Search

Cardiac myxoma is rare in children. Myxomas are exceedingly rare in infancy. Right atrial myxomas were recorded in a small number of case reports involving infants worldwide. We report the case of a 2-month-old infant with giant right atrial myxoma. The case presented to our hospital with respiratory distress, and had pericardial and pleural effusion. Diagnosis of cardiac tumor was made with the aid of computerized tomography scan and echocardiogram. The tumor size was 3.1 × 3.4 × 3.9 cm. The patient worsened rapidly and had sudden cardiac arrest which did not respond to interventions. Postmortem cardiac autopsy confirmed the diagnosis of myxoma on gross examination and histology. This article aims to focus attention to the atypical size and location of this atrial myxoma, causing diagnostic difficulty in this infant.

Background:Hematuria is an important and commonsign of urinary system diseases in children. Careful assessment is needed fordefinitive diagnosis and appropriate management. In this study we aimed toinvestigate the demographic and clinical characteristics of patients who wereadmitted to our pediatric nephrology clinic with hematuria and to determine thefeatures that will guide the diagnosis. Methods:Medical charts of 370 patients whowere referred to our clinic in a period of four years with gross or microscopichematuria were evaluated retrospectively. Demographical data, clinical andlaboratory findings, imaging modalities and diagnosis of the patients wereinvestigated. Findings: Of the patients 195 (52.7 %) were boys and 175 (47.3%) weregirls and the majority of the patients were in the age group of 6-10 years. Majorpresenting feature was red colored urine (64.1 %). Non-glomerular causes werefound to be significantly higher among patients with macroscopic hematuria (p=0.002).Acute poststreptococcal glomerulonephritis formed the majority of glomerular hematuria and urinary systeminfections formed the majority of non-glomerular hematuria causes respectively. Conclusions: This study suggests that a carefulhistory, detailed physical examination and clinical evaluation may elucidatethe etiology in the majority of the patients with hematuria. Advanced imagingmodalities and renal biopsy are indicated only in selected cases., Amaç: Hematüri çocuklarda üriner sistem hastalıklarının önemli bir bulgusudur. Altta yatan ciddi hastalıkların belirtisi olabileceğinden ayırıcı tanı için dikkatli bir değerlendirme ve iyi bir klinik yaklaşım gerektirmektedir. Bu çalışmada pediatrik nefroloji kliniğimize hematüri nedeni ile başvuran hastaların demografik ve klinik özelliklerinin incelenmesi ve tanıya yol gösterecek özelliklerin belirlenmesi amaçlandı.Hastalar ve Yöntem: Bu çalışma, kliniğimizde makroskopik ve/veya mikroskopik hematüri nedeniyle izlenmiş olan 370 hastanın dosyası retrospektif olarak değerlendirilerek gerçekleştirildi. Hastaların demografik verileri,l aboratuar sonuçları ve klinik bulguları, görüntüleme tetkikleri ve nihai tanıları araştırıldı.Bulgular: Hastaların 195’i (%52.7) erkek, 175 (%47.7)’si kızdı ve çoğunluğu 6-10 yaş grubunda yer almaktaydı. Hastaların 234 ‘ü (%63.2) makroskopik hematüri ile başvururken , 44’ünde (%15.3) glomerüler hematüri saptandı. Makroskopik hematüri grubunda non-glomerular nedenler anlamlı olarak yüksek bulundu. Glomerüler hematüri nedenleri arasında ülkemizde halen yaygın olarak saptanan akut poststreptokokkal glomerülonefrit (APSGN), non glomerüler nedenlerden de üriner sistem enfeksiyonu en yüksek oranda görülen hastalıkları oluşturmaktaydı. Tartışma: Bu çalışma dikkatli bir öykü, ayrıntılı fizik muayene ve klinik değerlendirme ile hematüri ile başvuran hastaların büyük çoğunluğunda etiyolojinin aydınlatılabileceğini göstermektedir. İleri görüntüleme tetkikleri ve renal biyopsi sadece seçilmiş vakalarda endikedir.

