Search

Objective : This study aims to evaluate clinic situations, investigate arrhythmias, and assess ventricle function by echocardiography and aortic root dilation of patients with Tetralogy of Fallot who had undergone total correction surgery. Material and Methods: 50 children with Tetralogy of Fallot and who had undergone a total correction operation in I.U. Faculty of Medicine Pediatric Cardiology Department were included to study. Corrected Tetralogy of Fallot cases were divided into two main groups as “aortic root dilated group” and “aortic root not-dilated group”. Groups were evaluated as to their gender, age of correction surgery, presence of aortic failure, tricuspid failure, pulmonary failure, aorta pulmonary shunt treatment and usage of transannular patch. Pulmonary valve failure was compared as those with and without transannular patches applied during the total correction surgery. Echocardiographically measured aortic root and ascending aorta diameters were assessed according to age, gender, and body surface area of the corrected Fallot Tetralogy cases. Results: 50 subjects were involved to study in date between 24th Jan. 2014 and 15th May 2014. Age of the cases in study varied between 2 and 21 years and average age was 9.9 ± 4.5 years. 52% (n=26) of them are male and 48% are female (n=24). Total correction age was varied between 6 months (0.5 years) and 190 months (15.8 years) and average age was 28.3±28.3 months (2.3 ±2.3 years). Their postoperative periods were varied between 1.3 and 17.8 years and average period was 8.0±4.1 years. 28% (n=14) of the cases were received palliative treatment. Aortic root size was varied between 18 and 42.5 and average size was measured as 27.9±5.3 mm. Aortic root dilation ratio of the cases was 88% (n=44) while 12% of cases (n=6) had no aort root dilatation. All patients had right ventricle and ascending aorta dilation. Moderate and severe pulmonary failure was found less in those who underwent transannular patch. Conclusion: Aortic root dilation was detected in 88% (n=44) of the cases. Providing annual aortic root diameter measurements will help to plan a more effective strategy for the treatment of progressive aortic root dilatation in patients with Fallot tetralogy.

Diabetic patients may experience more severe Staphylococcus aureus-related respiratory tract infections such as necrotizing pneumonia (NP). A 13-year-old girl with uncontrolled diabetes mellitus was admitted to pediatric intensive care unit (PICU) with diabetic ketoacidosis and respiratory distress. Her initial evaluation revealed diffuse pneumonic infiltration that progressed to NP within days. She was intubated and placed on mechanical ventilator (MV) support. Positive inotropes and broad-spectrum antibiotics were initiated. Methicillinresistant S. aureus was isolated from blood and pleural specimen cultures. In spite of aggressive medical treatment, infection could not be controlled and lobectomy was performed. However, the patient was fistulized and right total pneumonectomy was performed on the 16th and 29th days of PICU admission. She gradually got better and was weaned from MV. On the 59th day, she was discharged oxygen-free from the hospital. Early surgical intervention should be considered for the treatment of NP resistant to medical therapy.

Objectives Turner Syndrome (TS) is associated with a high risk of cardiac anomalies and cardiovascular disease. We aimed to evaluate patients with TS (n=33) for cardiac and aortic pathology using thorax magnetic resonance angiography (MRA). Subjects and methods Clinical findings, karyotypes, echocardiogram (ECHO) findings and thorax MRA results were evaluated. Aortic dimensions were measured and standard Z scores of aortic diameters along with aortic size index (ASI) were calculated. Results Mean age of the patients was 13.7±3.4 years. MRA revealed cardiovascular pathology in 10 patients (30%). CoA (n=4), aberrant right subclavian artery (n=3), dilatation of the ascending aorta (n=1), tortuosity of the descending aorta (n=1) and fusiform dilatation of the left subclavian artery (n=1) were found. Two of the four patients with CoA found on MRA were detected with ECHO. Mean diameter of the sinotubular junction was found to be elevated [mean±SD: 2.4±1.5]. Z scores for the diameters of the isthmus, ascending aorta and descending aorta were in normal ranges. 45,X patients were found to have significantly higher ASI values than non 45,X patients (p=0.036). Conclusion Our findings indicate that patients with TS should be evaluated with MR imaging studies in addition to ECHO to reveal additional subtle cardiac and vascular anomalies. CoA which is very distally located or which has mild nature may not be seen by ECHO. The increase in ASI observed in 45,X patients may herald the development of life-threatening complications. Therefore, frequent follow-up is warranted in these patients.

