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Objective: To assess endothelial dysfunction and the risk for coronary atherosclerosis in children with irreversible pulmonary hypertension due to congenital heart disease (CHD). Methods: The study included 18 cyanotic patients (the mean age was 12.28 ± 3.26 years) who developed irreversible pulmonary hypertension due to cyanotic and acyanotic CHDs, and 18 control patients (the mean age was 11.78 ± 3.00 years). Study groups were compared for flow-mediated dilatation (FMD), carotid intima media thickness (CIMT) and atherosclerotic risk factors. Results: Compared to the control group, the mean FMD was significantly reduced in the cyanotic group (5.26 ± 2.42% and 9.48 ± 2.60%, respectively; P-value < 0.001). No significant difference was observed between the groups in CIMT (0.41 ± 0.08 mm and 0.39 ± 0.06 mm, respectively; P-value = 0.299). The levels of total cholesterol, low-density lipoprotein-cholesterol and very low-density lipoprotein-cholesterol were statistically significantly lower compared tothe control group (P-value = 0.001, 0.006 and 0.014, respectively), whereas no statistically significant difference was found in the levels of high-density lipoprotein-cholesterol and triglycerides (P-value = 0.113 and 0.975, respectively). Conclusions: Systemic endothelial dysfunction in children with irreversible pulmonary hypertension due to CHD was noted but there was no increased risk for atherosclerosis.

Diabetic autonomic neuropathy (DAN) commonly complicates diabetes and is associated with increased mortality rates over 5 yr. This fact denotes the significance of DAN prevention, mainly with effective glycemic control. However, total prevention of autonomic neuropathy in diabetic patients is not achievable. Thus, the timely detection of DAN and the use of effective means to improve autonomic nervous system function or slow down its progression become of utmost significance. Heart rate variability (HRV) is a technique that measures the beat-to-beat variability in RR intervals, which reflects changes in autonomic activity and their impact on cardiovascular function. Circadian variation in time and frequency domains of heart variability has been shown to correlate with circadian rhythm of ambulatory ischemia and suggests that relative changes in vagal and sympathetic tone at different times during the day may have a direct relationship to the severity of clinical events. Forty-seven (21 boys and 26 girls) type I insulin-dependent diabetics and 46 control subjects (19 boys and 27 girls) were included in the study. Our investigation demonstrated that overall HRV is markedly depressed in diabetes mellitus (DM). All time domain parameters except standard deviation of all 5-min mean RR intervals and all frequency domain indices maintain significant circadian variation. These changes in overall HRV and HRV circadian rhythms reflect significant reductions in cardiac parasympathetic activity and, possibly, increased sympathetic tone.

Simsek A, Turan O, Ciftel M, Kardelen F, Durmaz E, Ozdem S, Akcurin G, Ertug H. Evaluation of left ventricular functions with twodimensional speckle-tracking echocardiography (2D-STE) and N-terminal ProBNP in diabetic children. Turk J Pediatr 2018; 60: 633-641. The purpose of this study was to examine the existence of subclinical left ventricular dysfunction by using 2D-STE and NT-ProBNP levels in children and adolescent patients with type 1 diabetes mellitus. Furthermore, it was also aimed to investigate the effects of the diabetes duration and the metabolic control of the disease on cardiac functions. The patient group was composed of 63 children who were being followed up for the type 1 diabetes mellitus. The control group was composed of 36 healthy children who were of the similar age. Patients with type 1 diabetes mellitus were divided into groups; according to the duration of the disease; group 1: 3-5 years, group 2: 6-10 years of follow-up. The conventional echocardiography and 2D-STE were applied to all of the patients and control individuals. NT-Pro BNP level was measured in the diabetes group. In the conventional echocardiographic examination; there was no difference between the patient and control groups in terms of left ventricular systolic functions, left ventricular diastolic functions; late-diastolic flow velocity in mitral valve (A) values increased and E-wave/A-wave ratio (E/A ) values decreased in diabetes mellitus patients. According to the 2D-STE results; global longitudinal strain, (-17.28±2.24 vs. -19.49±2.22; p < 0.05) and circumferential strain (-12.86±3.19 vs. -17.71±4.62; p < 0.05) were lower in diabetic patients compared to the parameters of control group individuals. There was no difference between levels of NT-ProBNP of the group 1 and group 2 diabetes mellitus patients. Our study showed that there was a dysfunction on the left ventricular systolic functions of the patients with type 1 diabetes mellitus. NT-Pro BNP levels were not considered as a distinguishing factor for the early stages of diabetes mellitus.

