Fetal bradyarrhythmias: classification, monitoring and outcomes of 40 cases at a single center.

OBJECTIVES: To assess congenital fetal bradyarrhythmias with regard to etiological causes, features, risk factors, and prognosis. METHODS: This retrospective study involved fetuses with fetal bradyarrhythmias. All fetuses were evaluated by ultrasonography. Parental ECGs and family histories were obtained, and maternal autoantibodies were measured. Gestational age at diagnosis, fetal atrial and ventricular rates at presentation, type of bradyarrhythmias, the presence or absence of a congenital heart defect (CHD), fetal hydrops, fetal myocardial dysfunction, extra-cardiac abnormalities, maternal autoimmune diseases, maternal autoantibodies as well as prenatal treatment, and neonatal outcome were collected. RESULTS: Of the 40 fetuses included in the study, 11 had maternal rheumatologic disease, 16 had complex cardiac anomalies such as left and right isomerism. Fetuses with CHD significantly differed from those without CHD with increased rates of extra-cardiac anomalies, hydrops, fetal deaths and shorter survival after 28 days (p<0.05). Survival was significantly better in fetuses with maternal rheumatic disease as compared with those with no maternal rheumatic disease (p<0.05). Maternal anti-arrhythmic therapy was administered in 11 fetuses. In utero maternal treatment resulted in no significant difference in the course of arrhythmia or hydrops in fetuses with or without maternal rheumatic disease (p<0.05). In regression analysis, the absence of fetal hydrops was the only independent factor associated with survival (p=0.04). CONCLUSIONS: The course of bradyarrhythmias, along with survival, seems to be more favorable in fetuses with maternal rheumatic disease than in those with CHD, especially left and right isomerism. Hydrops was the sole independent factor associated with poor survival.

Assessment of Subclinical Atherosclerosis in Children with Atopic Dermatitis.

INTRODUCTION: Data from studies conducted to date have evaluated clinical atherosclerotic conditions in adult patients with atopic dermatitis (AD). Subclinical atherosclerotic changes that are a precursor of atherosclerotic conditions may begin in childhood. The aim of this study was to investigate the presence of subclinical atherosclerosis in pediatric patients with AD and to determine the associated risk factors. METHODS: A total of 59 patients who were referred to our department over a 6-month period and diagnosed with AD, and 53 healthy controls with a similar age and gender were included in the study. Subclinical atherosclerosis markers (carotid intima media thickness [CIMT], distensibility, stiffness, and strain) were measured using conventional echocardiography. The patients' age, SCORAD index, and duration of symptoms were recorded. Serum total immunoglobulin E, C-reactive protein (CRP), blood lipid profile, and complete blood count markers were measured. RESULTS: The median age of the patients was 61 (10-103) months, and 59.3% of them were male. The patients with AD had a higher CIMT (1.60 ± 0.35 vs. 1.30 ± 0.50 mm) and a lower distensibility (0.006 ± 0.009 vs. 0.01 ± 0.008) and strain (0.10 ± 0.14 vs. 0.19 ± 0.14) than the healthy controls (p < 0.01 for all), but there was no significant difference with regard to stiffness (10.16 ± 21.75 vs. 8.99 ± 12.66). Significant correlations between CIMT and disease duration, age, and the SCORAD index were found (p < 0.01, p < 0.01, and p < 0.05, respectively). No correlation between the subclinical atherosclerosis markers and the other laboratory results was found (p > 0.05 for all). CONCLUSION: This study demonstrates that pediatric patients with AD may express subclinical atherosclerosis markers. The evaluation of subclinical atherosclerosis in these patients revealed that CIMT may be the most important marker, as it displayed positive correlations with symptom duration, age, and disease severity.

Evaluation of subclinical atherosclerosis and cardiac functions in children of mothers with gestational diabetes and maternal obesity.

