degerlendirme yapilmalidir. Tedavisi tamamlanan hastalarda ise genel kabul gormus yaklasim asemptomatik hastalarda bu degerlendirmenin 5 yilda bir yapilmasi, semptomatik hastalarda ise semptomlar basladiginda yapilarak bulgulara gore sikliginin belirlenmesi gerektigi seklindedir. Ayrica en dusuk etkili dozun belirlenmesi, alternatif doz ve uygulama metodlarinin gelistirilmesi, protokollere kardiyak koruyucu ajanlarin eklenmesi, radyoterapi kullanimi ve dozunun azaltilmasi ile kardiyak toksisiteye bagli hastalik insidansinin azaltilmasi uzerine yeni tedavi protokolleri gelistirilmelidir. Sag-kalim sonrasi obezitenin onlenmesi, metabolik sendrom ve hipertansiyon ile mucadele, hiperlipideminin onlenmesi, erken ateroskleroz gelisiminin takibi, aritmi acisindan dikkatli olunmasi, sigara, alkol kullanimi ve madde aliskanligi ile mucadele de onemlidir.

Burkitt lymphoma manifesting as an intracardiac mass is a rare entity. This report describes the case of a 10-year-old boy who presented with an intracardiac mass and tumor thrombosis in the anterior mediastinum that proved to be Burkitt lymphoma. The LMB-96 chemotherapy protocol was given and at the end of the treatment there was still residual mass. A biopsy was performed and the pathology revealed thymus tissue. The patient has been in complete remission for 3 months. Burkitt lymphoma has a short doubling time and an intracardiac lesion can become life threatening. Early recognition and prompt treatment are crucial in achieving optimal outcomes.

Amaç: Terapötik hipotermi tedavisi alan hipoksik iskemik ensefalopatili (HİE) yenidoğanların kardiyak bulgularının; biyobelirteçler, EKG ve ekokardiyografi ile değerlendirilmesi ve bu belirteçlerin hipoksik myokard hasarı şiddetinin belirlenmesindeki etkisinin araştırılması amaçlanmıştır.Gereç ve Yöntemler: Evre II ve III HİE tanısı alan ve terapötik hipotermi uygulanmış 50 yenidoğan bebeğin verileri retrospektif olarak incelendi. Demografik özellikler, sistemik ve kardiyak muayene bulguları, serum CK-MB, troponin-I, EKG ve ekokardiyografi raporları kaydedildi. Evre II ve Evre III HİE’li hastaların bulguları karşılaştırıldı.Bulgular: Elli olgunun 11’inde (%22) miyokardiyal disfonksiyon saptandı. EKG kayıtlarında tüm olgular normal sinüs ritmine sahipti. EKG’de patolojik bulgu evre II’de 2 (%8.3) olguda, evre III’de ise 5 (%19.8) olguda mevcuttu. Serum CK-MB ve troponin-I olguların 30’unda (%60) yüksek izlendi. Tüm olguların 5’inde (%10) orta derecede triküspit yetersizliği, 1’inde (%2) şiddetli triküspit yetersizliği, 4’ünde (%8) sağ ventrikül hipokinezisi, 6’sında (%12) sol ventrikül hipokinezi ve 1’inde (%2) biventriküler hipokinezi mevcuttu. Evre III HİE’li olgularda enzim düzeylerinin daha yüksek, EKG ve EKO anormalliklerinin daha yaygın olduğu görüldü (p, Objective: The aim of this study is to evaluate the severity of cardiac complications of hypoxic ischemic encephalopathy (HIE) according to the degree of hypoxia and to evaluate the efficacy of cardiac biomarkers, electrocardiography (ECG) and echocardiography (ECHO) for myocardial injury.Material and Methods: Fifty term babies with HIE without any additional disease were selected. Myocardial dysfunction was evaluatedusing clinical examination, serum CK-MB, troponin-I and ECG and ECHO.Results: According to Sarnat and Sarnat classificiation, 24 neonates were diagnosed stage II and 26 neonates were diagnosed stage IIIHIE. Among these 50 cases, 11 (22%) had evidence of myocardiac dysfunction. All the cases had normal sinus rhythm. ECG changes(ST depression, T wave inversion, pathological Q wave as signs of ischemia) were present in 2 (8.3%) cases in the stage II, and 5 (19.8%)cases in the stage HIE group. ECG showed. Serum levels of troponin-I and CK-MB were increased in 30 (60%) patients. Moderatetricuspid regurgitation was present in 5 (10%), severe tricuspid regurgitation in 1 (2%), right ventricular hypokinesia in 4 (8%), left ventricularhypokinesia in 6 (12%) and biventricular hypokinesia in 1 (2%) neonate among all the cases. Enzyme levels were higher, ECG and ECHOabnormalities were more common in the neonates with stage III HIE (p

