Prolonged P-Wave and QT Dispersion in Children with Inflammatory Bowel Disease in Remission

Objectives. Ulcerative colitis (UC) and Crohn’s disease (CD) are chronic inflammatory bowel diseases (IBD) with unclear underlying aetiologies. Severe cardiac arrhythmias have been emphasised in a few studies on adult IBD patients. This study aimed to investigate the alteration of the P-wave and QT interval dispersion parameters to assess the risk of atrial conduction and ventricular repolarisation abnormalities in pediatric IBD patients. Patients and Methods. Thirty-six IBD patients in remission (UC: 20, CD: 16) aged 3–18 years and 36 age- and sex-matched control patients were enrolled in the study. Twelve-lead electrocardiograms were used to determine durations of P-wave, QT, and corrected QT (QTc) interval dispersion. Transthoracic echocardiograms and 24-hour rhythm Holter recordings were obtained for both groups. Results. The P-wave dispersion, QT dispersion, and QTc interval dispersion (Pdisp, QTdisp, and QTcdisp) were significantly longer in the patient group. The mean values of Pminimum, Pmaximum, and QTcminimum were significantly different between the two groups. The echocardiography and Holter monitoring results were not significantly different between the groups. Furthermore, no differences in these parameters were detected between the CD and UC groups. Conclusion. Results suggest that paediatric IBD patients may carry potential risks for serious atrial and ventricular arrhythmias over time even during remission

Evaluation of Electrocardiographic Markers for the Risk of Cardiac Arrhythmia in Children with Obesity

Aim:This study was conducted to examine the electrocardiographic markers used in the risk assessment of cardiac arrhythmia in children with obesity.Materials and Methods:In this prospective study, 60 children aged 3-17 years with exogenous obesity and 60 age and sex-matched healthy controls were included. Demographic data, assessment of atrial and ventricular arrhythmia risk markers in electrocardiography, and standard echocardiography measurements were performed. Values of p<0.05 were considered significant.Results:The mean ages of the study and control groups were 11.51±3.48 years and 10.74±3.72 years, respectively. Both groups had 30 males and 30 females. The study group had significantly higher average mean body mass index (BMI) compared to the control group. In electrocardiographic examinations, P-wave dispersion, QT dispersion (QTd), corrected QTd (QTcd), Tpeak-Tend (Tp-e), Tp-e/QT, and Tp-e/QTc values were significantly higher in the obese group compared to the control group. In echocardiographic examinations, the dimensions of the heart chambers and vascular structure and wall thicknesses were found to be significantly higher in those children with obesity.Conclusion:The electrocardiographic risk markers used to predict cardiac arrhythmias were found to be increased in those children with obesity. This may suggest that increased body weight and adiposity may have unfavorable effects on the cardiac conduction system

Evaluation of changes in cardiac conduction system in children with familial mediterranean fever