Background and Aim: The new coronavirus (SARS-COV-2) pandemic has rapidly spread to many countries and caused morbidity and mortality all over the world Central nervous system (CNS) can be affected in this disease because of the coronavirus capability to invade brainsteam Olfactorial neurons blame to be entry for neuroinvasion and the spread the infection to the brainsteam via transneural route Medulla oblangata contains cardiac, respiratory and vasomotor centers A quarter of admitted COVID-19 complain from anosmia (loss sense of smell) and which may be indicator of CNS involvement Heart rate variability (HRV) has long been associated to be a surrogate markers of cardiac autonomic tone Inflammatory bowel disease (IBD) has effects on neural cardiovascular control mechanisms the effect of IBD disease status on cardiovascular autonomic functions by measuring (HRV) parameters with 24-hour holter electrocardiogram (ECG) recording The aim of this study was to evaluate the heart rate variability (HRV) in a patient with coronavirus disease 2019 (COVID-19) as a marker disease severity and CNS involvement Methods: A prospective analytical case control study was conducted in Health Science University, Tepecik Education and Research Hospital Izmir, Turkey We enrolled 50 patients with COVID-19 and 54 matched control subjects in the study All participants underwent a 24-hour Holter recording to assess HRV parameters Firstly, we design two groups as patient and controle group Than the study population was separated into 3 groups of symptomatic disease, asymptomatic disease and control group to analyse the effect of disease severity on the HRV parameters Results: Age and gender distribution were well matched between groups (38 5±8 46 vs 39 9±15 3;p=0 95) Disease duration was 10 46±4 67 and %26 5 of patient complained from lost sense of smell and taste %50 of the COVID-19 patient had symptomatic disease on admission Control patient have higher BMI (26 2±3 34 vs 28 1±4 0;p=0 02) than COVID patients Plasma CRP (2 88±4 2 vs 0 63±1 12 p=0 003) levels were found significantly high in patient with COVID-19 No difference was determined between the COVID-19 and control groups in respect of any HRV parameters (Table 2) Significant differences were found both between the symptomatic patients and the control group and between symptomatic and asymptomatic patients in terms of the HRV measurements of SDANN5, RMSSD, CCVLF, LF/HF (Table 3) Conclusions: The results of this study demonstrated for the first time that the disease severity and symptom status in COVID-19 patients is associated with cardiac autonomic abnormalities compared to both a control groups and asymptomatic COVID-19 patients Patients who have had symptomatic COVID-19 infection appear to be at risk for arrhythmia, particularly patient with sign of CNS involvement Further research is needed to clarify long term effect of COVID-19 infection on HRV

Factors associated with aortic dilation and dissection in patients with Turner syndrome (TS) remain unclear. We assessed magnetic resonance imaging-based aortic diameters at nine predefined anatomic positions and examined associations of increased aortic diameters with B-type natriuretic peptide (BNP), A-type NP (ANP), growth hormone treatment, insulin-like growth factor 1 (IGF1), and estrogen status. Forty-seven patients with TS aged 7.3–21 years and 34 healthy peers were enrolled in this study. Aortic diameters were higher in patients with TS at three positions than in controls (p

Pallister-Killian sendromu, kraniyofasiyal dismorfizim, gelisme geriligi, zeka geriligi, hipotoni, nobetler, gorme ve isitme bozukluklari, dogumsal malformasyonlar ve dogumsal kalp hastaliklari ile birliktelik gosteren nadir bir genetik hastaliktir. Ventrikuler septal defekt ve atriyal septal defekt en sik gorulen kardiyak malformasyonlardir. Aritmiler, bu hastalarda nadiren bildirilmistir. Bu olgu sunumunda tekrarlayan ventrikuler tasikardi ataklarinin eslik ettigi onbir yasinda Pallister-Killian sendromlu bir hasta sunuldu ve Pallister-Killian sendromlu hastalarin aritmi acisindan da taranmasi gerektigi vurgulanmak istendi.

Influenza virus can cause serious complications, especially in patients with comorbid illnesses. Acute respiratory distress syndrome (ARDS) is among the most common causes of influenza-related morbidity and mortality. Herein we present the case of a boy with a genetic disorder who recovered from influenza-related ARDS. A 3-year-old boy with 3p deletion syndrome was admitted for refractory cough and fever. During his follow-up, sudden respiratory failure concomitant with ARDS occured. He was transferred to the pediatric intensive care unit (PICU) and intubated. Broad-spectrum antibiotherapy and oseltamivir were administered. Nazopharnygeal swab polymerase chain reaction (PCR) analysis revealed H1N1. Venitalition parameters were set according to that recommended for ARDS. On the ninth day of his admission, the patient was discharged from PICU. Appropriate ventilation strategies together with early oseltamivir therapy improves outcomes of influenza-related ARDS.

Aim Healthcare-associated infections cause increased morbidity and mortality in intensive care units. In this study, it was aimed to compare infections with multi-drug resistance and extended drug resistance, while evaluating the characteristics of resistant Gram-negative infections in the pediatric intensive care unit in our university hospital. Material and methods In this study, pediatric patients who were found to have Gram-negative infections during hsopitalization in the pediatric intensive care unit in our faculty between January 2011 and December 2015, were evaluated retrospectively. Results One thousand thirty patients were internalized in our unit in the study period. The incidence for healthcare-associated infection was found as 17.2% and the incidence density was found as 32.7 per 1000 patient days. The incidence for healthcare-related infection per 1000 device days and the rate for device use were calculated as 66.9 and 0.59, respectively. One hundred thirty Gram-negative infection episodes were found in 79 patients whose median age was 22 (1-205) months. The most common infections included ventilator-related pneumonia (n=78, 60%) and bloodstream infections (n=38, 29.2%). The most common causative agents included Pseudomonas aeruginosa (n=50, 38.5%), Kleibsiella pneumonia (n=32, 24.6%) and Acinetobacter baumannii (n=28, 21.5%). Among A. baumannii isolates, the rates for resistance against piperacillin-tazobactam and meropenem were found as 96.4% and 89.3%, respectively. Empirical use of carbapenems, aminoglycosides, and fluoroquinolones, the presence of total parenteral nutrition and history of Gram-negative bacterial infections prior to pediatric intensive care unit admission were significantly more common among extended-drug Gram-negative bacterial infections. The late mortality rate was found to be higher in presence of extended drug resistance. History of Gram-negative infection was found to be an independent risk factor in terms of extended drug resistance. Conclusion Healthcare-associated infections are an important health problem and it is important for infection control committees of hospitals to determine and apply strategies according to hospital colonization in prevention.

