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Background Ventricular repolarization (VR) increases the risk of sudden cardiac death due to ventricular arrhythmia. We aimed to evaluate the blood pressure (BP) parameters affecting VR in obese children.Methods Obese (BMI = 95p) and healthy children = 120 cm between January 2017 and June 2019 were included. Demographic and laboratory data, peripheral and central BPs evaluated by a device capable of ambulatory blood pressure monitoring (ABPM), and pulse wave analysis were assessed. Electrocardiographic ventricular repolarization indices, left ventricular mass index (LVMI), and relative wall thickness (RWT) were calculated.Results A total of 52 obese and 41 control patients were included. Uric acid, triglyceride, total cholesterol, LDL, and ALT values, systolic and diastolic office BPs, 24-h, daytime and nighttime systolic and mean arterial BPs, daytime diastolic BP SDS levels, daytime and nighttime systolic loads, daytime diastolic load, 24-h, daytime and nighttime central systolic and diastolic BPs, and pulse wave velocity values were significantly higher, whereas 24-h, daytime and nighttime AIx@75 were similar between the groups. fT4 levels of obese cases were significantly lower. QTcd and Tp-ed were higher in obese patients. Although RWT was higher in obese cases, LVMI values and cardiac geometry classifications were similar. The independent factors affecting VR in obese cases were younger age and higher diastolic load at night (B = - 2.83, p = 0.010; B = 0.257, p = 0.007, respectively).Conclusion Obese patients have higher peripheral and central BP, arterial stiffness, and higher VR indices that develop before an increase in LVMI. It would be useful to prevent obesity from an early age and follow up nighttime diastolic load to control VR associated sudden cardiac death in obese children.

Aim: Cardiac abnormalities associated with celiac disease (CD) have been reported. However, changes in electrocardiographic (ECG) parameters in children with CD have not been previously evaluated in the literature. In this study, we aimed to evaluate these parameters which may be determinative in the development of atrial and ventricular arrhythmia of children with CD. Materials and Methods: Patients diagnosed with CD in the gastroenterology clinic were included in the study. ECG measurements of QT, T peak to end (Tp-e) and P intervals were performed. The Tp-e/QT ratio was calculated from these measurements. Echocardiographic and ECG parameters of the patients and controls were compared. Those patients with elevated anti-tissue transglutaminase immunoglobulin A (IgA-tTG) levels were considered positive serology. The patients were divided in three groups and compared with each other according to ECG parameters; namely newly diagnosed patients, patients with seropositive and patients with seronegative who had been on a gluten free diet for at least 6 months. Results: Fifty-two patients with CD and 59 healthy volunteers were included. P-wave dispersion, QTd, corrected QT dispersion (QTcd), Tp-e dispersion and TP-e/QT ratio were higher in those children with CD compared to the controls. We did not find any correlation between IgA-tTG levels and P-wave, QT and Tp-e dispersions. There was no difference between the three groups of CD patients for ECG and echocardiographic parameters. Conclusion: CD is associated with changes in some ECG parameters, which are considered as predictors of atrial and ventricular arrhythmias in special populations. For this reason, children with CD should be evaluated in terms of these parameters, and more electrophysiological studies are necessary to reveal the clinical and prognostic effects of these parameters for CD.

Patients with solitary kidneys (SKs) are at risk of hypertension (HT) and associated end-organ damage. The authors aimed to evaluate whether children with congenital SKs (CSKs) have higher office, ambulatory, or central blood pressure (BP), increased arterial stiffness or left ventricular mass index, or any risk for arrhythmia. With this purpose, patients with CSK and healthy controls being followed up between January 2018 and June 2019 were enrolled in the study. Demographic, biochemical, and office blood pressure (BP) data were recorded. Then, ambulatory blood pressure monitoring (ABPM) and measurements of central BP (cBP), pulse wave velocity (PWV), and augmentation index (AIx@75) were obtained. Ventricular repolarization parameters were acquired by 12-lead electrocardiography. Left ventricular mass index (LVMI) and abdominal aortic stiffness parameters including strain, pressure strain elastic modulus (Ep), and normalized Ep (Ep*) were calculated with echocardiographic measurements. Finally, 36 children with CSK and 36 healthy controls were included. Serum creatinine, uric acid, total cholesterol levels, ABPM parameters, cBP levels, and PWV values were significantly higher, and eGFR levels were significantly lower in the CSK group. VR parameters, abdominal aortic stiffness indices, and LVMI were similar between the groups. CSK increased the risk of HT in ABPM (HTABPM ) by 6 times. PWV was significantly correlated with Ep and Ep* in cases with CSK. Determination of cBP and PWV along with 24-hour ABPM would be a useful tool in children with CSK.