Objective: Echocardiography is an important and pratical diagnostic tool for early diagnosis and follow-up of CHD. In our study, we aimed to evaluate the frequency, distribution of diagnoses and risk factors of cases diagnosed with CHD in the neonatal intensive care units (NICU) and neonatal services.Material and Methods: 2766 patients who were followed in NICU and neonatal services between May 2013 and May 2017 were retrospectively analyzed. 365 newborn diagnosed with CHD of 762 babies were requested pediatric cardiology consultation were included in the study. Cases diagnosed with patent foramen ovale and patent ductus arteriosus (PDA) that closed in first 3 days were excluded from study.Results: The frequency of CHD in our center was 13.2%. Most of the cases were term births, 60.5% of them were male and 30.9% were female.The most common reasons for hospitalization were respiratory diseases (28.3%). There was kinship between the parents of 19.9%.The most common reasons for cardiology consultation were murmur (27.4%), respiratory distress (15.9%) and prematurity (10.1%). Ninety six point two percent of the patients had acyanotic CHD, and 3.8% had cyanotic CHD. The most common acyanotic CHD, in order of frequency, were atrial septal defect (ASD) (40.8%), pulmonary stenosis (PS) (15.3%), PDA (13.4%), ventricular septal defect (VSD) (9.3%). The most common cyanotic CHD, in order of frequency, were hypoplastic left heart syndrome (1.1%), pulmonary atresia (0.8%) and double outlet right ventricle(0.8%). Seven point one percent of the patients had a genetic syndrome accompanying CHD, and Down syndrome was in the first place with 38.5%. Major extracardiac anomaly was found in 10.1% of the patients. While 9.3% were followed up as diabetic mothers’ babies, 20.6% of them had septal hypertrophy. One-time pulse oximetry measurements were made in our study. In 72.7% of the cyanotic CHD group SatO2 was less than 90%, whereas in 76.3% of the acyanotic CHH group, SatO2 was 95% and above, and it was found to be significantly different (p, Amaç: Ekokardiyografi DKH’nin erken tanı ve izleminde önemli ve pratik bir tanı aracıdır. Çalışmamızda yenidoğan kliniğinde DKH tanısı alan olguların sıklığı, tanılarının dağılımı ve risk faktörleri açısından değerlendirilmesi amaçlandı.Gereç ve Yöntemler: Mayıs 2013-Mayıs 2017 tarihleri arasında YD servis ve yoğun bakımda takip edilen 2766 hasta geriye dönük incelendi. Kardiyoloji konsültasyonu istenen 762 term ve preterm YD’den DKH tanısı alan 365 bebek çalışmaya dahil edildi. Patent foramen ovale (PFO) ve ilk 3 günde kapanan patent duktus arteriozus (PDA) olguları çalışmaya dahil edilmedi.Bulgular: Merkezimizde DKH sıklığı %13.2 olarak bulundu. Çoğunluğu term olan olguların %60.5’i erkek, %39.5’u kızdı. Hastaların en sık yatış nedeni solunumsal (%28.3)’dü. Yüzde 19.9’unun ebeveynleri arasında akrabalık mevcuttu. En sık kardiyoloji konsültasyonu istenme nedeni üfürüm (%27.4), solunum sıkıntısı (%15.9) ve prematürite (%10.1)’di. Hastaların %96.2’sinde asiyanotik, %3.8’inde siyanotik DKH vardı. Yenidoğanlarda en sık görülen asiyanotik DKH sırasıyla atriyal septal defekt (ASD) (%40.8), pulmoner darlık (PD) (%15.3), PDA (%13.4), ventriküler septal defekt (VSD) (%9.3) iken, siyanotik DKH sırasıyla hipoplastik sol kalp sendromu (HSKS) (%1.1), pulmoner atrezi (PA) (%0.8) ve çift çıkışlı sağ ventrikül (ÇÇSV) (%0.8)’di. Hastaların %7.1’inde DKH’ye eşlik eden genetik sendrom mevcut olup, bunların içinde %38.5 ile Down Sendromu ilk sırada yer almaktaydı. Hastaların %10.1’inde major ekstrakardiyak anomali saptanmıştır. Diyabetik anne bebeklerinin %20.6’sında septal hipertrofi görüldü. Çalışmamızda tek zamanlı nabız oksimetre ölçümleri yapılmış olup siyanotik DKH grubunun %72.7’sinde SatO2’nin

