Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms.

Yilmazer MM, Özdemir R, Mese T, Küçük M, Öner T, Devrim I, Bayram N, Güven B, Tavli V. Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms. Turk J Pediatr 2019; 61: 648-656. Prompt diagnosis and the administration of intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery abnormalities (CAA) in Kawasaki Disease (KD). The resistance to treatment and development of the coronary sequelae remain the most important problems in KD. We aimed to determine the predicting factors of nonresponse to initial IVIG therapy and to analyze the cases who had giant coronary aneurysms. A total of 120 KD cases, including 61 children fulfilling the criteria for KD and 59 with incomplete KD were enrolled into this study. Demographic, laboratory, clinical, echocardiographic characteristics, and treatment regimens were reviewed, retrospectively. The median age of the patients was 33.5 months (range: 3-168 months). Coronary artery aneurysms were detected in 35 patients (29%) at the time of diagnosis. Twenty-eight patients had coronary aneurysms small or medium in size, one had a large, and seven had giant coronary aneurysms. CAA persisted in 8 cases in the follow-up, all of which were large or giant aneurysms. A ten month-old girl with a giant coronary aneurysm was referred to coronary bypass surgery in the subacute phase of follow-up, due to myocardial ischemia. Eighteen patients were unresponsive to the initial IVIG therapy (%15), of whom 10 were diagnosed as cKD and 8 were iKD. Patients who did not respond to initial IVIG therapy, had higher white blood cell (WBC) count, higher C-reactive protein (CRP) and lower albumin levels than those who did (P < 0.05). In univarite analysis; CRP, WBC and albumin were found to be significant predictors of nonresponse to initial IVIG therapy, while a stepwise multiple linear regression analysis showed that WBC count and albumin levels were significantly correlated with nonresponse to initial treatment with IVIG. Our study showed that WBC count and albumin levels might be used as predictors of nonresponse to the IVIG therapy in Turkish children with KD.

Cardiac function in children with premature ventricular contractions: the effect of omega-3 polyunsaturated fatty acid supplementation.

BACKGROUND: Premature ventricular contractions are accepted as benign in structurally normal hearts. However, reversible cardiomyopathy can sometimes develop. Omega-3 polyunsaturated fatty acids have anti-arrhythmic properties in animals and humans.AimWe evaluated left ventricular function in children with premature ventricular contractions with normal cardiac anatomy and assessed the impact of omega-3 fatty acid supplementation on left ventricular function in a prospective trial. METHODS: A total of 25 patients with premature ventricular contraction, with more than 2% premature ventricular contractions on 24-hour Holter electrocardiography, and 30 healthy patients were included into study. All patients underwent electrocardiography, left ventricular M-mode echocardiography, and myocardial performance index testing. Patients with premature ventricular contraction were given omega-3 fatty acids at a dose of 1 g/day for 3 months, and control echocardiography and 24-hour Holter electrocardiography were performed. Neither placebo nor omega-3 fatty acids were given to the control group. RESULTS: Compared with the values of the control group, the patients with premature ventricular contraction had significantly lower fractional shortening. The myocardial performance index decreased markedly in the patient groups. The mean heart rate and mean premature ventricular contraction percentage of Group 2 significantly decreased in comparison with their baseline values after the omega-3 supplementation. CONCLUSION: In conclusion, premature ventricular contractions can lead to systolic cardiac dysfunction in children. Omega-3 supplementation may improve cardiac function in children with premature ventricular contractions. This is the first study conducted in children to investigate the possible role of omega-3 fatty acid supplementation on treatment of premature ventricular contractions.

Mid-term results of Solysafe® septal occluder for percutaneous closure of secundum atrial septal defects.

BACKGROUND: In this article, we report mid-term follow-up results of the Solysafe® septal occluder for percutaneous closure of secundum atrial septal defects. METHODS: A total of 25 patients (8 males, 17 females; mean age 8.4±3.6 years; range 5 to 12 years) who underwent percutaneous closure of secundum atrial septal defect between July 2008 and June 2010 were included in this study. RESULTS: The mean follow-up was 6.1±0.5 (range, 5.2 to 7.2) years. The device was successfully implanted in 22 of 25 patients. The mean stretched diameter of the atrial septal defect as assessed by balloon sizing was 13.6±4.4 (range, 8 to 26) mm. Nine 15-mm devices, eight 20-mm devices, six 25-mm devices, and two 35-mm devices were used. A 20-mm and two 35-mm devices were used in three patients and the procedure failed in these patients. Among the remaining 22 patients, no pericardial effusion, endocarditis, hemolysis, electrocardiographic changes, valvular problems, or suspicious echocardiographic findings were observed during or after the procedure. Only in one patient, a wire fraction was seen at six years, while another patient had a residual shunt during a six-year follow-up. Device embolization (n=1) and hemiparesis (n=1) were the early major complications related to the procedure. CONCLUSION: Although percutaneous closure of secundum atrial septal defects is successful, it would be wiser to check the device regularly, at least once a year, as the manufacturing of the device has been discontinued due to wire fractions.

Echocardiographic evaluation of cardiac functions in newborns of mildly preeclamptic pregnant women within postnatal 24-48 hours.