Iron overload is associated with an increased risk of atrial and ventricular arrhythmias. Data regarding the relationship between electrocardiographic parameters of atrial depolarisation and ventricular repolarisation with cardiac T2* MRI are scarce. Therefore, we aimed to investigate these electrocardiographic parameters and their relationship with cardiac T2* value in patients with β-thalassemia major. In this prospective study, 52 patients with β-thalassemia major and 52 age- and gender-matched healthy patients were included. Electrocardiographic measurements of QT, T peak to end interval, and P wave intervals were performed by one cardiologist who was blind to patients’ data. All patients underwent MRI for cardiac T2* evaluation. Cardiac T2* scores less than 20 ms were considered as iron overload. P wave dispersion, QTc interval, and the dispersions of QT and QTc were significantly prolonged in β-thalassemia major patients compared to controls. Interestingly, we found prolonged P waves, QT and T peak to end dispersions, T peak to end intervals, and increased T peak to end/QT ratios in patients with T2* greater than 20 ms. No significant correlation was observed between electrocardiographic parameters and cardiac T2* values and plasma ferritin levels. In conclusion, our study demonstrated that atrial depolarisation and ventricular repolarisation parameters are affected in β-thalassemia major patients and that these parameters are not correlated with cardiac iron load.

Objective: Arrhythmias due to congenital heartdiseases are found to be the second most common cause of mortality afterischemic heart diseases in several studies. Coarctationof the aorta,hypertrophic cardiomyopathy and aortstenosis increase afterload of the left ventricleand as a result of this, they cause an increase in wall stress in the early andhypertophy of the wall in the late phase. This condition leads a corruptionin ventricular andatrial conduction and predisposesto arrhythmias. In this study, weintended to examine the risks with indicators like P dis, QT dis, Tp-e for arrhythmias in diseases with leftventricular outflow tract stenosis in children. Method: 101 patients with with left ventricular outflow tract stenosis (coarctation of the aorta, idiopathichypertrophic subaortic stenosis, hypertrophic cardiomyopathy and aortic stenosis) in pediatric age group whowere diagnosed between 2008-2014 in Dr. Behçet Uz Children’s Training and Research Hospital were included inthis study. 110 healthy children were included in thisstudy as a control group. Pdis, corrected QT, QT dis ve Tp-e and Tp-e /QT indicesin both groups were were calculated and compared using standard 12-leadECG. Results: P max, corrected QT, Pdis, QTdis, Tp-e andTp-e/QT in patient group were found to be significantly higher than the control group. Therewas no difference between sub-groups of patient group according to Pmax, corrected QT, P dis, QT dis, Tp-e and Tp-e/QT. Therewas also no difference between beta blocker users and non-usersaccording to P max,corrected QT, P dis, QT dis, Tp-e and Tp-e/QT.Conclusions: High P max, corrected QT, P dis, QT dis, Tp-eand Tp-e/QT in the patient group showed the risk for ventricular arrhythmias and atrial fibrillation in patients with left ventricular outflow tractstenosis., Amaç: Konjenital kalp hastalıklarınaait aritmilerin ani kardiyak ölüm riski açısından iskemik hastalıklara bağlıaritmilerden sonra en sık mortalite nedeni olduğu gösterilmiştir. Aortkoarktasyonu, hipertrofik kardiyomyopati ve aort stenozu gibi hastalıklarınvarlığında sol ventikül ard yükü belirgin artmakta bunun sonucu olarak da erkendönemde artmış duvar stresi ve geç kompansatuvar ventrikül hipertrofisi ortayaçıkmaktadır. Tüm bunlar da ventrikül ve atriyal iletimi bozarak aritmilereyatkınlık oluşturabilmektedir. Bu çalışmada, çocukluk çağında sol ventrikülçıkış yolunda darlığa yol açan kardiyak patolojilerde aritmilere yatkınlığınortaya konması amaçlanmıştır. Yöntem: 2008-2014 yılları arasında tanısı konanve hastanemizde izlenen sol ventrikül çıkış yolunda darlığa neden olacakkardiyak patolojisi olan 101 hasta çalışmaya dahil edildi. Kontrol grubu olarak110 sağlıklı çocuk alındı.Standart 12 derivasyonlu EKG’de hastaların P dispersiyonu(dis),QTd(değişkenlik), QT intervali, QTdis ve Tp-e ve Tp-e /QT indeks değerlerihesaplandı.Bulgular: Hasta grubunda Pmaks, QTd,Pdis, QTdis, Tp-e ve Tp-e/QT indeksleri kontrol grubuna göre belirgin yükseksaptandı. Hasta grubunu oluşturan alt gruplarda Pmaks, QTd, Pdis, QTdis, Tp-eve Tp-e/QT indeksleri açısından gruplar arası anlamlı istatistiksel farksaptanmadı. Beta bloker kullanan ve kullanmayan hasta gruplarında da buparametreler açısından anlamlı fark saptanmadı.Sonuç: Çalışmamızdaaritmi belirteçleri olarak değerlendirdiğimiz Pmaks, Pdis, QTd,QTdis, Tp-e, Tp-e/QT hasta grubunda kontrol grubuna göre belirgin artmışizlenmiş ve bu da bu hastaların hematriyal fibrilasyon hem de ventriküler aritmiler açısından risk altındaolduğunu göstermiştir.