To identify prognostic factors and indications in patients receiving continuous renal replacement therapy (CRRT) in the pediatric intensive care unit (PICU), and to demonstrate their effect on mortality. A total of 63 patients admitted between 2011 and 2014 were included in the study. The demographic information, pediatric risk of mortality (PRISM) scores, vasoactive-inotropic score, indication for CRRT, time of starting CRRT, presence of fluid overload, durations of CRRT, and pediatric intensive care unit (PICU) stay were compared between survivors and non-survivors. The overall rate of survival was 69,8%. The most common indication for CRRT was fluid overload (31.7%) followed by acute attacks of metabolic diseases (15.9%), and resistant metabolic acidosis (15.9%). The median duration of CRRT was 58 (IQR 24–96) h. The most common CRRT modality was continuous venovenous hemodiafiltration. The CRRT modality was not different between survivors and nonsurvivors. Sepsis, as the diagnosis for admission to intensive care unit was significantly related to decreased survival when compared to acute kidney injury and acute attacks of metabolic diseases. Patients with fluid overload had significantly increased rate of death, CRRT duration, use of mechanical ventilation, and PICU stay. The CRRT, can be effectively used for removal of fluid overload, treatment of acute attacks of metabolic diseases, and other indications in critically ill pediatric patients. It has a positive effect on mortality in high-risk PICU patients. This treatment modality can be used more frequently in pediatric intensive care unit with improved patient outcomes, and should be focused on starting therapy in early stages of fluid overload.

Key Clinical Message We report the occurrence of supraventricular tachycardia during intravenous immunoglobulin (IVIG) infusion. Supraventricular tachycardia was observed in two newborn patients during IVIG infusion. Both of the babies responded to adenosine treatment. Cardiorespiratory monitoring during IVIG infusion can be recommended because of the possibility of this potentially lifethreatening adverse effect.

BACKGROUND Cirrhotic cardiomyopathy (CCMP) is a functional disorder characterized by electrophysiological disturbances, and diastolic and/or systolic dysfunction in patients with liver disease. This disorder is a well-defined entity in adults, but pediatric data are limited. The aim of the study was to determine the incidence, features, and risk factors of CCMP in children with portal hypertension (PHT). METHODS This study included 50 children with cirrhotic PHT (40/50) and noncirrhotic PHT (10/50). Fifty healthy children were also selected for the control group. Electrocardiography and echocardiography were used to evaluate cardiac functions. Corrected QT (QTc) ≥ 0.45 was accepted as prolonged on electrocardiography. The study group was divided into 3 groups: cirrhotic, noncirrhotic, and control. Then, the CCMP group was created according to the diagnostic criteria. Latent CCMP was diagnosed in the presence of prolonged-QTc along with a minor criterion (tachycardia). Manifest CCMP was diagnosed in the presence of at least 2 major criteria (prolonged-QTc along with abnormal echocardiographic findings). Moreover, in this study, the risk factors for CCMP were investigated. RESULTS The CCMP group included 10 cases (20%). Nine of these cases had latent CCMP (18%), and the remaining one (2%) had manifest CCMP. All of the cases with CCMP had cirrhosis and ascites. None of the patients with CCMP had severe cardiac symptoms, but they were already using some cardioprotective drugs such as propanolol and spironolactone. As risk factors for CCMP, pediatric end-stage liver disease scores, Child-Pugh scores, and ascites grades were found to be significant for the determination of CCMP. The most important risk factor was ascites severity (P = 0.001, odds ratio 9.4). CONCLUSIONS Approximately 20% of children with PHT have CCMP. A detailed cardiac examination should be carried out periodically in children with cirrhotic PHT, especially in the presence of ascites and high Child-Pugh score.