Trisomy 18 is the second most common autosomal trisomy in liveborn infants. Various congenital malformations, mental retardation, and high rate of infant mortality in the first year of life are characteristic features of trisomy 18. Congenital heart disease occurs in over 90% of these patients and the most common cardiac lesions are ventricular septal defect, patent ductus arteriosus and atrial septal defect. This is a case report of a baby born with trisomy 18 (postnatal diagnosis) in whom there was an unusual echocardiographic appearance of a mobile structure (“flap-like”) around the area of a VSD—which was imaged prenatally. (Echocardiography 2011;28:E191-E193)

Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) is a rare congenital heart defect and generally associated with other cardiac anomalies. Patients with AORPA have high pulmonary fl ow results in congestive heart failure and unilateral pulmonary hypertension. Thus, it is frequently a fatal malformation in the neonatal or early infancy periods if early surgical repair is not performed. Herein, we report a 1.5 month-old girl who was diagnosed with AORPA associated with aortopulmonary window and interrupted aortic arch and successfully operated using a single pericardial xenograft 3D patch.

Objective: The objective of this study is to investigate endothelial dysfunction (ED) and arterial stiffness (AS) and determine the association with diastolic dysfunction in children with type 1 diabetes mellitus (DM). Methods: A total of 42 patients without diabetic complications (mean age: 13.21 years) and 40 healthy (mean age: 13.07 years) children were included in this study. AS was assessed with ascending aorta M-mode measurements, diastolic dysfunction with pulsed wave (PW) Doppler and tissue Doppler echocardiography measurements and flow-mediated dilatation (FMD) and carotid intima–media thickness (CIMT) with high-resolution ultrasonography. Results: Results of diabetic group and healthy children were compared. In diabetic group, aortic strain (8.40 ± 2.98, 20.12 ± 5.04; p < 0.001), aortic distensibility (7.36 ± 2.92, 16.59 ± 4.25; p < 0.001) and FMD% (7.70 ± 2.83, 11.33 ± 2.85; p < 0.001) were found decreased, and CIMT (0.52 ± 0.09 mm, 0.47 ± 0.08 mm; p < 0.05) was found increased. Additionally, left ventricular lateral segment and right ventricular free-wall isovolumic relaxation time (IVRT) and myocardial performance index (MPI) were found increased. Correlation analyses demonstrated a negative correlation between FMD and IVRT and MPI. Conclusions: ED and AS were found in type 1 DM patients without diabetic complications. Additionally, correlation was shown between increased AS and ED and right and left ventricular diastolic dysfunctions.

We aimed to assess early-onset chronic progressive cardiotoxicity in the left and right ventricles with increasing cumulative anthracycline doses. We evaluated 72 patients within the first year after doxorubicin and/or daunorubicin treatment (median 1.3 months; range 0.3–11.5) and 31 healthy controls. Pretreatment and posttreatment QT interval analyzes were performed in 27 newly diagnosed patients. The echocardiographic data of all examinations of 72 patients were classified into three groups according to instant cumulative anthracycline doses: treatment group (TG)-I (≤120 mg/m2; n = 26), TG-II (120–240 mg/m2; n = 39), and TG-III (≥240 mg/m2; n = 40). Diastolic and systolic parameters were analyzed by conventional echocardiography and tissue Doppler imaging (TDI) and compared with those of healthy controls. The mean age for patients and controls was 8.2 ± 4.5 and 9.6 ± 4.2 years, respectively (p > 0.05). QTc dispersion significantly increased after anthracycline treatment (p = 0.02). TDI showed decreased E′ velocity (p