Echocardiography is the mainstay of screening and disease surveillance in isolated left ventricular non-compaction (iLVNC). The aim of our study is to determine the early regional and global myocardial functional changes and whether the myocardial changes that cannot be detected by conventional echocardiography could be detected by tissue Doppler imaging (TDI) or two-dimensional speckle-tracking echocardiography (STE) in iLVNC cases without symptoms. Longitudinal and circumferential strain (S) and strain rates (SR) as determined by STE in 20 children aged 12.1 ± 3.3 years was compared with those in 20 controls. All children underwent echocardiographic assessment using two-dimensional, tissue Doppler and speckle-tracking echocardiography. iLVNC patients who had normal systolic function by ejection and shortening fractions were included in this study. According to the TDI in all three segments [the non-compacted (NC), neighboring NC (NNC) and compacted (C) segments], isovolumic contraction time, isovolumic relaxation time and myocardial performance index values were significantly higher, while ejection time were significantly lower in the iLVNC group. According to STE in two segments (NC and NNC-segments) longitudinal S and SR values and also circumferential S and SR values were significantly lower in the iLVNC group compared with the control group; whereas, in the global measurements both longitudinal and circumferential S and SR values in all three segments were significantly lower in the iLVNC group compared with the control group. We believe that TDI and STE that evaluates myocardial deformation can be used for the detection of early myocardial dysfunction in the iLVNC patients who are subclinical and whose left ventricular functions were detected as normal by conventional methods with normal ejection and shortening fractions.

Objective: Dilated cardiomyopathy (DCM) is a disorder featuring left ventricular dysfunction, heart failure, and a poor prognosis. The etiology is still unclear, despite diagnostic and therapeutic developments. This study was an evaluation of factors affecting the life span of a group of idiopathic DCM patients. Methods: A total of 79 patients from between October 2005 and October 2017 with a diagnosis of idiopathic DCM were evaluated retrospectively. Demographic characteristics, clinical information, left ventricular function, treatment, and follow-up of the patients were reviewed based on hospital records. Age, gender, parental consanguinity, cardiomegaly on telecardiography, reduced ejection fraction (EF) and shortening fraction (SF), degree of mitral regurgitation, and intracardiac thrombosis were determined to affect prognosis. Results: The patients were aged 20+-60 months, and the male/female ratio was 1.02/1. The patients most frequently presented with heart failure signs and symptoms (n=59, 74.7%). The most common physical examination findings were a murmur (n=53, 67.1%) and tachycardia (n=48, 60.8%). Cardiomegaly was observed on telecardiography in 73.4% of the patients. The EF and SF values were 35.7+-12.6% and 17.3+-6.5%, respectively. In all, 42 (53.2%) patients had mitral regurgitation of grade 2 or higher. The duration of follow-up was between 1 and 156 months (20+-34.9 months). Intracardiac thrombosis was detected in 4 (5.1%) patients. The mortality rate was 36.7%. When the prognostic factors were compared according to survival time, it was determined that survival was reduced in cases of parental consanguinity, low EF, and cardiomegaly. Conclusion: The most important negative markers affecting the length of survival of DCM patients were parental consanguinity, cardiomegaly detected on telecardiography, and a reduced EF level.