Ailevi Akdeniz Ateşi (FMF), ateş ve steril poliserozit atakları ile karakterize, genetik geçişli, tekrarlayan otoinflamatuvar bir hastalıktır. Ailevi Akdeniz Ateşi tanılı çocuk hastaların elektrokardiyografik ölçümlerinde kardiyak ileti sistemindeki değişikliklerinin saptanması amacıyla bu çalışma yapılmıştır. Yöntem: Çalışmaya Aralık 2019- Nisan 2020 tarihleri arasında FMF tanısıyla takip edilen 87 çocuk hasta ve aynı yaş grubunda sağlıklı 87 olgu dahil edildi. Hasta grubun laboratuvar parametreleri ve genetik analizleri ile birlikte tüm olguların demografik özellikleri değerlendirildi. Her iki grupta elektrokardiyografide atriyal ve ventriküler aritmi riski belirteçleri ve standart ekokardiyografi ölçümleri yapıldı. İstatistiksel analizler için SPSS 25 paket programı kullanıldı. p<0,05 olması anlamlı kabul edildi Bulgular: Hasta grubun yaş ortalaması 10,30±4,02 yıl ve kontrol grubun ise 10,03±3,93 yıl idi. Hasta grubunun; %49,04'ü kız (n=43) %50,6'sı erkek (n=44), kontrol grubunun; %55,2'si kız (n=48) ve %44,8'i (n=39) erkek idi. Hasta ve kontrol grupları arasında yaş, cinsiyet, boy, kilo, vücut kitle indeksi ve tansiyon değerleri bakımından istatistiksel olarak anlamlı fark yoktu. Ailevi Akdeniz Ateşi tanısıyla takipli hastaların genetik incelemelerinde %18,4 homozigot mutasyon, %17,2 heterozigot mutasyon ve %25,3 compound heterozigot mutasyon olduğu görüldü. Hastaların %11,5'inde mutasyona rastlanmazken, %27,6 hastanın genetik sonuçlarına ulaşılamadı. Hasta ve kontrol grubunun standart ekokardiyografik incelemesinde kalp yapı ve fonksiyonlarının iki grupta birbirine benzer olduğu görüldü. Elektrokardiyografi incelemelerinde hasta grubunda P dispersiyonu (Pd), QT dispersiyonu (QTd), düzeltilmiş QTd (QTcd), Tp-e, Tp-e /QT ve Tp-e/QTc değerleri kontrol grubuna göre istatistiksel olarak yüksek bulunmuştur (p<0,001). Ayrıca hasta grubunda ventriküler repolarizasyon parametrelerinin önemli bir kısmının da birbiriyle anlamlı derecede korele olduğu görülmüştür (p<0,05). Sonuç: Çalışmamızda; FMF tanılı çocuk hastalarda kontrol grubuna göre atriyal depolarizasyon (Pd) ve ventriküler repolarizayon (QTd, QTcd, Tp-e, Tp-e/QT, TP-e/QTc) belirteçleri yüksek saptandı. Bu durum hastalığın kardiyak aritmiler için risk oluşturabileceğini düşündürmektedir. Bununla birlikte altta yatan mekanizma ve aritmojenik potansiyeli aydınlatmak için uzun süreli kapsamlı çalışmalara ihtiyaç vardır.Familial Mediterranean Fever (FMF) is a genetic, recurrent autoinflammatory disease characterized by episodes of fever and sterile polyserositis. This study was conducted in order to determine changes in cardiac conduction system via electrocardiographic measurements of pediatric patients diagnosed with Familial Mediterranean Fever. Method: Eighty-seven pediatric patients who were being followed-up with the diagnosis of FMF between December 2019 and April 2020, and 87 age-matched healthy cases were included in the study. Demographic characteristics of all cases, together with laboratory parameters and genetic analyses of the patient group were evaluated. For both groups, atrial and ventricular arrhythmia risk markers in electrocardiography and standard echocardiographic measurements were obtained. For statistical analyses, SPSS 25 package program was used. A p <0.05 was considered significant. Results: Mean age was 10.30±4.02 years in the patient group and 10.03±3.93 years in the control group. Of the patient group; 49.04% (n=43) were female and 50.6% (n=44) male, whereas of the control group; 55.2% (n=48) were female and 44.8% (n=39) male. There were no statistical differences between the patient and control groups in regard to age, gender, height, weight, body mass index and blood pressure values. In genetic examinations of patients diagnosed with Familial Mediterranean Fever; 18.4% were determined to have homozygous mutation, 17.2% heterozygous mutation and 25.3% compound heterozygous mutation. Of patients, 11.5% had no mutation and genetic results of 27.6% could not be reached. Cardiac structure and functions of the patient and control groups with standard echocardiographic examination were found to be similar. In electrocardiographic examination, P dispersion (Pd), QT dispersion (QTd), corrected QTd (QTcd), Tp-e, Tp-e /QT and Tp-e/QTc values were determined to be statistically higher in the patient group compared to the control group (p<0.001). Furthermore, in the patient group, significant correlations were observed between the majority of ventricular repolarization parameters (p<0.05). Conclusion: In our study, atrial depolarization (Pd) and ventricular repolarization (QTd, QTcd, Tp-e, Tp-e/QT, TP-e/QTc) markers were determined to be higher in pediatric patients with FMF compared to control group. This suggests that the disease may pose a risk for cardiac arrhythmias. However, extensive studies with longer durations are needed in order to clarify the underlying mechanism and arrhythmogenic potential

Demographic, clinical and echocardiographic characteristics of children with chest wall deformities