BACKGROUND AND AIMS: We aimed to evaluate the effects of maternal obesity or gestational diabetes on body composition, lipid, and glucose metabolism, arterial morphology, and functions in children, and to investigate these effects in terms of cardiometabolic diseases. METHODS: The study group was composed of 48 children who had a history of gestational diabetes or maternal obesity, and the control group was composed of 33 children. Echocardiographic assessments were performed. Socio-economic status and education level of mothers were obtained. RESULTS: In the study group, carotid intima-media thickness, epicardial adipose tissue thickness, and arterial stiffness values were found to be significantly higher compared to the control group (p < 0.001, p < 0.001, p = 0.003, respectively), while arterial distensibility and arterial strain values were found to be significantly lower (p = 0.003, p = 0.008, respectively). Among the children who had similar body mass index in both groups, children in the study group had higher carotid intima-media thickness and epicardial adipose tissue thickness values. Arterial stiffness values were significantly reduced (p = 0.028) and arterial distensibility and strain values were significantly increased (p = 0.039, p = 0.033, respectively) in the children whose mothers had gestational diabetes and high socio-economic status. Left ventricular mass and left ventricular end-diastolic internal thickness were found to be significantly increased in the children who had obese and unemployed mothers (p = 0.04, p = 0.03, respectively). CONCLUSION: Low socio-economic status was found to be associated with increased maternal obesity and gestational diabetes. Poor socio-economic status, poor glycaemic control and being overweight during pregnancy indicate negative cardiometabolic outcomes for children in the long term.

Subclinical cardiovascular dysfunction in children and adolescents with asthma.

BACKGROUND: There is close association between asthma and cardiovascular functions as both diseases share common inflammatory pathways. The current study was aimed at investigating the risk factors, associated with endothelial and cardiac functions in children with newly-diagnosed mild-persistent asthma. METHODS: A total of 33 steroid-naive asthmatic children [median(interquartile-range); 9.1 years(7.8-13.5)] and 16 healthy controls [11.5 years(9.9-13.6)] were included. Their demographic, clinical and laboratory findings were recorded. Carotid Artery intima-media thickness (CIMT), stiffness, distensibility and strain were measured as atheroclerosis markers. Conventional and tissue Doppler imaging was performed to evaluate ventricular function. RESULTS: The patients with asthma had higher CIMT and stiffness and lower strain and distensibility compared to controls (p < 0.001 for all). There was a significant correlation between the duration of asthmatic symptoms and subclinical-atherosclerosis as well as peripheral eosinophil count (p < 0.001, p < 0.05). The patients had lower tricuspid-annular-plane-systolic-excursion (TAPSE), ejection time, and higher isovolumetric relaxation time (IRT), isovolumetric contraction time (ICT), and left ventricle myocardial performance index (LVMPI) than the control subjects (p < 0.001 for all). A positive correlation was also observed between the duration of asthmatic-symptoms and cardiac-function parameters. CONCLUSION: Children with mild persistent asthma had subclinical atherosclerosis and ventricular dysfunction even in the early stage of disease. Symptom duration was closely associated with both subclinical atherosclerosis and ventricular dysfunction. Myocardial performance index was abnormal in the asthmatic children when assessed by tissue Doppler Imaging even though they had normal ejection fraction in conventional echocardiography. Future prospective studies with larger sample sizes are needed to confirm these findings and to assess the possible protective effect of ICSs in the prevention of subclinical atherosclerosis.

Analysis of tissue Doppler parameters and 24-hour heart rate variations in children with newly diagnosed untreated idiopathic epilepsy in interictal period.