15-year-old girl having a seizure was brought to the emergency room. She took 1.5 g propafenone for suicidal intention. She had metabolic acidosis. Long QRS interval and ST elevation in leads V1 through V3 were seen on electrocardiography. Background: Brugada syndrome is an inherited arrhythmogenic disease that may cause sudden cardiac death due to ventricular fibrillation in young adults. Brugada syndrome caused by propafenone intoxication has been noted rarely in the literature. We report a rare case, Brugada phenocopy due to propafenone intoxication and its treatment. Case Report: A 15-year-old girl having a seizure was brought to the emergency room. She took 1.5 g propafenone (Rythmol, Abbott, Chicago, IL, USA) for suicidal intention. She had metabolic acidosis. Long QRS interval and ST elevation in leads V1 through V3 were seen on electrocardiography. After bicarbonate infusion for 4 hours, haemodynamic and neurologic findings were recovered, and all electrocardiography abnormalities disappeared. The Brugada-like electrocardiography pattern was not recognized in her surface and 24-hour Holter electrocardiography at follow-up. Ajmaline challenge test was negative 2 weeks later. Conclusion: Absence of symptoms and documented ventricular tachycardia, negative challenge test, and a negative family history demonstrated that the Brugada phenocopy was a transient finding in this case and related to propafenone intoxication.

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.

Amaç: Çocuklarında üfürüm saptanan ailelerin endişe düzeyinin, çocuk kardiyolojisi kliniğine başvurma farkındalığının ve çocuk kardiyolojisi uzmanından beklentilerinin belirlenmesidir.Gereç ve Yöntemler: Genel muayene sırasında üfürüm saptanan ve çocuk kardiyolojisi kliniğine ilk kez başvuran hastalar çalışmaya alındı. Çalışmaya alınan hastalarda patolojik/masum üfürüm ayırımı yapılmadı. Mayıs-Ağustos 2013 tarihleri arasında kliniğimize başvuran 354 aileye 7 sorudan oluşan anket verilerek soruları cevaplamaları istendi.Bulgular: Çocuklarında üfürüm olduğunu öğrendiklerinde ailelerin içinde bulundukları endişe düzeyi ortalama 10 üzerinden 8 puandı. Ailelerin çocuk kalp hastalıkları ile ilgilenen bir bölümün varlığının farkındalığı %60.7 iken, beklentileri sorulduğunda; %8.4’ü kardiyak muayenenin yeterli olacağını, %82.2’si ekokardiyografi yapılması gerektiğini düşünmekteydi. Çocuklarında üfürüm olduğunu öğrenmeleri ile çocuk kardiyolojisi kliniğine başvurma arasında geçen süre sorulduğunda; %59’unun bir gün içinde, %29.6’sının bir hafta içinde başvurduğu görüldü. Çocuklarında üfürüm olduğunu öğrendiklerinde %79.9’u bunun önemli bir kalp hastalığının bulgusu olduğunu düşündüklerini belirtirken, %51.4’ünün araştırma ve bilgi edinme yoluna gittiği, %55.4’ünde bilgi edinme yolunun internet olduğu öğrenildi.Sonuç: Çocuklarda duyulan kardiyak üfürümler sıklıkla masum vasıflı olmasına rağmen ailelerde ciddi düzeyde endişeye neden olmaktadır. Çocuğunun kalbinde üfürüm olduğu söylenen ve bilgilendirilmeyen aileler mutlaka bir üçüncü basamak sağlık kuruluşuna başvurmakta, çoğu kardiyak muayenenin yeterli olmayacağını düşünmekte ve ekokardiyografi ile değerlendirilmeyi talep etmektedir., Objective: To determine the anxiety levels, awareness of the need to go to a pediatric cardiology clinic and the expectations from pediatric cardiologists of parents whose children have a cardiac murmur. Material and Methods: Children with cardiac murmurs diagnosed during general physical examinations and presented at pediatric cardiology clinics for the first time were included in the study. Both pathological and innocent murmurs were included. The questionnaire forms consisting of 7 questions were answered by 354 families presenting at the pediatric cardiology clinic between May and August 2013Results: The anxiety level of the parents when they learned that their children had cardiac murmur was 8 out of 10 points. The parents’ awareness of a department dealing with pediatric heart disease was 60.7%, When their expectations were asked 8.4% of the parents said that a cardiac examination would be enough while 82.2% thought that echocardiography should be performed. It was determined that 59% of the parents presented at a pediatric cardiology clinic within a day and 29.6% within a week. According to 79.9% of the parents, the murmur was a sign of a serious heart disease; 51.4% of them investigated and obtained information about the murmur, and 55.4% of them used the internet for this purpose Conclusion: Cardiac murmurs cause a significant level of anxiety in parents even though they are mostly innocent. The parents of a child with cardiac murmur who are not provided information by a health care provider at the primary or secondary care health centers typically visit a pediatric cardiology clinic and ask for echocardiography examination