Aim We aimed to study electrocardiographic changes in children with diabetic ketoacidosis and ketosis and to evaluate the relation of the changes with serum electrolyte levels and ketosis. Material and methods This study was performed in Istanbul Medical Faculty, Pediatric Emergency and Intensive Care Department between May 2008 and May 2009. The electrocardiographic parameters and QT length of children with diabetic ketoacidosis and ketosis were evaluated at diagnosis and after the treatment. Results Forty patients were included in the study; 16 (40%) were diagnosed as having diabetic ketosis and 24 (60%) had diabetic ketoacidosis. Twenty-four (60%) patients were male and 16 (40%) were female and the mean age was 9.21±4.71 years (range, 1-16 years). Twelve (30%) cases of diabetic ketoacidosis were mild, three (7.5%) were moderate, and nine (22.5%) were severe. One patient had premature ventricular beats, and four had ST depression. The electrocardiographic parameters were all normal beyond the QTC length prolongation. The mean QTC length was 447±45 ms (380-560 ms) at diagnosis and 418±32 ms (350-500 ms) after treatment. The change in the QTC length was statistically significant. None of the patients had significant electrolyte disturbance and the prolongation of QTc length was not correlated with serum electrolyte levels. The prolongation of QTc length was statistically correlated with anion gap (r=0.33, p=0.03). Conclusions In our study, we showed QTc length prolongation and the importance of performing electrocardiography during the diagnosis of diabetic ketoacidosis and ketosis. We also demonstrated that ketosis was responsible for the prolongation of QTc length.

We report a 14-year-old boy with a previous diagnosis of Blackfan-Diamond anemia (BDA) who presented with the complaint of bradycardia. Electrocardiogram (ECG) showed complete atrioventricular (AV) block with the mean heart rate 51 bpm on admission. Laboratory data revealed endocrine abnormalities and severe hypothyroidism. Soon after chelation and levothyroxine therapy , AV block was disappeared gradually.

OZET Amitriptilin zehirlenmesi cocuk acil servislerinde sik karsilasilan zehirlenmelerden biridir. Amitriptilin zehirlenmesi, cesitli sistemleri etkileyen yan etkileri nedeniyle ciddi sorunlara yol acmakta ve hayati tehdit edebilmektedir. En ciddi etkileri santral sinir sistemi ve kardiyovaskuler sistem uzerine olmaktadir. Siddetli zehirlenmesi olan hastalarda kalp iletim bozukluklari, supraventrikuler tasikardi, ventrikuler disritmiler ve hipotansiyon gelisebilir. Bilinmeyen miktarda amitriptilin tablet aldiktan sonra ciddi kardiyak belirtiler (hipotansiyon, aritmi, ventrikuler fibrilasyon) gelisen onsekiz aylik kiz hasta yogun bakima alindi. Hastaya defibrilasyon, magnezyum sulfat, lidokain tedavileri uygulandi. Bu tedaviler ile tamamen duzelen hasta taburcu edildi (CAYD 2014; 1(1):39-42). Anahtar Kelimeler: Amitriptilin zehirlenmesi, aritmi, defibrilasyon, ventrikuler fibrilasyon

Objectives: Children with Turner syndrome (TS) are at increased risk of cardiovascular disease (CVD), but associations with subclinical CVD are not well-characterized. The purpose of this study was to assess myocardial function using strain imaging (SI) by echocardiography in children with TS and without known CVD. Methods: The study included 48 children with TS aged 4–16 years and 20 healthy control children. Children with TS were excluded if they had a cardiac malformation, a decreased left ventricular (LV) systolic function, or any chronic disease. Each child had an echocardiographic examination with conventional echocardiography and one-dimensional longitudinal strain (1DST) echocardiography. Results: Septal and lateral systolic strain (S) and strain rate (SR) values, which are indicative of longitudinal myocardial function, were significantly decreased in TS patients. However, LV ejection fraction (LVEF) and LV fractional shortening (LVFS) was not significantly different between groups. LV mass index (LVMi), interventricular septum (IVS) thickness, LV posterior wall (LVPW) thickness, and left atrial (LA) diameter index were significantly higher in TS children compared to controls. Peak transmitral flow velocity in late diastole (peak A) was significantly higher, whereas peak transmitral flow velocity in early diastole (peak E), deceleration time (DT), and the ratio of early to late diastolic filling were significantly lower, in TS patients. Conclusion: Reduced LV systolic S and SR in children with TS may indicate early myocardial dysfunction before any detectable change in LVEF. (Echocardiography 2013;00:1–6)

Left ventricular non-compaction is a rare form of cardiomyopathy believed to be the result of intrauterine arrest of compaction of the endomyocardial morphogenesis, leading to persistence of the embryonic myocardium. Clinical manifestations are highly variable, ranging from no symptoms to a progressive deterioration in cardiac function that results in congestive heart failure, systemic thromboemboli, arrhythmias, and sudden cardiac death. Presented here is the case of a 4-year-old child with a history of aborted sudden cardiac death. Following resuscitation, he was admitted to the intensive care unit with neurologic sequelae that regressed later on. Transthoracic echocardiography and magnetic resonance imaging showed numerous prominent trabeculations and deep intertrabecular recesses at the apical and anterolateral region of the left ventricle. Electrophysiologic study showed polymorphic ventricular tachycardia. An implantable cardioverter-defibrillator (ICD) was implanted following clinical recovery. Five months after implantation, appropriate ICD shock due to ventricular fibrillation was documented.