Kawasaki disease (KD) is a systemic febrile condition leading to the inflammation of vessels including coronary arteries (1). Coronary artery aneurysms, occuring in approximately 15–25% of the patients, are the most important and life-threatening complication of KD (2). KD mainly affects children younger than five years old. Although it has been known for years, the etiology of the disease remains largely unexplained (3,4). Due to the lack of specific tools, the diagnosis of KD is still based on clinical features. At least four of the following five clinical criteria together with fever lasting equal to or longer than five days should be present for the diagnosis: bilateral non-purulent conjunctivitis; extremity findings including erythema and/or indurative edema on the hands and feet, and membranous desquamation starting in the periungual regions; changes in the lips and oral cavity; polymorphous exanthema; and cervical lymphadenopathy (usually unilateral with a diameter of 1.5 cm or more). However, those patients with prolonged fever (≥5 days) together with two or three clinical criteria may be diagnosed as incomplete KD in the presence of some supplemental laboratory findings (5). It has been reported increasingly in recent years, especially among young infants and children. Because incomplete KD also results in coronary artery lesions (CALs), it should be diagnosed promptly (5,6). However, the diagnosis is still difficult and further laboratory studies are needed. Neutrophil to lymphocyte ratio (NLR) is a novel parameter, studied in the conditions with inflammatory pathogenesis including ischemic heart disease, stroke, and infections (7–9). It was reported to be a strong predictor of adverse cardiovascular outcome and severity of coronary artery ectasia in adult population (10–12). In this study, the usefulness of NLR in the diagnosis of KD or incomplete KD and the correlation between NLR and the presence of coronary artery lesions were evaluated. The usefulness of NLR has not been studied in patients with KD so far.

OBJECTIVE To assess the feasibility of 12-lead electrocardiographic (ECG) measures such as P wave dispersion (PWd), QT interval, QT dispersion (QTd), Tp-e interval, Tp-e/QT and Tp-e/QTc ratio in predicting poor outcome in patients diagnosed with sepsis in pediatric intensive care unit (PICU). METHODS Ninety-three patients diagnosed with sepsis, severe sepsis or septic shock and 103 age- and sex-matched healthy children were enrolled into the study. PWd, QT interval, QTd, Tp-e interval and Tp-e/QT, Tp-e/QTc ratios were obtained from a 12-lead electrocardiogram. RESULTS PWd, QTd, Tp-e interval and Tp-e/QT, Tp-e/QTc ratios were significantly higher in septic patients compared with the controls. During the study period, 41 patients had died. In multivariate logistic regression analyses, only Tp-e/QT ratio was found to be an independent predictor of mortality. CONCLUSION The ECG measurements can predict the poor outcome in patients with sepsis. The Tp-e/QT ratio may be a valuable tool in predicting mortality for patients with sepsis in the PICU.