Plasma concentrations of inflammatory cytokines (IL-1α, IL-1β, IL-6, IL-8 and TNFα) were determined by ELISA in 27 patients with acute rheumatic fever (RF), 12 with only arthritis (RFA) and 15 with rheumatic heart disease (RHD), before, during and after treatment. Altogether, significant increases in TNFα, IL-8 and IL-6 levels were observed in the acute phase as compared to the data found during and after treatment. No significant differences were observed for the other cytokines. Elevations of one or more of the inflammatory cytokines were observed in 9 of 12 patients with RFA, and 12 of 15 with RHD. Increase of TNFα (6/9) and IL-8 (5/9) levels were higher in RHD patients with cardiac failure. These cytokines were below the detection limits on day 7 of treatment in all 22 patients, except in two, and in all 10 days after treatment. Conclusions These findings suggest that inflammatory cytokines, as TNFα, IL-8 and IL-6, may play a patho‐genic role in rheumatic fever.

PurposeThere may be an increase in the risk of atrial arrhythmia due to left atrial enlargement and the influence on conduction system in acute rheumatic fever. The aim of this study is to investigate atrial electromechanical delay and P-wave dispersion in patients with acute rheumatic fever.PatientsA total of 48 patients diagnosed with acute rheumatic fever and 40 volunteers of similar age, sex, and body mass index were included in the study. The study groups were compared for M-mode echocardiographic parameters, interatrial electromechanical delay, intra-atrial electromechanical delay, and P-wave dispersion.ResultsMaximum P-wave duration, P-wave dispersion, and interatrial electromechanical delay were significantly higher in patients with acute rheumatic fever compared with the control group (p < 0.001). However, there was no difference in terms of intra-atrial electromechanical delay (p > 0.05). For patients with acute rheumatic fever, a positive correlation was identified between the left atrium diameter and the P-wave dispersion and interatrial electromechanical delay (r = 0.524 and p < 0.001, and r = 0.351 and p = 0.014, respectively). Furthermore, an important correlation was also identified between the P-wave dispersion and the interatrial electromechanical delay (r = 0.494 and p < 0.001).ConclusionThis study shows the prolongation of P-wave dispersion and interatrial electromechanical delay in acute rheumatic fever. Left atrial enlargement can be one of the underlying reasons for the increase in P-wave dispersion and interatrial electromechanical delay.

Kawasaki Disease (KD) is a vasculitic disease and can affect any organ system in the body. The development of coronary artery aneurysms is the most common and life threatening complication of KD and makes this disease the leading cause of acquired heart disease in children in the developed world. Facial nerve palsy has been reported as a possible marker of more severe disease and increased risk of coronary artery involvement in KD. Herein, the authors report an 8-mo-old infant who had left sided facial nerve palsy and multiple coronary aneurysms associated with KD.

Trisomy 18 is the second most common autosomal trisomy in liveborn infants. Various congenital malformations, mental retardation, and high rate of infant mortality in the first year of life are characteristic features of trisomy 18. Congenital heart disease occurs in over 90% of these patients and the most common cardiac lesions are ventricular septal defect, patent ductus arteriosus and atrial septal defect. This is a case report of a baby born with trisomy 18 (postnatal diagnosis) in whom there was an unusual echocardiographic appearance of a mobile structure ("flap-like") around the area of a VSD-which was imaged prenatally.

An infant under follow-up evaluation since the fetal stage was detected to have subclavian steal syndrome (SSS) associated with heterotaxy syndrome and atrioventricular septal defect. Such a complex association has not been reported previously. Congenital SSS occurs as a result of a subclavian artery originating from the main pulmonary artery through the ductus instead of the aortic arch. In rare cases, as in the reported case, it may occur due to proximal segment atresia of the left subclavian artery.