The objective of our study was to analyze the lipid profile and some risk factors of atherosclerosis such as oxidized-low density lipoprotein (ox-LDL), small dense LDL (sd LDL) in the offspring of patients with premature coronary heart disease (CHD).Children whose parents had early onset CHD were matched with age and sex pairs. Study and controls were analyzed for lipid levels, apolipoproteins (Apo- A,B,E), ox-LDL, sd LDL and lipoprotein (a) [Lp(a)]. The data were evaluated with SPSS using "Student t and Mann-Whitney U" tests.The study group children (n: 43) had higher LDL, Lp(a) and ox-LDL levels, ratios of TC/HDL, Apo-B/A, LDL/HDL and ox-LDL/HDL (p0.05) than control group.These findings suggest that dyslipidemia and increased LDL, Lp(a) and ox-LDL levels are common in the offspring of patients with early onset CHD and account largely for their familial predisposition for CHD.Antecedentes/Objetivo. El objetivo de nuestro estudio fue analizar el lipidograma y ciertos factores de riesgo de ateroesclerosis, tales como las lipoproteínas de baja densidad oxidadas (ox-LDL, por su sigla en inglés) y las lipoproteínas de baja densidad pequeñas y densas (sdLDL, por su sigla en inglés) en los hijos de pacientes con cardiopatía coronaria (CC) prematura. Población y métodos. Hijos de padres con CC de inicio temprano emparejados con pares de su misma edad y mismo sexo. Se analizaron las concentraciones de lípidos, apolipoproteínas (ApoA, B, E), ox-LDL, sdLDL y lipoproteína (a) [Lp(a)] en los niños de estudio y de referencia. Los datos se evaluaron con el programa SPSS, junto con la prueba t de Student y la prueba U de Mann-Whitney. Resultados. Los niños del grupo de estudio (n: 43) tenían niveles más elevados de LDL, Lp(a) y ox-LDL y cocientes mayores de CT/HDL, ApoB/ApoA, LDL/HDL y ox-LDL/HDL (p0,05) que los del grupo de referencia. Conclusión. Con base en estos hallazgos, se sugiere que la dislipidemia y las concentraciones elevadas de LDL, Lp(a) y ox-LDL son frecuentes en los hijos de pacientes con CC de inicio temprano y representan gran parte de la predisposición familiar a tener CC

AIMS Williams syndrome (WS) is a microdeletion syndrome affecting cardiovascular and connective tissue as well as the endocrine and central nervous systems in 1 in 10,000 live births. This study aims to identify and evaluate cardiovascular abnormalities (CVAs) in 45 WS patients. PATIENTS AND METHODS We retrospectively reviewed a cohort of WS patients who were followed at our institution from January 1, 1990 through December 31, 2010. WS was clinically diagnosed by an experienced medical geneticist and confimed by fluorescence in situ hybridization. CVAs were assessed using electrocardiography, echocardiography or cardiac catheterization. RESULTS Twenty-seven patients (60%) were male; 18 were female (40%).The mean age at presentation was 4.6 +/- 3.1 years (3 months-13 years); the follow-up period was 6.9 +/- 4.4 years (6 months-18 years). CVAs were found in 86% of patients, the most common one being supravalvar aortic stenosis (SVAS) in 73% (isolated in 48%), peripheral pulmonary artery stenosis (PAS) in 42%, and mitral valve prolapse (MVP) in 22%. Less common were aortic insufficiency (15%), ventricular septal defect (11%), valvular pulmonary stenosis (11%), and aortic arch hypoplasia (8%) and coarctation (2%). Hypertension was present in 22% of patients. Surgical or catheter-based interventions were performed in 22% of cases. Two patients were lost in the postoperative period. CONCLUSION CVAs were found in more than four out of five patients, the most common ones being SVAS and PAS. Although surgery was performed in more patients with SVAS than with PAS, SVAS was minimal or mild in most patients and improved in few cases.

SCN1A mutations are found in up to 80 % of patients with Dravet syndrome (DS), and the sudden unexpected death in epilepsy (SUDEP) rate is higher in DS than in most forms of severe epilepsy. The aim of this study is to examine the autonomic cardiac function and the risk of arrhythmia in DS patients by evaluating QT and P wave dispersion and heart rate variability (HRV) using standard electrocardiography (ECG) and 24-h ECG. The study group consisted of 15 patients (9 boys and 6 girls aged 3.5-17 years) who were genetically diagnosed with DS. The control group comprised 20 healthy subjects, 13 boys and 7 girls aged 4-17 years. P wave dispersion (44.6 +/- A 3.5 ms), QT dispersion (58.8 +/- A 7.5 ms) and QTc dispersion (70.8 +/- A 7.4 ms) were significantly higher in DS patients as compared to the control group (p < 0.001 for all values). However, there was no significant difference in PR, QT or QTc length between the groups. 24-h Holter ECG showed that all HRV parameters were significantly lower in patients with DS. The decreased HRV and increased P wave and QT dispersion seen in DS patients are important signs of autonomic dysfunction with increased adrenergic tone. To determine whether autonomic dysfunction is correlated with SUDEP in DS, long-term electrocardiographic monitoring and wider prospective studies are necessary.