INTRODUCTION: Cardiac mortality has increased in patients with epilepsy. Although majority of cardiac autonomic and ventricular function abnormalities were detected in ictal and postictal period, interictal epileptogenic activity may induce the autonomic imbalance as well. In our study, we aimed to investigate the interictal, subclinical cardiac changes in terms of cardiac autonomic balance via 24-hour Holter electrocardiography (ECG) and ventricular functions by tissue Doppler echocardiography (TDI) in children with newly diagnosed untreated idiopathic epilepsy. MATERIAL AND METHODS: Thirty children with newly diagnosed untreated idiopathic epilepsy (12 males, 18 females; mean age: 125.13 ±â€¯35.2 months) (patient group) and 40 healthy, age and body mass index (BMI)-matched children (18 males, 22 females; mean age: 129.43 ±â€¯38.5 months) (control group) were enrolled. Included patients underwent 24-hour Holter electrocardiographic and tissue Doppler echocardiographic study. RESULTS: Time domain measures were found significantly lower in the patient group. Mean high frequency (HF) values were significantly lower, and mean low frequency (LF) and mean LF/HF parameters were significantly higher in the patient group. Mean isovolumetric contraction time (ICT), isovolumetric relaxation time (IRT), and myocardial performance index (MPI) values were significantly higher, and mean ejection time (ET) values were significantly lower among the patients with untreated idiopathic epilepsy. CONCLUSION: We found that patients with untreated newly diagnosed epilepsy have a significant subclinical deterioration of left ventricular functions, and they also showed changes in heart rate variability (HRV) regarding the sympathovagal imbalance in interictal period. These findings can be related with increased cardiac mortality.

Increased QT and P-wave dispersion during attack-free period in pediatric patients with migraine attacks.

INTRODUCTION: Migraine is a common neurovascular disease characterised with recurrent attacks by pain-free periods. It has been suggested that both sympathetic and parasympathetic dysfunctions play a role in its pathophysiology. AIM: The aim of our study was to investigate the ECG changes during attack-free period in children with migraine, in terms of QTc interval, QTc, and P-wave dispersion to evaluate the autonomic nervous system disturbance. METHODS: Sixty children who were diagnosed with migraine were included as patient group and 50 healthy, age- and body mass index-matched children who were examined for innocent murmur were included as control group. The patients' routine ECG records were screened from the outpatient clinic files. The durations of P-wave, QT, and QTc intervals and dispersion values and heart rates (beats/minute) were compared between the patient and control groups. RESULTS: P maximum and P dispersion were significantly higher, and P minimum was significantly lower in the migraine group compared with the control group. QT-QTc maximum and QT-QTc dispersion were significantly higher and QT-QTc minimum was significantly lower in the migraine group compared with the control group. CONCLUSION: According to our findings, although migraine patients were asymptomatic and no arrhythmia was detected in the surface ECG, sympathovagal balance in the sympathetic system, which may be disrupted in favour of the sympathetic system, should continue even in the attack-free period, and we should be careful in terms of serious arrhythmias that may develop in these patients.

Atypical presentation of an infant with idiopathic pulmonary arterial hypertension: pericardial tamponade.

In this report, we present a five-month-old female patient with a diagnosis of idiopathic pulmonary arterial hypertension (IPAH). This patient was accepted by our clinic after a sudden deterioration in her clinical condition. We performed echocardiography and observed a 17 mm pericardial fluid near the left ventricle posterior wall, which was seen to compress the right atrium and right ventricle outflow tract. Cardiac contraction was impaired, the interventricular septum was hypertrophic and the right ventricle systolic pressure measured by tricuspid regurgitation was at systemic level. Pericardiocentesis was performed for pericardial tamponade, and a drainage tube was placed in the pericardium. Cardiac catheterization was performed before and after nitric oxide inhalation, and there were no changes in the parameters of pulmonary vascular resistance, pulmonary vascular resistance/systemic vascular resistance ratio and mean pulmonary arterial pressure. No cardiac lesion that could cause PAH was detected during catheterization. Under mechanical ventilatory support and intensive medical therapy, the patient died on her 16th day of hospitalization. The importance of our case is that it is the first infantile case presenting with pericardial tamponade associated with IPAH.

Anatomically corrected malposition of the great arteries: two case reports.

Anatomically corrected malposition of the great arteries (ACMGA) is defined as parallel arising of aorta and main pulmonary artery (PA) roots although ventriculoarterial connection is normal. Abnormally related aorta arises from the left ventricle, while abnormally related PA arises from the right ventricle. It can be diagnosed with via echocardiography. In some cases, additional imaging modalities such as computerized tomographic angiography and magnetic resonance are required. In this article, we presented two cases of ACMGA, 5-month-old boy and 1-month-old girl. We wanted to point out the importance of differential diagnosis of other great artery anomalies from this rare pathology.