Congenital generalized lipodystrophy type 4 is an extremely rare autosomal recessive disorder. We report our clinical experience on two unrelated Turkish patients with congenital generalized lipodystrophy type 4. A 13-year-old girl (patient-1) presented with generalized lipodystrophy and myopathy. Further tests revealed ventricular and supraventricular arrhythmias, gastrointestinal dysmotility, atlantoaxial instability, lumbosacral scoliosis, and metabolic abnormalities associated with insulin resistance. A 16-year-old girl (patient-2) with congenital generalized lipodystrophy type 4 was previously reported. Here, we report on her long term clinical follow-up. She received several course of anti-arrhythmic treatments for catecholaminergic polymorphic ventricular tachycardia and rapid atrial fibrillation. An implantable cardioverter defibrillator was also placed. A homozygous PTRF mutation, c.259C > T (p.Gln87*), was identified in patient-1. Congenital generalized lipodystrophy type 4 was caused by homozygous PTRF c.481-482insGTGA (p.Lys161Serfs*41) mutation in patient-2. Our data indicate that patients with congenital generalized lipodystrophy type 4 should be meticulously evaluated for cardiac, neuromuscular, gastrointestinal and skeletal diseases, as well as metabolic abnormalities associated with insulin resistance. (C) 2016 Elsevier Masson SAS. All rights reserved.

Reports state that Down syndrome (DS) patients with congenitally normal hearts might experience the development of cardiac abnormalities such as cardiac autonomic dysfunction, valvular lesions, bradycardia, and atrioventricular block. However, the presence of any difference in terms of P-wave dispersion (PWd) and QT dispersion (QTd) was not evaluated previously. This study prospectively investigated 100 DS patients with structurally normal hearts and 100 age- and sex-matched healthy control subjects. Standard 12-lead electrocardiograms were used to assess and compare P-wave and QT durations together with PWd and QTd. The median age of the DS patients and control subjects was 48 months. Heart rates and P-wave and QT dispersions were significantly greater in the DS group than in the control group (113 +/- A 22.9 vs 98.8 +/- A 16.6 bpm, p < 0.001; 31.3 +/- A 9.5 vs 24 +/- A 8.6 ms, p < 0.001; and 46.6 +/- A 15.9 vs 26 +/- A 9.1 ms, p < 0.001, respectively). A positive correlation was found between PWd and age in the DS patients (p < 0.05; r = 0.2). All children with DS should be followed up carefully with electrocardiography in terms of increased P-wave and QT dispersions even in the absence of concomitant congenital heart disease for management of susceptibility to arryhthmias.

WOS: 000331063500007 PubMed ID: 24087940 Intravenous (IV) ferric iron (Fe)-carbohydrate complexes are used for treating Fe deficiency in children with iron-refractory iron-deficiency anemia (IRIDA). An optimal treatment has yet to be determined. There are relatively little publications on the responsiveness to IV iron therapy in children with IRIDA. Patients and Method: This study analyzed responses to IV iron sucrose therapy given to 11 children, ranging in age from 2 to 13 years (mean 4.8 years), with iron-deficiency anemia who were unresponsive to oral iron therapy. Results: The hemoglobin and ferritin values (mean) of the 11 children with IRIDA were 7.7 g/dL and 4.8 ng/mL at diagnosis. Both hemoglobin and ferritin levels increased to 9.5 g/dL, and 24 ng/mL, respectively, at 6 weeks after the first therapy. Although the level of hemoglobin was steady at 6 months after the first, and 6 weeks after the second therapy, the ferritin levels continued to increase up to 30 ng/mL and 47 ng/mL at 6 months after the first and 6 weeks after the second therapy, respectively. Conclusion: We recommend that IRIDA should be considered in patients presenting with iron-deficiency anemia of unknown cause that is unresponsive to oral iron therapy. Our results suggest that IV iron therapy should be administered only once in cases of IRIDA. Continued administration of IV iron would be of no benefit to increase hemoglobin levels. On the contrary, ferritin levels may continue to increase resulting in untoward effects of hyperferritinemia.