Background: Sudden death risk in Williams syndrome (WS) patients has been shown to be 25–100 times higher than in the general population. This study aims to detect coronary artery anomalies and myocardial perfusion defects in WS patients using noninvasive diagnostic methods. Methods: This study features 38 patients diagnosed with WS. In addition to physical examination, electrocardiography, and echocardiography, computed tomography (CT) angiography and rest/dipyridamole stress technetium-99m sestamibi ( 99 mTc-sestamibi) single photon emission computed tomography (SPECT) myocardial perfusion scintigraphy (MPS) were performed. Results: Twenty-one (55%) patients were male; 17 (45%) were female. The average patient age was 12 ± 5 years (2.5–26 years); the average follow-up period was 7.2 ± 4.2 years (6 months–18 years). Cardiovascular abnormalities were found in 89% of patients, the most common one being supravalvar aortic stenosis (SVAS). CT angiography revealed coronary anomalies in 10 (26%) patients, the most common ones being ectasia of the left main coronary artery and proximal right coronary artery as well as myocardial bridging. SVAS was present in 80% of patients with coronary artery anomalies. 99mTc-sestamibi SPECT MPS revealed findings possibly consistent with myocardial ischemia in 29% of patients, and ischemia in 7 out of 10 patients (70%) with coronary anomalies shown on CT angiography (p = 0.03). Conclusions: Coronary artery abnormalities are relatively common in WS patients and are often accompanied by SVAS. CT angiography and dipyridamole 99 mTc-sestamibi SPECT MPS seem to be less invasive methods of detecting coronary artery anomalies and myocardial perfusion defects in WS patients.

Aim We evaluated ambulatory patients with Duchenne muscular dystrophy from the cardiovascular standpoint and studied the correlation between the results of electrocardiographic (ECG) findings, left ventricular ejection fraction (LVEF), troponin T and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels and patients' North Star Ambulatory Assessment scores. Methods: Fifty patients of ages 6–12 (8.9 ± 2.8) were enrolled in this cross-sectional study. Cardiac evaluation included electrocardiography, echocardiography and cardiac enzyme tests. Results North Star scores ranged from 6/34 to 34/34. Twenty-eight patients (56%) had ECG changes. The most frequently seen ECG abnormalities were short PR interval (14%, n= 7), right ventricular hypertrophy (16%, n= 8), prolonged QTc interval (10%, n= 5), prominent Q wave (10%, n= 5) and T wave inversion (44%, n= 22). In 10 patients (20%), LVEF was below 55%, troponin T and NT-proBNP levels were significantly elevated (P= 0.003 and P < 0.001, respectively). When North Star scores were compared to patients' age, enzyme levels, ECG and echocardiographic results, we discovered negative correlation with age (P < 0.001) and troponin T levels (P= 0.02) and positive correlation with LVEF (P= 0.02). Conclusion Patients with North Star scores of ≤16 are more at risk of developing cardiomyopathies. Troponin T is a cardiac index that can be used for evaluating myopathic patients and it seems to be correlated with the proBNP levels and LVEF values.

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung. We present a 15-month-old boy who was diagnosed with Scimitar syndrome after examinations for recurrent wheezing and respiratory distress. The chest radiograph showed a scimitar sign in the right hemithorax, obscuring the contours of the right atrium. Echocardiography showed dilatation of the right heart cavities and increased flow in the inferior vena cava, without a cardiac abnormality. The patient underwent cardiac catheterization for radiographic and hemodynamic evaluations, during which a scimitar vein was detected, draining the right pulmonary veins to the inferior vena cava. Coil occlusion was performed on the abnormal artery arising from the infradiaphragmatic aorta. The patient was referred to surgery for repair of the anomalous pulmonary venous return and resection of the sequestered pulmonary segment.

Autosomal recessive dystrophic epidermolysis bullosa (DEB) is a chronic skin disorder characterized by widespread bullous formation, erosions, and scar formation. There have been reports of dilated cardiomyopathy and death in patients with DEB. The pathogenesis of cardiomyopathy in DEB remains uncertain, but some drugs, viral infections, iron loading, micronutrient deficiencies such as selenium and carnitine have been implicated. A 16-year-old boy who was followed-up from birth with the diagnosis of DEB presented with respiratory distress and heart failure symptoms of two-week history and early fatigue within the past year. Etiological evaluation showed a low plasma selenium level. Echocardiographic examination yielded the diagnosis of dilated cardiomyopathy. Findings of viral serology tests and metabolic screening were normal. Selenium replacement and anticongestive treatment were initiated, which led to partial improvement in cardiac functions. The authors draw attention to the possible role of micronutrient deficiency in the development of cardiomyopathy in patients with DEB.