The aim of this study is to detect preeclampsia-related cardiac dysfunction within 24-48 hours of delivery in newborns born from preeclamptic mothers. Forty newborns from mildly preeclamptic mothers formed the study group and the control group was formed by 40 healthy newborns. Cardiac function for the groups were evaluated using conventional echocardiography and myocardial performance index (MPI) within the first 24-48 hours of their lifetime and the results of both groups were compared. A significant difference between the groups was observed especially in the PW Doppler MPI measurements (the left ventricle MPI 0.37 ± 0.09 and 0.26 ± 0.11, p < .001; the right ventricle MPI 0.29 ± 0.08 and 0.26 ± 0.07, p < .035) for the control group and the study group. Elongation in the left and right ventricle MPI was detected to be more significant in terms of comparing systolic and diastolic functions to determine preeclampsia-related cardiac injury in newborns from preeclamptic mothers within the first 24-48 hours of their lifetime. Impact statement Today, the methods which may detect cardiac injury earlier than conventional echocardiographic methods are used for evaluating cardiac functions. Among them, myocardial performance index (MPI) measurement with PW Doppler is the most common ones. While studies are available in the literature evaluating foetal cardiac functions with MPI in foetuses of preeclamptic women, studies evaluating cardiac functions with MPI index within the first 24-48 hours in postnatal period are not available. This is the first study to detect cardiac injury by measuring cardiac functions of the newborns of preeclamptic babies using conventional echocardiography (EF, SF, mitral and tricuspid E/A) and myocardial performance index within the first 24-48 hours of life and compare these values with those of a control group composed of healthy newborns with similar demographic characteristics. According to the results of the study, elongation in right and left ventricle MPI was detected to be more significant compared to systolic and diastolic functions for determining preeclampsia-related cardiac injury in newborns of preeclamptic mothers within 24-48 hours of delivery. Ventricle functions of the newborns of preeclamptic mothers should also be evaluated with MPI measurement besides conventional echocardiographic measurements.

Parameters indicative of persistence of valvular pathology at initial diagnosis in acute rheumatic carditis: the role of albumin and CD19 expression / Parâmetros indicativos de persistência de patologia valvular no diagnóstico inicial de cardite reumática aguda: o papel da albumina e da expressão de CD19

Abstract 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: 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 feitos 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 6º mês de acompanhamento. Os pacientes foram divididos em dois grupos de acordo com a persistência da patologia valvular no 6º 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%) apresentaram regressão da patologia valvular. Houve desaparecimento de regurgitação moderada inicial em oito pacientes (36,3%). Entre sete (31,8%) pacientes com regurgitação moderada inicialmente, a regurgitação desapareceu em três e quatro apresentaram melhoria para regurgitação leve. Dois pacientes com regurgitação grave inicialmente apresentaram melhoria para regurgitação moderada (9,1%). Em cinco (22,8%) pacientes o grau de regurgitação (regurgitação moderada em um [4,6%] e regurgitação grave em quatro [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 com o 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.

Parameters indicative of persistence of valvular pathology at initial diagnosis in acute rheumatic carditis: the role of albumin and CD19 expression.

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±0.48g/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.

The association between brain natriuretic peptide and tissue Doppler parameters in children with hypertrophic cardiomyopathy.

In this study, we investigated the association between brain natriuretic peptide (BNP) levels and tissue Doppler imaging measurements and also screening for deadly mutations in patients with hypertrophic cardiomyopathy (HCM). We enrolled 20 patients diagnosed with HCM (age:10.7±5 years (1-17), 85% male, weight:42.25±23.10 kg, height:141.80±32.45 cm) and 20 age, gender and body weight-matched control subjects. We performed electrocardiography, transthoracic echocardiography, and tissue Doppler echocardiography in each group, as well as genetic tests (for Arg403Gln, Arg453Cys, Arg719Trp and Arg719Gln mutations in MYH7 Exons 13, 14, 19) and BNP in the patients. The patients were divided into two groups according to the presence (Group 1) or absence (Group 2) of left ventricular (LV) outflow tract obstruction. QTc dispersion and the LV ejection fraction and left atrial (LA) volume index were increased in Group 1. The LA volume index and the mitral and septal E/Ea ratio and septum Z-score were increased while the mitral lateral annulus and septal annulus Ea wave velocities and the mitral and tricuspid E/A ratio were decreased in patients with high levels of BNP compared to those with normal BNP levels. There were no mutations that are associated with increased risk of sudden death found in patients included in this study. In the light of our data, we conclude that such parameters BNP levels above the 98 pg/mL, septal thickness Z-score ˃6, and higher mitral and septal E/Ea ratios can be used for management of patients with HCM according to life-threatening conditions.

Evaluation of myocardial function in pediatric patients with transposition of great arteries after arterial switch operation.