In children, cardiac involvement can be observed during the course of COVID-19, which is mostly mild, and COVID-19 may develop in children who have previously been known to have congenital or acquired heart disease. The subject of this article is how these children should be evaluated cardiacly, and how the follow-up and treatment of children with heart disease will be managed during the pandemic. Considering the articles and guides published in the literature, some determinations were made and the suggestions developed were presented in this article., Çocuklarda çoğu zaman hafif seyreden COVID-19 seyri sırasında kardiyak tutulum görülebileceği gibi, daha önce doğumsal yada edinsel kalp hastalığı olduğu bilinen çocuklarda da COVID-19 gelişebilir. Bu çocukların kardiyak açıdan nasıl değerlendirilmesi gerektiği, ayrıca kalp hastalığı bulunan çocukların takip ve tedavilerinin pandemi süresince nasıl yönetileceği bu makalenin konusudur. Literatürde yayınlanmış olan makale ve rehberler dikkate alınarak bazı saptamalar yapılmış ve geliştirilen öneriler bu yazıda sunulmuştur.

The aim was to assess the utility and feasibility of a comprehensive cardiac screening protocol in young athletes before participation in sports. A total of 380 athletes referring before participation in sports, between April, 2014 and April, 2015, were included in this study. The mean age was 12.4 years. A screening protocol has been applied to all, including personal and family history, physical examination, 12-lead electrocardiography, transthoracic echocardiography, 24-hour rhythm Holter analysis, and treadmill exercise test. The most frequent complaints were chest pain in 19 (5%), dyspnoea in 13 (3.4%), and dizziness and fainting in five patients (1.3%) on exercise. There was sudden death and arrhythmia in 41 patients (10.7%) owing to family history. Heart murmur was present in 20 (5.2%) and hypertension in 10 patients (2.6%) on physical examination. The 12-lead electrocardiography was abnormal in 9 patients (2.4%). The findings of transthoracic echocardiography were insignificant in 47 patients (12.3%) and in five patients (1.3%) a haemodynamically important condition was detected. The 24-hour rhythm Holter analysis was abnormal in six patients (1.5%). There were significant ST changes in two patients (0.5%) on treadmill exercise test with normal findings on myocardial perfusion scans. No significant relation was present between findings of screening protocol and transthoracic echocardiography, 24-hour rhythm Holter analysis, or treadmill exercise test results.Pre-participation screening in young athletes should consist of a targeted personal history, family history, physical examination, and 12-lead electrocardiography. Other tests should be applied only if the screening indicates the presence of a cardiovascular disease.

Paediatric cardiology is arguably the sub-specialty in which the greatest advances have been made in both disease diagnosis and treatment over the past half a century. Paediatric cardiology emerged as a discipline in the 1930s. Since then, advances in imaging techniques such as echocardiography, angiography, CT, or magnetic resonance and extracorporeal circulation have provided excellent diagnosis and treatment of CHD. The pioneers of paediatric cardiology are more than eponyms, for each used in new and original ways the tools and concepts available in his or her era. This brief overview of the history of paediatric cardiology on stamps begins from William Harvey up to our own time, and includes the milestones in paediatric cardiology.