Background: The aim of this study was to comprehensively evaluate electrocardiographic (ECG) findings of isolated left ventricular noncompaction (IVNC) patients at initial diagnosis and to explore the correlation between them and the clinical, echocardiographic, and magnetic resonance imaging (MRI) findings. Methods: Twenty-three patients diagnosed with IVNC by echocardiography and cardiac MRI between January 2006 and June 2010 were enrolled in this study. The patients were examined with standard ECG and 24-hour Holter ECG. For comparison purpose, ECGs of 50 healthy children of similar ages and demographic characteristics were taken. Results: In 87% of patients, ECG abnormalities were found. The most frequently seen ECG findings were left ventricular hypertrophy, ST-segment depression, and negative T wave related to abnormal repolarization particularly in DII, DIII, and V4–6 leads, as well as prolonged PR and QTc intervals. No ECG features or patterns were found that were specific to the disease. In contrast to adult patients, while no intraventricular conduction defects (particularly in the left bundle brach) were found in any of our patients, 13% had considerable bradycardia and one required a pacemaker. The Holter ECG recordings showed supraventricular tachycardia attacks in two patients and a short ventricular tachycardia attack in one. Patients whose echocardiograms and MRI showed left ventricular systolic dysfunction and left ventricular dilatation had signs of left ventricular hypertrophy and repolarization abnormality on their ECGs, but there was no significant difference in PR, QRS, and QTc intervals. Conclusion: Regardless of how frequently left ventricular hypertrophy and repolarization abnormalities are found on IVNC patients’ initial ECGs, we think that they are not unique to the disease but are related to the severity of the cardiomyopathy. Ann Noninvasive Electrocardiol 2011;16(2):184–191

Objective The purpose of this study is to describe morphologic features and delayed contrast-enhancement pattern of the noncompaction of the left ventricle in cardiac magnetic resonance (MR) imaging. Methods We retrospectively reviewed morphological cardiac MR imaging findings of ventricular noncompaction in 15 patients (eight men, seven women, and ages 6 months to 73 years old, mean 22 year). In 10 patients delayed contrast enhanced images were obtained after the morphological examination. Results In all patients, noncompaction was seen in the apical and midventricular-lateral segment. Basal-septal segment involvement was not determined in any patients. Noncompacted/compacted ratio was 2–4.5 (mean 3). In nine patients, right ventricular involvement was observed in addition to left ventricular noncompaction. Delayed contrast-enhancement was seen in eight out of 10 patients not only involved segments but also normal segments of the heart. Conclusion Cardiac MR imaging is a valuable imaging method in patients with suspected ventricular noncompaction by showing increased trabeculations, deep intertrabecular recesses and fibrosis. Fibrosis is a common finding in ventricular noncompaction.

Down's syndrome (DS) is the most common chromosomal abnormality due to a trisomy of chromosome 21 commonly associated with congenital heart defects (CHDs). This study aimed to evaluate the frequency and types of CHD patterns in Turkish children with DS.The data relate to paediatric patients with DS who underwent cardiologic screening between 1994 and 2007 and were reviewed in our Paediatric Cardiology unit.Four hundred and twenty-one out of the 1042 paediatric patients with DS studied over a 13-year period had associated CHD. Of these, 320 (77.6%) had a single cardiac lesion, while the remaining 92 patients (22.4%) had multiple defects. The most common single defect was an atrioventricular septal defect (AVSD) found in 141 patients (34.2%), followed by 69 patients (16.7%) showing secundum type atrial septal defect, and ventricular septal defect in 68 patients (16.5%). AVSDs were the leading type, isolated or combined with other cardiac anomalies with an overall occurrence of 19.8% of paediatric patients with DS, and 49.2% of paediatric patients with both DS and CHD.This is the first study concerning the frequency and type of CHD observed in Turkish children with DS. The high frequency of AVSD in Turkish children with DS implied that early screening for CHDs by echocardiography is crucial. The correction of AVSDs in paediatric patients with DS should be performed in the first 6 months of life to avoid irreversible haemodynamic consequences of the defect.

WOS: 000258652900006 PubMed: 18670742 Introduction: Atrial septal defect (ASD) is one of the most common congenital heart diseases in children. P-wave dispersion has been reported to be associated with non-homogeneous propagation of sinus impulses. The heterogeneity of atrial conduction time may predispose the atria to arrhythmias. The aim of this study was to determine the impact of surgical repair on P-wave indices in children with isolated secundum ASD. Methods: Children with isolated secundum ASD undergoing surgical repair (n=50; mean age, 7.0 +/- 3.0 years) and healthy controls (n=51; mean age, 7.6 +/- 2.7 years) were compared. Maximum P-wave duration (Pmax), shortest duration (Pmin) and P-wave dispersion (Pd) were measured using 12-lead surface electrocardiography. Results: Mean Pmax was found to be significantly higher in children with ASD compared with controls (95.2 +/- 10.8 vs 84.1 +/- 9.2 msec; P < 0.001), and Pd before surgery was significantly higher compared with controls (47.4 +/- 12.0 vs 38.8 +/- 9.7 msec; P < 0.001). Both P-wave indices were significantly decreased within the first year after surgical closure - the values decreased to those comparable to healthy controls (Pmax, 86.2 +/- 9.7 msec; Pd, 39.8 +/- 10.7 msec; P > 0.05). Conclusion: Surgical closure of ASD in children decreases Pmax and P-wave conduction time. We speculate that earlier closure of the defect may play an important role in avoiding permanent changes in the atrial myocardium and atrial fibrillation in adulthood.

OBJECTIVE. The purpose of this article is to review the CT and MRI findings of cardiac hydatid disease.CONCLUSIONS. CT and MRI are helpful for localizing and defining the morphologic features of hydatid cysts. Specific signs include calcification of the cyst wall, presence of daughter cysts, and membrane detachment. CT best shows wall calcification, whereas MRI depicts the exact anatomic location and nature of the internal and external structures.