BACKGROUND The purpose of this study is to determine early myocardial dysfunction in β-thalassemia major (BTM) patients. Where the myocardial dysfunction cannot be detected by conventional echocardiography, it could be detected by tissue Doppler imaging (TDI) or speckle tracking echocardiography (STE). METHODS In this study, we analyzed 60 individuals, 30 of whom were BTM patients and the other 30 of whom were the control group. T2* magnetic resonance imaging (MRI) was used to measure cardiac iron deposition. The myocardial functions were evaluated by conventional echocardiography, TDI and STE. RESULTS When basal lateral left ventricular and basal septal wall TDI values were compared between the patient group and control group, only isovolumic contraction time values were significantly longer in the patients. The global circumferential strain was significantly lower in the patients. When evaluated as segmental, longitudinal strain values of basal inferoseptum and circumferential strain values of anteroseptum, anterior, and inferolateral segments were significantly lower in the patients. In the patients, global longitudinal and circumferential strains in the group who had pathological T2* values were significantly lower than the group who did not. In addition, circumferential strain values in anteroseptum, anterolateral, inferior, and inferoseptum segments were significantly lower in the patients with T2* values

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

In childhood, pulmonary thromboembolism (PTE) is an uncommonbut potentially life-threatening disease. The greater numbers of venous thromboembolism (VTE) are complications of underlying risk factors such as malignancies, chemotherapy (L-asparaginase), and central venous catheter. We report a patient with acute lymphoblastic leukemia and PTE, who presented with near-syncope, and was successfully treated with low molecular weight heparin and calcium channel blockers.

Objectives: Cardiovascular adverse effects can be seen rarely in patients receiving methylphenidate for attention deficit and hyperactivity disorder (ADHD). In this study, we planned to investigate the effects of methylphenidate on the cardiovascular system in ADHD patients.

Objectives: To review the data of 11 term newborns with persistent pulmonary hypertension of the newborn (PPHN) resistant to conventional therapy and treated with intravenous (IV) iloprost and oral sildenafil as rescue medicine. Materials and Methods: Iloprost was started 2 ng/kg/min intravenously, if oxygenation index (OI) 25 in spite of conventional methods in 24 h. During follow-up, if OI remains 25, iloprost dosage was increased by 1 ng/kg/min by 6-h intervals. If there was improvement in OI in 24 h, treatment continued with IV iloprost and thereafter with inhaled iloprost during weaning. However, if there was no improvement in OI, treatment continued with 0.25 mg/kg/dose oral sildenafil given at 4 doses. If OI remains 25, sildenafil dosage was increased by 0.25 mg/kg at each following dose. Similarly, if there was improvement in OI in 24 h, treatment continued with oral sildenafil and thereafter with inhaled iloprost during weaning. Results: In 5 patients, treatment continued with oral sildenafil because of no response to IV iloprost. The treatment continued median 5 days in subjects given IV iloprost, and median 6 days with oral sildenafil. The extubation time was median 10 days and the follow-up continued median 17 days. The mean systolic pulmonary artery pressure was 63.3 mmHg before treatment, and 38.5 mmHg before discharge (P < 0.01). Ten of 11 subjects (% 91) were discharged and 1 subject died because of noncardiopulmonary reasons. Conclusion: These results showed that, the use of IV iloprost and oral sildenafil as rescue medicine in patients with resistant PPHN could be effective and safe.