Prenatal diagnosis and management of fetal supraventricular tachyarrhythmia and postnatal outcomes.

OBJECTIVE: We aimed to describe a single institutional experience managing tachyarrhythmic fetuses, to investigate the underlying pathological findings and to evaluate the postnatal follow-up results. METHOD: This retrospective study included 24 fetuses, treated and followed up with the diagnosis of supraventricular tachyarrhythmia between January 2014 and July 2020. Fetal tachyarrhythmia was evaluated by M mode and Doppler ultrasound. Patients were divided into two categories, fetuses with supraventricular tachycardia (SVT) and those with atrial flutter. Also, patients with SVT were subgrouped as short ventriculo-atrial (VA) SVT and long VA SVT. The presence of hydrops was recorded. Prenatal and postnatal data were all collected from hospital records. RESULTS: SVT and atrial flutter were diagnosed in 23 fetuses and in one fetus, respectively. Of the 23 fetuses with supraventricular tachycardia, 12 cases had short VA time interval and 11 had long VA time interval. Antiarrhythmic therapy was applied to 19 cases, but was not initiated in five cases. Thirteen cases responded to single antiarrhythmic drug. Seven (88%) out of 8 cases with short VA SVT without any sign of hydrops responded to digoxin as a single therapy. Six (67%) out of 9 cases with long VA SVT responded to single therapy. In 6 cases (including atrial flutter), combined antiarrhythmic drug was required. In only one fetus, SVT (long-VA) did not convert to sinus rhythm despite high dose combined antiarrhythmic therapy. Six fetuses (25%) had signs of hydrops. In hydrops cases, overall fetal and neonatal mortality rate was 33%. In the absence of hydrops the mortality rate was zero. Second or third line antiarrhythmic treatment options were required in 83% (5 cases) of hydropic fetuses, whereas in only 8% (one case) of non-hydropic cases. Only one fetus recurred. Maternal complications due to antiarrhythmic therapy developed in four cases. Postnatal antiarrhythmic therapy was required in seven cases. CONCLUSION: We found that in the absence of hydrops, all tachycardiac fetuses responded to treatment regardless of the treatment option. In cases with hydrops, mortality was not observed when anti-arrhythmic treatment was started before significant cardiac dysfunction and heart failure. The need for postnatal treatment was common among those with recurrent tachyarrhythmia under treatment, recurrent arrhythmia after discontinuation of fetal therapy, arrhythmia followed up without treatment in the late gestational period and arrhythmias such as Junctional ectopic tachycardia (JET) and Wolff-Parkinson-White syndrome (WPW) syndrome.

Cardiac MRI in surgically repaired tetralogy of Fallot: Our initial experience.

OBJECTIVE: Pulmonary regurgitation (PR) required pulmonary valve replacement (PVR) is usually seen after surgically repaired tetralogy of Fallot (TOF). Assessment by cardiac magnetic resonance imaging (CMR) plays a crucial role in the decision of PVR. Herein, we presented our 3-year interdisciplinary CMR experience in the assessment of repaired TOF. METHODS: CMR examinations of 196 patients with repaired TOF performed between 2016 and 2018 were enrolled in this retrospective study. Only 165 were included in the study. CMR findings were assessed according to the American College of Cardiology/American Heart Association guideline and recommendations of Geva. RESULTS: Among those 165 patients (median age 14 years [mean age 15.62±7.42 years], M/F=114/61; 1.86/1), 73 patients were found eligible for PVR (59 patients for transcatheter while 14 patients for surgical). The mean QRS duration was 170.2±16.89 ms. On CMR assessment, mean indexed right ventricular end-diastolic volume, end-systolic volume, right, and left ventricular ejection fraction were 187.64±45.07 ml/m2, 39.90±6.60%, and 47.83±6.12%, respectively. The PR fraction was as 50.10±2.54% and 2.25±1.92. Balloon dilatation and/or stenting of branch pulmonary arteries in 12 patients and ventricular septal defect closure in four patients were performed at the same session of percutaneous PVR. At the time of the surgical PVR, repair of partial anomalous pulmonary venous return in one patient, ventricular septal defect in two patients, and subaortic membrane in one patient were performed. An implantable cardioverter-defibrillator was also performed in one patient. CONCLUSION: Our CMR experience has the largest patient population in our country and may contribute to the national data pool. We believe that our collaborative experience between radiologists, cardiologists, and cardiovascular surgeons may also enhance the use of CMR in determining the appropriate technique or timing for PVR.