Balloon valvuloplasty is an effective therapy for severe congenital aortic valve stenosis, with mild aortic insufficiency and minimal intermediate-term restenosis. No consensus currently exists regarding optimal vascular approach for balloon dilatation in newborns with critical or severe aortic valve stenosis. Critical aortic valve stenosis in newborns must be treated promptly and effectively. Transcatheter therapy may offer marked advantages, as surgical therapy has been associated with significant rates of morbidity and mortality. Percutaneous balloon dilatation is usually performed as emergent therapy of valve stenosis, with various options for vascular approach. While umbilical artery and vein access is rarely used in the treatment of critical aortic valve stenosis and aortic coarctation, this approach is a safe, simple, and effective choice for balloon dilatation in newborns, even in those weighing under 2.5 kg.

Fish oil has a cardioprotective effect in adults with ischemic heart disease. The authors examined the effects of fish oil in children with idiopathic dilated cardiomyopathy (DCM). Eighteen DCM patients (group I) and 12 healthy children (group III) were given fish oil (10 mL/d). Their cardiac findings were compared with those of 11 patients with DCM who did not receive fish oil (group II). After 6.62+/-1.70 months, left ventricular ejection fraction had increased by 8.44%+/-3.80% (P.05), in group I; 2.48%+/-3.85% (not statistically significant) in group II; and 0.84%+/-2.34% (not statistically significant) in group III. Left ventricular internal diastolic diameter (mm) was reduced by 4.36+/-4.86 (P=.001) in group I and 1.92+/-5.37 (P=.263) in group II, but increased by 0.22+/-2.54 (not statistically significant) in group III. The results suggest that fish oil leads to accelerated improvement of left ventricular function. The authors believe that if these results are confirmed in larger studies, fish oil should be added to the standard anticongestive therapy of children with DCM.

Strokes, cardiac or noncardiac, generally affect the elderly and only occasionally occur in children. A tendency to stroke occurs in cyanotic congenital heart disease; however, this report describes two female patients, aged 6 years and 4 years, who had no cyanotic disease. The first patient was admitted to the hospital after the first stroke but suffered a second one after admission. Her initial cardiac examination was normal. The other patient was admitted after a second right-sided hemiparesis. A mild pansystolic ejection murmur was observed at the lower left sternal border on auscultation; slight late diastolic murmurs with opening snap were found in the mitral valve area, and the second heart sound was increased. Transthoracic echocardiography revealed a mass in the anterior mitral valve in both patients. These masses were completely removed surgically and pathology revealed myxomas. Hemiparesis completely resolved after surgical and medical treatment. The clinical manifestations of cardiac tumors primarily depend on their number, size, location, and histology. Strokes and anterior mitral valve masses are extremely rare in children. In the two cases reported here, anterior mitral valve myxomas caused systemic embolization as a result of their location. Echocardiography should be performed early in patients presenting with recurrent strokes to exclude such pathology.

Amac: Bu arastirmanin amaci tip fakultesi dekanlarinin Turk Tabipleri Birligi Mezuniyet Oncesi Tip Egitimi Arastirmasi Anket Formu rsquo;na verdikleri yanitlarla Turkiye 39;deki mezuniyet oncesi tip egitimi ile ilgili bir durum saptamasi yapmaktir Gerec ve Yontem: Bu arastirma tanimlayici tipte bir calismadir Veri toplama formu ogrenci aldigi belirlenen 65 tip fakultesine gonderilmistir Bulgular: Turkiye rsquo;de yeni acilacak dokuz tip fakultesi ile birlikte tip fakultesi sayisi 2010 yilinda 74 rsquo;e yukselmektedir Tip fakultelerinin 58 rsquo;i devlet 16 rsquo;si vakif universitesi tip fakultesidir Turkiye 39;de 2009 2010 ders yilinda tip fakultelerinde toplam ogrenci sayisi 38 536 rsquo;dir Temel bilimler ogretim uyeleri icin bir ogretim uyesine ortalama 22 6 ogrenci klinik bilimler ogretim uyeleri icin ortalama 4 5 ogrenci toplamda ise bir ogretim uyesine 3 5 ogrenci dustugu gorulmektedir Fakultelerde profesorlerin ve bes yilik docentlerin tam zamanli calisma orani 2010 yili icin 78 3 rsquo;dur Elli alti tip fakultesinden 34 rsquo;unde 60 7 karma 18 rsquo;inde 32 1 egitici merkezli ve dordunde 7 1 ogrenci merkezli egitim modeli kullanilmaktadir Mufredat incelendiginde 47 rsquo;sinde 83 9 sistem temelli entegre besinde 8 9 disiplin temelli klasik ve ucunde 5 3 probleme dayali mufredat birinde 1 7 ise entegre ve klasik mufredat uygulandigi bildirilmektedir Cikarimlar: Son iki yilda tip fakultesi sayisindaki artis carpicidir Turk Ped Arfl 2011; 46: 331 6