We present a newborn diagnosed with Beckwith-Wiedemann syndrome and hypoglycemia, and developed pulmonary hypertension due to initiated diazoxide treatment because of these indications. Beckwith-Wiedemann syndrome was considered due to abdominal wall defect, macrosomia, macroglossia and hypoglycemia in a 34 week newborn with respiratory distress, hypoglycemia and syndromic appearance. Blood glucose level was measured as 1 mg/dL. Oral feeding, glucose infusion, prednisolone and then diazoxide treatment were started. At the first admission transthoracic echocardiographic examination, ventricular and atrial septal defects were detected. Control echocardiography performed under diazoxide treatment, showed development of enlarged right heart chambers, severe tricuspid regurgitation, and pulmonary hypertension. The development of pulmonary hypertension was thought to be related to diazoxide treatment. Diazoxide was discontinued after the patient became normoglycemic during follow--up period. Subsequently performed echocardiography revealed that the systolic pulmonary artery pressure regressed to 20 mmHg, and cardiac chambers returned to their physiologic balance. The patient without any problem during monitorization was discharged with the recommendation to attend further controls visits. In this case report, it was aimed to be reminded that pulmonary hypertension can develop due to diazoxide treatment, and it can regress with discontinuation of diazoxide. Besides, transthoracic ECHO should be performed to check for the development of pulmonary hypertension in newborns treated with diazoxide.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors. The clinical presentation of pediatric PPGLs is highly variable. In cases with pheochromocytoma (PCC), excess catecholamine may stimulate myocytes and cause structural changes, leading to life-threatening complications ranging from stress cardiomyopathy (CM) to dilated CM. Herein, we report the case of catecholamine-induced myocarditis in a child with asymptomatic PCC. A 12-year-and-2-month-old male patient with a known diagnosis of type-1 neurofibromatosis was brought to the emergency department due to palpitations and vomiting. On physical examination, arterial blood pressure was 113/81 mmHg, pulse was 125/min, and body temperature was 36.5 °C. Laboratory tests showed a leucocyte count of 12.8x103 μL/L and a serum C-reactive protein level of 1.1 mg/dL (Normal range: 0-0.5). Thyroid function tests were normal, while cardiac enzymes were elevated. Electrocardiogram revealed no pathological findings other than sinus tachycardia. The patient was diagnosed with and treated for myocarditis as echocardiography revealed a left ventricular ejection fraction of 48%. Viral and bacterial agents that may cause myocarditis were excluded via serological tests and blood cultures. Blood pressure, normal at the time of admission, was elevated (140/90 mmHg) on the 5th day of hospitalization. Magnetic resonance imaging revealed a 41x46x45 mm solid adrenal mass. The diagnosis of PCC was confirmed by elevated urinary and plasma metanephrines. The patient underwent surgery. Histopathology of the excised mass was compatible with PCC. It should be kept in mind that, even if there are no signs and symptoms of catecholamine elevation, CM may be the first sign of PCC.

PH is a rare condition with high mortality rate after pediatric HSCT. As clinical presentation is non-specific and may mimic other conditions, a high degree of suspicion is required for diagnosis. Here, we present a patient with stage-IV neuroblastoma who developed PAH after autologous HSCT. After exclusion of other causes of PH, we regarded that this condition was secondary to HSCT.

Summary– Atrioventricular (AV) block in the neonatal period is a rare disorder. It is frequently associated with underlying structural congenital heart disease and maternal lupus. Presently described is premature baby who developed 2: 1 AV block and congestive heart failure due to hypocalcemia. Dramatic clinical improvement was observed following treatment of intravenous 10% calcium gluconate. Therefore, it is suggested that serum calcium level of newborns with AV block and congestive heart failure be measured.

Objective: To determine the indication and necessity of echocardiographic assessment and therapeutic interventions in critically ill children. Methods: A total of 140 children, including 75 mechanically ventilated (MV) and 65 spontaneously breathing (SB) children, who were admitted consecutively from March to August 2013 were evaluated prospectively. Data regarding the indication for echocardiography and therapeutic approaches used were documented. For evaluating disease severity, the Pediatric Risk of Mortality Score III (PRISM) was ascertained. The correlation between PRISM score and the requirement of echocardiographic evaluations were analyzed. Results: Patients ages were between 45 days to 18 years. The male-to-female ratio was 1.33. In 35.4% patients who underwent echocardiographic evaluation, no definitive alteration occurred in treatment approach, whereas in the remaining 64.6% patients, decisive or supplemental information was gathered. Echocardiography was indicated in 88% MV children and 46.2% SB children. Echocardiographic evaluation was necessary in MV children and there was a positive correlation between the PRISM score and the requirement of echocardiographic assessment (p