Objective: The aim of this study is to define the predictors of chronic carditis in patients with acute rheumatic carditis (ARC). Methods: Patients diagnosed with ARC between May 2010 and May 2011 were included in the study. Echocardiography, electrocardiography, lymphocyte subset analysis, acute phase reactants, plasma albumin levels, and antistreptolysin-O (ASO) tests were performed at initial presentation. The echocardiographic assessments were repeated at the sixth month of follow-up. The patients were divided into two groups according to persistence of valvular pathology at 6th month as Group 1 and Group 2, and all clinical and laboratory parameters at admission were compared between two groups of valvular involvement. Results: During the one-year study period, 22 patients had valvular disease. Seventeen (77.2%) patients showed regression in valvular pathology. An initial mild regurgitation disappeared in eight patients (36.3%). Among seven (31.8%) patients with moderate regurgitation initially, the regurgitation disappeared in three, and four patients improved to mild regurgitation. Two patients with a severe regurgitation initially improved to moderate regurgitation (9.1%). In five (22.8%) patients, the grade of regurgitation [moderate regurgitation in one (4.6%), and severe regurgitation in 4 (18.2%)] remained unchanged. The albumin level was significantly lower at diagnosis in Group 2 (2.6 ± 0.48 g/dL). Lymphocyte subset analysis showed a significant decrease in the CD8 percentage and a significant increase in CD19 percentage at diagnosis in Group 2 compared to Group 1. Conclusion: The blood albumin level and the percentage of CD8 and CD19 (+) lymphocytes at diagnosis may help to predict chronic valvular disease risk in patients with acute rheumatic carditis. Resumo: Objetivo: O objetivo deste estudo é definir os preditores da cardite crônica em pacientes com cardite reumática aguda (CRA). Métodos: Os pacientes diagnosticados com CRA entre maio de 2010 e maio de 2011 foram incluídos no estudo. Foram realizados os testes de ecocardiografia, eletrocardiograma, uma análise do subgrupo de linfócitos, provas de fase aguda, níveis de albumina plasmática, antiestreptolisina-O (ASO) na manifestação inicial. As avaliações ecocardiográficas foram repetidas no 6° mês de acompanhamento. Os pacientes foram divididos em dois grupos de acordo com a persistência da patologia valvular no 6° mês como Grupo 1 e Grupo 2 e todos os parâmetros clínicos e laboratoriais na internação foram comparados entre dois grupos de comprometimento valvular. Resultados: Durante o período do estudo de um ano, 22 pacientes apresentaram doença valvular. 17 (77,2%) pacientes apresentaram regressão da patologia valvular. Houve desaparecimento de regurgitação moderada inicial em 8 pacientes (36,3%). Entre 7 (31,8%) pacientes com regurgitação moderada inicialmente, a regurgitação desapareceu em 3 e 4 pacientes apresentaram melhora para regurgitação leve. 2 pacientes com regurgitação grave inicialmente apresentaram melhora para regurgitação moderada (9,1%). Em 5 (22,8%) pacientes, o grau de regurgitação [regurgitação moderada em 1 (4,6%) e regurgitação grave em 4 (18,2)] continuou inalterado. O nível de albumina foi significativamente menor no diagnóstico no Grupo 2 (2,6 ± 0,48 gr/dL). A análise do subgrupo de linfócitos mostrou uma redução significativa no percentual de CD8 e um aumento significativo no percentual de CD19 no Grupo 2 em comparação ao Grupo 1. Conclusão: O nível de albumina no sangue e o percentual de linfócitos CD8 e CD19 (+) no diagnóstico podem ajudar a prever risco de doença valvular crônica em pacientes com cardite reumática aguda. Keywords: Acute rheumatic carditis, Albumin, CD8, CD19, Lymphocyte subtypes, Palavras-chave: Cardite reumática aguda, Albumina, CD8, CD19, Subtipos de linfócitos