5'isomiR-183-5p|+2 elicits tumor suppressor activity in a negative feedback loop with E2F1.

BACKGROUND: MicroRNAs (miRNAs) and isomiRs play important roles in tumorigenesis as essential regulators of gene expression. 5'isomiRs exhibit a shifted seed sequence compared to the canonical miRNA, resulting in different target spectra and thereby extending the phenotypic impact of the respective common pre-miRNA. However, for most miRNAs, expression and function of 5'isomiRs have not been studied in detail yet. Therefore, this study aims to investigate the functions of miRNAs and their 5'isomiRs. METHODS: The expression of 5'isomiRs was assessed in The Cancer Genome Atlas (TCGA) breast cancer patient dataset. Phenotypic effects of miR-183 overexpression in triple-negative breast cancer (TNBC) cell lines were investigated in vitro and in vivo by quantifying migration, proliferation, tumor growth and metastasis. Direct targeting of E2F1 by miR-183-5p|+2 was validated with a 3'UTR luciferase assay and linked to the phenotypes of isomiR overexpression. RESULTS: TCGA breast cancer patient data indicated that three variants of miR-183-5p are highly expressed and upregulated, namely miR-183-5p|0, miR-183-5p|+1 and miR-183-5p|+2. However, TNBC cell lines displayed reduced proliferation and invasion upon overexpression of pre-miR-183. While invasion was reduced individually by all three isomiRs, proliferation and cell cycle progression were specifically inhibited by overexpression of miR-183-5p|+2. Proteomic analysis revealed reduced expression of E2F target genes upon overexpression of this isomiR, which could be attributed to direct targeting of E2F1, specifically by miR-183-5p|+2. Knockdown of E2F1 partially phenocopied the effect of miR-183-5p|+2 overexpression on cell proliferation and cell cycle. Gene set enrichment analysis of TCGA and METABRIC patient data indicated that the activity of E2F strongly correlated with the expression of miR-183-5p, suggesting transcriptional regulation of the miRNA by a factor of the E2F family. Indeed, in vitro, expression of miR-183-5p was regulated by E2F1. Hence, miR-183-5p|+2 directly targeting E2F1 appears to be part of a negative feedback loop potentially fine-tuning its activity. CONCLUSIONS: This study demonstrates that 5'isomiRs originating from the same arm of the same pre-miRNA (i.e. pre-miR-183-5p) may exhibit different functions and thereby collectively contribute to the same phenotype. Here, one of three isomiRs was shown to counteract expression of the pre-miRNA by negatively regulating a transcriptional activator (i.e. E2F1). We speculate that this might be part of a regulatory mechanism to prevent uncontrolled cell proliferation, which is disabled during cancer progression.

Prenatal diagnosis and postnatal course in four fetuses with very rare pulmonary artery anomalies.

Pulmonary artery (PA) anomalies are very rare congenital cardiac malformations, a significant number of which remain unrecognized or misdiagnosed during the prenatal period. We report the prenatal diagnosis and outcome of pregnancy with fetal PA anomalies and discuss the related management issues. We identified four cases of prenatally diagnosed rare PA anomalies that were seen and confirmed in the newborn period by echocardiography and computed tomographic angiography at our center from 2018 to 2020. The course of the pregnancy, perinatal outcome, and the postnatal course in each case were analyzed. Three fetuses were born by repeat cesarean section approximately at 39 weeks of gestation and the other woman delivered vaginally. Of the abnormal origin of the left PA (LPA) in two patients, the first had right PA abnormalities derivating from the ascending aorta, and in the second, the LPA originated from the right PA. Two patients had agenesis of ductus arteriosus (DA), the first was accompanied with tetralogy of Fallot (TOF) and right aortic arch with a normal pulmonary valve, the second patient presented with an Absent Pulmonary Valve syndrome with TOF. Prenatal ultrasonography can be used to correctly diagnose the abnormal origin of the PA branches. Branching of the PA, presence of DA, location of the aortic, and ductal arch by the trachea should be routinely screened in the prenatal anatomic examination and the three-vessel and trachea view can determine the primary clues of PA malformations.