The objective was to compare the efficacy and safety of naproxen (NXN) to acetylsalicylic acid (ASA) in the treatment of acute rheumatic fever (ARF). The data of 338 children were retrospectively analyzed. The patients were grouped according to joint and valve involvement and also drug chosen [methyl prednisolone (mPSL), ASA or NXN]. The treatment results and adverse events in each group were compared. The mean age was 10.3 years and the median follow-up was 62 months. Median time for normalization of acute phase reactants was 1 week in patients given steroids and 2 weeks in patients given ASA or NXN. ASA was replaced with NXN in 18 patients (10.2%) due to hepatic toxicity. The rate of rebound, recurrence and the prevalence of rheumatic valve disease were not different in patients given NXN, ASA or mPSL. In conclusion, NXN is a safe and effective alternative to ASA in the treatment of ARF in children.

Objective: We aimed to examine the etiology of chest pain in children and to clarify the most common causes in this study. Material and Methods: A total of 603 patients who applied to our hospital with chest pain between December 2010 and July 2011 were retrospectively reviewed.results: The female and male percentages of the study group were 46.8% and 53.2% respectively. The mean age of the patients was 11.8 ± 2.7 years. The etiology of the chest pain was related to the musculoskeletal system in 46.9%, the cardiovascular system in 17.5%, the respiratory system in 5.1%, and the gastrointestinal system in 3.3% and was psychogenic in 3%. No etiology was found in 21.4% of the patients and they were considered to be idiopathic. Other diagnoses such as connective tissue disease, Marfan syndrome, breast swelling and herpes zoster were present in 2.8% of the patients and these were classified as miscellaneous.conclusion: Chest pain in children should be investigated carefully, Amaç: Hastanemize göğüs ağrısı yakınması ile başvuran çocukların etiyolojik açıdan incelenmesi ve en sık karşılaşılan sebeplerin belirlenmesi hedeflenmiştir.Gereç ve Yöntemler: Çalışma kapsamında hastanemize Aralık 2010 ile Temmuz 2011 tarihleri arasında göğüs ağrısı yakınması ile başvuran 603 olgu geriye dönük olarak incelendi.Bulgular: Çalışma grubundaki hastaların %46.8’i kız, %53.2’si erkekti. Hastaların yaş ortalaması 11.8 ± 2.7 (5.5–18) yıldı. Hastaların %46.9’unda kas iskelet sistemi, %17.5’inde kardiyovasküler sistem, % 5.1’inde solunum sistemi, %3.3’ünde gastrointestinal sistem, %3’ünde psikojenik nedenli göğüs ağrısı tespit edildi. %21.4’ünde ise göğüs ağrısının nedeni bulunamadı ve idiyopatik göğüs ağrısı olarak değerlendirildi. Hastaların %2.8’inde göğüs ağrısının nedeni kollajen doku hastalığı, Marfan sendromu, meme büyümesi ve zona gibi diğer nedenlere bağlandı.Sonuç: Çocuklardaki göğüs ağrısının ayrıntılı araştırılması gerekliliği sonucuna varılmıştır.

We have reported a patient with aortopulmonary window (APW) simulating truncus. In classically defined APW, aorta and main pulmonary artery separate again after a distance from the window and form the aortic arch and pulmonary arteries. However, in our case, there was no separation and they formed a very large pouch from which the great vessels originated. Thus, we believe that we have presented the first case of APW simulating truncus, but with two separate semilunar valves.

This report describes a patient who had bilateral facial nerve paralysis, external ophthalmoplegia, absence of pectoralis major muscle at right side, ipsilateral hand and foot, and controlateral hand anomalies. To our knowledge, this is the first patient with Poland syndrome reported in combination with Moebius syndrome, presenting with controlateral hand and ipsilateral foot anomalies.

This report describes a patient who had bilateral facial nerve paralysis, external ophthalmoplegia, absence of pectoralis major muscle at right side, ipsilateral hand and foot, and controlateral hand anomalies. To our knowledge, this is the first patient with Poland syndrome reported in combination with Moebius syndrome, presenting with controlateral hand and ipsilateral foot anomalies.