Giriş ve Amaç: Göğüs duvarı deformitesi olan çocukların demografik, klinik ve ekokardiyografik özelliklerinin değerlendirilmesi amaçlandı. Yöntem ve Gereçler: Çocuk kardiyoloji ünitesinde üç yıl süreyle göğüs duvarı deformitesi tanısı alan hastalar retrospektif olarak değerlendirildi. Bulgular: Ortanca yaşı 8 olan hastaların ortalama yaşı 7.6 ± 4.5 idi. Göğüs duvarı deformitesi olan yüz altmış dört (% 80) hastanın ekokardiyografik değerlendirmesi normaldi. Ekokardiyografik tanıları 10 (% 4.87) atriyal septal defekt, 8 (% 3.90) mitral kapak prolapsusu, 7 (% 3.41) hafif mitral yetersizliği, 5 (% 2.44) darlık olmayan biküspit aort kapağı, 4 (% 1.95) ventriküler septal defekt, 2 (% 0.98) hafif aort kapak yetersizliği, 2 (% 0.98) dekstrokardi, 2 (% 0.98) aort koarktasyonu ve 1 (% 0.49) kompleks kalp defekti (pulmoner atrezi ve ventriküler septal defekt) idi. Ekokardiyografi ile değerlendirilen sağ kalbe bası bulgusu 15 (% 7.3) idi. Tartişma ve Sonuç: Göğüs duvarı deformitelerinin doğuştan kalp hastalıkları ile ilişkili olması ve kalbe bası bulgusu oluşturabilmeleri sebebiyle ekokardiyografi ile değerlendirilmesi defektlerin en uygun şekilde yönetilmesini sağlayacaktır.Introduction: To determine the demographic, clinical and echocardiographic characteristics of children with chest wall deformities. Methods: The patients diagnosed with chest wall deformities were investigated retrospectively in the pediatric cardiology unit over a period of three years. The study enrolled 205 children under the age of 18 years diagnosed with chest wall deformities. Results: The mean age of the patients was 7.6 ± 4.5 years with a median of 8 years. One hundred and sixty four (80 %) patients with chest wall deformities were found to have normal echocardiography results. Incidental echocardiographic diagnoses included 10 (4.87%) atrial septal defects, 8 (3.90%) mitral valve prolapses, 7 (3.41%) mild mitral regurgitations, 5 (2.44%) bicuspid aortic valve without aortic valve stenoses, 4 (1.95%) ventricular septal defects, 2 (0.98%) mild aortic regurgitations, 2 (0.98%) dextrocardia, 2 (0.98%) coarctations of the aorta and 1 (0.49%) complex cardiac defect (pulmonary atresia and VSD). Compression in the right heart was evaluated by echocardiography 15 (7.3%). Discussion and Conclusion: Because chest wall deformities are associated with congenital heart defects in children and may cause compression in the right heart, evaluation by echocardiography can ensure an optimal management of the defects

Ellis-van creveld syndrome (chondroectodermal dysplasia) a case report: Association of common atrium and persistent left superior vena cava

Ellis-van Creveld (EvC) sendromu, otosomal resesif olarak kalıtılan nadir bir kondral ve ektodermal hastalıktır. Kondro ve ektodermal displazi, polidaktili ve konjenital kalp defektleri bu sendromun karakteristik bulgularıdır. Tek atrium ve endokardiyal yastık defektlerinin bu sendromda en sık rastlanan konjenital kalp defektleri olduğu bildirilmektedir. EvC sendromunun nadir görülmesi nedeni ile ekokardiyografik ve anjiyokardiyografik incelemede, tek atrium ve persistan sol süperior vena kava gibi doğumsal kalp anomalilerinin yanı sıra EvC sendromunun diğer klasik bulguları saptanan dört yaşındaki kız hasta sunulmuştur.The Ellis-van Creveld (EvC) syndrome (EvC) is a rare chondral and ectodermal dysplasia inherited autosomal recessively. The EvC syndrome is characterized by chondrodysplasia and ectodermal dysplasia, polydactyly, and congenital cardiac defects. It is reported that common atrium and endocardial cushion defects are the most common congenital cardiac defects in patients with the EvC syndrome. Here, a four-year-old girl with the diagnostic features of EvC syndrome and a common atrium with persistent left superior vena cava demonstrated by echocardiographic and angiocardiographic investigations is described because of the rarity of this syndrome

Timomanın eşlik ettiği Loeys-Dietz sendromu tip 1

Loeys-Dietz sendromu kardiovasküler, kraniofasiyal ve iskelet sisteminin tutulduğu nadir bir otozomal dominant hastalıktır. Esas etyoloji, transforming growth faktör beta-reseptör tip 1 ve 2 genlerindeki mutasyonlardır. Günümüzde, Loeys-Dietz sendromu kraniofasiyal bulguların varlığına göre iki alt gruba ayrılmıştır. Aort genişlemesi olan hastalar diseksiyon ve rüptür açısından risk taşırlar. Sonuçta, tanıdaki gecikme kötü prognoz ile sonuçlanabilir. Timomanın eşlik ettiği ve Loeys- Dietz tip 1 sendromu tanısı konulan yeni bir vakayı sunduk. Ayrıca, vaka sunumu literatürde bir tümörün eşlik ettiği ilk Loeys-Dietz sendromudur.Loeys-Dietz syndrome is a rare autosomal dominant disorder characterized by the involvement of cardiovascular, craniofacial and skeletal systems. The main etiology of the disease is the mutation in the transforming growth factor betareceptor type 1 and 2 genes. Today, Loeys-Dietz syndrome has been classified into two subtypes due to the presence of craniofacial involvement. These patients have progressive aortic enlargement which increases the risk of dissection and rupture. So, delay in the diagnosis may be associated with poor prognosis. We present a new case with thymoma diagnosed as Loeys-Dietz syndrome type 1. Also, this is the first report of a tumor in Loeys-Dietz syndrome according to the current literature review