Cardiac hydatid disease is uncommon, especially in children. A 13-year-old girl had pulmonary and renal hydatidosis diagnosed by chest radiography, computed tomography, and magnetic resonance imaging. Serologic tests for hydatidosis were positive. A right atrial hydatid cyst was diagnosed on two-dimensional echo-cardiography. Medical treatment with albendazole was started and the cyst was successfully removed.

Objective Congenital heart disease (CHD) associated with thyroid disease has been reported in Down syndrome (DS). The purpose of this work was to assess abnormalities of the thyroid in relation to the frequency and type of CHD on admission among children with DS. Methods This retrospective study included 187 children with DS between August 1993- December 2005. Karyotype analysis, thyroid function tests and echocardiographic studies were performed in all children with DS. If necessary, hemodynamic study by catheterization was carried out. Thyrotropin releasing hormone (TRH) stimulation test was performed in having elevated thyroid stimulating hormone (TSH) level. Statistical analyses were performed using Chi-square, "t" test for independent samples or Mann-Whitney U test. Results It was found that 136 (72.73%) patients with DS had CHD. The age difference at the time of admission was statistically significant for these two groups (p=0.001) in children with /without CHD. There were 12 (11.88%) patients with congenital hypothyroidism and DS, of whom 11 had CHD. There were statistically significant differences in the levels of TSH and total thyroxine (tT4) between congenital and subclinical hypothyroid and euthyroid groups (p=0.001 for TSH and p= 0.001 for tT4). But, there was no significant relationship between having any kind of CHD and levels of TSH and tT4. Conclusion Our data suggest that all patients with DS should be evaluated with careful physical and echocardiographic examination on admission. In addition, congenital or subclinical hypothyroidism should also be kept in mind in children with DS and monitored accordingly.

Previous studies suggested that abnormal regulation of TNF-alpha production may have a role in the pathogenesis of rheumatic fever (RF). Polymorphism at the promoter region of TNF-alpha gene (-308 A) has recently been shown to be associated with rheumatic heart disease (RHD) in Mexican patients. Although this polymorphism has long been shown to affect TNF-alpha gene expression in cell lines, its role in production of the cytokine in RF patients has not been studied. We therefore investigated TNF-alpha G-308A single nucleotide polymorphism and its effect on TNF-alpha production in 71 Turkish RF patients and 89 ethnically matched healthy controls. The TNF-alpha-308A allele frequency was found to be significantly higher in RF patients (RHD + arthritis) than in healthy controls [p < 0.0032 Odds ratio (OR) = 3.4, 95% confidence interval (CI) (1.5-7.7)]. When RHD patients were analyzed as a separate group, significant difference persisted [p < 0.0055, OR = 3.3, 95% CI (1.5-7.6)]. More importantly, ELISPOT analysis demonstrated that existence of A allele was associated with higher TNF-alpha production compared with G allele. Our data suggest that carrying a high responder TNF-alpha-308A allele may be a genetic factor in increasing the susceptibility to develop RF disease. © 2005 Elsevier Ltd. All rights reserved., Scientific Research Foundation of Beijing Normal University, This study was supported by Akdeniz University Scientific Research Foundation.

A screening program was conducted to ascertain the incidence of hemoglobinopathies in the district of Antalya, Turkey. The survey sample was selected from the household registration forms of health centers by systematic random sampling. Heparinized blood samples were collected from 1,616 subjects from 884 families. The prevalence of beta thalassemia traits with increased Hb A2 was 10.2%. This is higher than that found in previous studies performed in Antalya. The prevalence of abnormal hemoglobins (Hbs) was found to be 0.8%. Four subjects had Hb AS; five had Hb D-Los Angeles (B 121 [GH4] Glu-Gln); one had Hb Ube-2 (68 [E1] Ans-Asp), one had Hb P-Nilotic (fusion between B 22 and) and two had Hb D-like variants.