SARS-CoV-2 associated multisystem inflammatory syndrome in children (MIS-C). A single center's experience.

BACKGROUND: SARS-CoV-2 related multisystem inflammatory syndrome in children (MIS-C) is a newly defined clinical entity in pediatric ages resembles Kawasaki Disease or toxic shock syndrome. Here we aimed to raise awareness about this SARS-CoV-2 related syndrome. METHODS: Children diagnosed with MIS-C and followed in Pediatric Clinic between November 2020 and January 2021, were included in study. Data about patients' demographic characteristics, clinical and laboratory findings, treatment and outcomes were collected from medical records. RESULTS: The median age of 20 children with MIS-C was 80.5 months, 11 of them were male. The most common symptoms at admission were fever (100%), abdominal pain (70%), myalgia (50%), and rash (50%). Lymphopenia, elevated inflammatory markers and cardiac enzymes were their main laboratory findings. Cardiac involvement (90%) consisted of myopericarditis, valvulitis, left ventricular dysfunction, and coronary arteritis. Symptoms mimicking acute appendicitis and ileus were due to gastrointestinal involvement (50%). Macular rash on the trunk, erythema on upper eyelids were striking. Empiric antibiotics and intravenous immunoglobulin were used in all patients, glucocorticoids (90%), anti-thrombotic (65%) and vasoactive (45%) agents were used according to severity of disease. Response to IVIG treatment was poor, whereas glucocorticoids have dramatic affect. Seven patients (35%) were monitored in intensive care unit, none of them required intubation, mechanic ventilation or ECMO. The median recovery time, that is, the period when fever subside and inflammatory markers returned to normal was 9.5 days. CONCLUSIONS: Glucocorticoids has critical role in treatment of MIS-C, early recognition and treatment may decrease need for intensive care by providing rapid recovery.

Effects of valproic acid and levetiracetam monotherapy on carotid intima-media and epicardial adipose tissue thickness in non-obese children with epilepsy.

OBJECTIVE: The aim of the study was to investigate the risk of subclinical atherosclerosis independent from obesity and high blood lipid levels in pediatric patients with idiopathic epilepsy receiving valproic acid or levetiracetam monotherapy by evaluating carotid intima-media thickness (CIMT) and Epicardial adipose tissue thickness (EATT). METHODS: A total of 75 patients (38 males, 37 females; mean age 127.2 ±â€¯37.9 months) with epilepsy receiving either valproic acid or levetiracetam monotherapy for more than 12 months (Epilepsy Group) and 75 sex, age, body mass index (BMI) matched healthy children (40 males, 35 females; mean age 133.8 ±â€¯38.7 months) (Control Group) were included in the study. The mean duration of therapy was 27.6 ±â€¯10.5 months. Serum lipid levels (total cholesterol, triglycerides, low density lipoprotein, high density lipoprotein) and CIMT-EATT of the patients and controls were assessed. Also, epilepsy group were divided according to antiepileptic drugs (valproic acid group and levetiracetam group). RESULTS: The CIMT was determined as 0.6 ±â€¯0.08 mm in epilepsy group and 0.49 ±â€¯0.15 mm in control group (p < 0.001). The EATT was measured as 5.96 ±â€¯0.8 mm in epilepsy group and 3.7 ±â€¯0.5 mm in control group (p < 0.001). Of epileptic patients, 45 were using valproic acid monotherapy and 30 were on levetiracetam monotherapy. There was no significant difference in terms of CIMT between valproic acid and levetiracetam groups (0.61 ±â€¯0.09 mm vs. 0.57 ±â€¯0.07 mm; p = 0.07). EATT measurements were significantly higher in valproic acid group compared to levetiracetam group (6.14 ±â€¯0.8 mm vs. 5.7 ±â€¯0.7 mm; p = 0.02). CIMT and EATT values were not associated with the dosage and duration of each antiepileptic drug. CONCLUSION: Non-obese children with epilepsy receiving valproic acid or levetiracetam monotherapy might have an increased risk for developing subclinical atherosclerosis despite normal lipid levels. The effect of valproic acid was more evident especially on EATT.

Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission.

Aghdasi-Bornaun H, Kutluk G, Keskindemirci G, Öztarhan K, Dedeoglu R, Yilmaz N, Tosun Ö. Evaluation of autonomic nervous system functions in frame of heart rate variability in children with inflammatory bowel disease in remission. Turk J Pediatr 2018; 60: 407-414. Heart Rate Variability (HRV) is one of the reliable and noninvasive parameters to evaluate autonomic control of the cardiovascular system in patients. The aim of our study was to assess autonomic function in pediatric patients with inflammatory bowel disease (IBD) in remission using Power Spectral Analysis of HRV. Autonomic cardiovascular function was evaluated by time and frequency-domain indexes of spontaneous heart rate in 36 IBD children patients in remission phase and 36 sex and age matched healthy controls. Twenty children with Ulcerative Colitis (UC) and 16 patients with Crohn`s disease (CD) were diagnosed according to their history, physical and laboratory examination, endoscopic, histopathological and radiological findings of upper and lower gastrointestinal system. Significant decrease was observed at HRV parameters in IBD patients when compared with control group. These differences was found in some of time domain parameters (NNmean, SDNNtotal, SDNNday) and frequency domain parameters (TP, LF,VLF) (p < 0.05). Also, there was a significant higher minimum heart rate ratio (p < 0.04) in patients in comparison to the control group. In the IBD group, there was a relative tendency for parasympathetic suppression and sympathetic predominance which reflects an autonomic dysfunction. This imbalance has a circadian rhythm and it is more obvious during the day. These observations may suggest a previously unrecognized role of chronic inflammation for autonomic modulation in IBD.

A rare pathology: Levoatriocardinal vein.

OBJECTIVE: Levoatriocardinal vein (LACV) is a rare cardiac pathology that represents a connection between the pulmonary venous and cardinal systems. The aim of the present study was to discuss morphological and clinical characteristics, as well as diagnostic methods, of experience with LACV. METHODS: Records of 11 patients (4 male, 7 female; mean age 79±1.83 days; range 1-390 days) diagnosed with LACV between 2010 and 2014 were retrospectively reviewed. Presence of LACV was confirmed with echocardiography. The primary obstructive lesion associated with cardiac defects and the integrity of the interatrial septum was identified in each patient with left-sided obstruction. RESULTS: Mean weight was 4.4±0.4 kg (range: 2-8). Age at presentation was under 1 year in 82% of patients. Nine patients had left-sided obstruction, and 2 had normal intracardiac anatomy and pulmonary venous return. In patients with left-sided obstruction, LACV was initially demonstrated with echocardiographic evaluation, performed in apical 4-chamber, high parasternal, and subcostal views. Atrial septum was restrictive or intact in patients with left-sided obstructions. LACV originated directly from the left atrium in all patients. CONCLUSION: Levoatriocardinal vein is an extremely rare cardiac pathology, presenting almost exclusively in patients with left-sided obstructive lesions. In patients with left-sided obstructions, LACV must be kept in mind. It may also present in patients with normal intracardiac anatomy and pulmonary venous return.

Heart rate variability improvement in children using transcatheter atrial septal defect closure.

OBJECTIVE: We evaluated autonomic behavior by examining heart rate variability (HRV) in the time domain and frequency domain in pediatric patients who underwent transcatheter closure of atrial septal defect (ASD). METHODS: A prospective study design was used. Holter ECG was performed in a control group of 30 healthy subjects and a group of 47 patients who underwent transcatheter ASD closure. ECG was taken one day before, one day after, and six months after the procedure to evaluate changes in the time domain [SDNN, rMSSD, NN, pNN50(%), and SDANN] and frequency domain (VLF, LF, HF, VHF, and LF/HF) in the patient group. Student's t-test was used to evaluate changes prior to and after the procedure. RESULTS: There were 28 females (60%) in the patient group and 21 females (70%) in the control group. The mean age and weight of the participants in the patient group were 9.61±4.72 years and 32.40±19.60 kg, respectively; the mean age and weight of the control subjects were 10.43±5.31 years and 32.83±13.00 kg, respectively. In both the time domain and frequency domain analyses, the patient group values were found to be lower than those in the control group prior to the procedure; the values in the patient group were found to approach the values in the control group following the procedure. By the sixth month, the values in the patient group reached the control levels with no statistically significant difference (SDNN: 145±0.84, 137.50±42.50; rMSSD: 72.18±48.22, 58.14±28.49; SDANN: 125.13±13.50, 122.40±41.06; VLF: 112.85±29.07, 114.41±98.39; LF: 50.40±24.09, 45.69±15.13; HF: 39.28±19.86, 44.29±13.14; VHF: 10.29±4.24, 9.99±6.47; LF/HF: 1.90±1.44, 1.24±0.81; p>0.05). CONCLUSION: The transcatheter closure of secundum ASDs was found to have a positive effect on HRV. Consequently, it may contribute to reduced mortality and morbidity. We can conclude that in children, HRV recovers approximately six months after transcatheter ASD closure.

Impact of Coronary Artery Anatomy in Arterial Switch Procedure on Early Mortality and Morbidity.

OBJECTIVE: Arterial switch operation has become the treatment of choice for neonates with transposition of the great arteries. The most important step of the procedure is transferring the coronary arteries to the neoaorta successfully. This study shows the impact of coronary anatomy on early mortality and morbidity after arterial switch operation. METHODS: Ninety-two patients with transposition of the great arteries who underwent arterial switch operation between October 2010 and September 2014 were included in this retrospective study. The patients were classified into two groups: group I (n = 68, patients with usual coronary artery anatomy) and group II (n = 24, patients with unusual coronary artery anatomy). Median age was 10 days (6-25 days) in group I and 14 days (7-29 days) in group II. In group I, 25 patients had ventricular septal defect, nine patients had coarctation of the aorta or distal aortic arch hypoplasia, seven patients had Taussig Bing anomaly. In group II, nine patients had ventricular septal defect, one patient had coarctation of aorta, and one patient had Taussig Bing anomaly. RESULTS: Regarding the postoperative variables, no significant statistical difference was found between two groups. But cardiopulmonary bypass time is significantly longer in group II (P = .004). There was no difference in complications including the mortality (P = .265). It is statistically found that associated anomalies did not affect the mortality and complication rates. All mortality cases (n = 4) were related to ventricular dysfunction in group II, whereas only four of the eight patients died because of ventricular dysfunction in group I. CONCLUSION: Coronary artery pattern was not a predictor of mortality in early postoperative period. There may be an impact of unusual coronary artery pattern on the development of ventricular dysfunction as a cause of mortality.

Complete atrioventricular septal defect and pulmonary stenosis diagnosed in a 49-year-old woman after 10 uneventful births.

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature.

Atrial fibrillation in a healthy adolescent after heavy smoking of contraband cigarettes.

The use of contraband cigarettes is a serious public health problem. We present a case of atrial fibrillation in a healthy adolescent suspected to be caused by smoking contraband cigarettes. A 15-year-old man was admitted to our emergency department experiencing syncope and palpitations. He was a cigarette smoker, but he had never smoked any illicit tobacco products before. He had finished a pack of counterfeit cigarettes (20 pieces) in 1.5 h. His electrocardiogram showed atrial fibrillation with a rapid ventricular response and irregular RR intervals. The patient had no history of alcohol use, surgery, palpitations, hypertension, chronic bronchitis, or any infectious diseases. His atrial fibrillation was converted to a normal sinus rhythm after the cardioversion treatment. Our patient was discharged from the pediatric cardiology service and advised to quit smoking cigarettes, strictly warning against illicit tobacco products. In conclusion, intensive smoking of counterfeit cigarettes may lead to occurrences of atrial fibrillation.