OBJECTIVE: An arterial switch operation converts the left ventricle (LV) into the systemic ventricle, replacing the right ventricle (RV), which is the systemic ventricle during the prenatal period. The procedure is performed in patients with arterial transposition and those in whom a coronary reimplantation procedure is performed. Therefore, the adaptation of LV and RV to the arterial switch operations is an interesting issue. This study aimed to evaluate systolic and diastolic functions in the LV and RV myocardium using echocardiography in pediatric patients with transposed great arteries after an arterial switch operation. METHODS: This observational case control study included 28 patients and 20 gender-and age-matched healthy controls. A group study was performed using patients who were followed for at least 6 months after the operation and who visited the pediatric cardiology outpatient between October 2009 and May 2011. Systolic and diastolic parameters, LV and RV myocardial performance index (MPI) values, and left atrium (LA) volume index were assessed in both groups. RESULTS: The LV MPI and tricuspid valve (TV) E/Ea ratio measured using pulsed-wave Doppler were higher in the patient group than in the control group. The TV E/A ratio and late velocity of lateral mitral annulus (Aa) and systolic velocity (Sa)-early diastolic velocity (Ea)-late velocity (Aa) of the septal mitral annulus and systolic velocity (Sa)-early diastolic velocity (Ea)-late velocity (Aa) of the lateral tricuspid annulus were lower in the patient group than in the control group. CONCLUSION: Considering the present study's findings obtained during short-to mid-term follow-up, the RV tissue Doppler flows and the LV MPI were found impaired.

Assessment of Microvolt T Wave Alternans in Children with Repaired Tetralogy of Fallot during 24-Hour Holter Electrocardiography.

BACKGROUND: We aimed to examine microvolt T wave alternans (MTWA) in 24-hour Holter electrocardiography (ECG) of children with repaired tetralogy of Fallot (TOF) to assess associations of MTWA with ventricular arrhythmias, ECG parameters, and echocardiographic findings. METHODS: Holter ECG records and archive files of 56 repaired TOF patients (62.5% male) who were analyzed retrospectively. Subjects' ECG parameters and MTWA values were compared with age-sex-matched control group. T wave changes were analyzed by time-domain-modified moving average method from the three channels of 24-hour Holter ECG. RESULTS: Mean age was 123.4 ± 48.3 months. Median MTWA value was 55.5 µV in the control group, whereas 95.5 µV in patients group (P < 0.001). A significant weak positive correlation was found between the presence of ventricular extrasystoles and tricuspid regurgitation. There was no correlation between ECG parameters, echocardiographic findings, and MTWA. CONCLUSIONS: MTWA was increased in children with repaired TOF as reported before. To our knowledge, this is the first study analyzing MTWA with 24-hour Holter ECG in repaired TOF patients.

Risk factors for persistence of coronary artery abnormalities in Turkish children with Kawasaki disease.

The aim of this study was to identify the risk factors for persistence of coronary artery abnormalities (CAAs) in Kawasaki disease and to compare the differences between complete (n=25) and incomplete (n=18) forms of the disease in relation to CAAs. The patients' demographic (age and sex), clinical (season of admission, duration of fever, form of Kawasaki disease), laboratory (complete blood count, eosinophil count, serum biochemistry, C-reactive protein level, erythrocyte sedimentation rate [ESR], urinary analysis), echocardiographic and therapeutic data were evaluated retrospectively from the medical records. The incidence of sterile pyuria was nearly twofold greater in patients with initial CAAs than in patients in which CAAs were not present (25% and 13%, respectively; p=0.33). In multivariate logistic regression analysis, ESR and pyuria were found to be associated with persistence of CAAs (p=0.035 and p=0.046, respectively). In addition, we found that duration of fever was significantly associated with persistence of CAAs (p=0.045). However, gender, age at presentation, peripheral blood eosinophilia, low albumin level, CRP, leukocytosis and anemia were not predictive for persistence of CAAs. There was no difference between the complete and incomplete form of the disease in regard to persistence of CAAs. As a result, we have determined that duration of fever, high levels of ESR and presence of sterile pyuria can be used to predict the persistence of CAAs in Kawasaki disease.

Pulmonary and ventricular functions in children with repaired tetralogy of Fallot.

OBJECTIVE: This study aimed to evaluate biventricular function, brain natriuretic peptide levels, respiratory function test and 6 minute walking test (6MWT) in children with repaired tetralogy of Fallot (TOF), and analyse the correlation between these variables and clinical status. METHODS: Twenty-five children (14 boys, 11 girls; aged 6 to 17 years) with repaired TOF (Group 1) and 25 age-sex matched healthy controls (Group 2) were enrolled in the study. Tissue Doppler echocardiography, respiratory function test, 6MWT distance and brain natriuretic peptide levels were measured. RESULTS: Mean ages of the children at TOF corrective surgery and at study time were 5.1±3.5 years and 11.6±2.7 years respectively. The duration between palliative operation and corrective surgery was 4.3±2.0 years, and the follow-up period after corrective surgery was 6.3±3.0 years. The right ventricular and left ventricular myocardial performance indices (MPIs), and isovolumic relaxation and contraction times were significantly higher in Group 1 than in Group 2 (p<0.01). Spirometry displayed significantly reduced forced vital capacity (FVC), forced expiratory volume in one second (FEV1), forced expiratory flow 25-75% (FEF25-75) and inspirational capacity in Group 1 compared to Group 2 (p<0.01). In Group 1, 6MWT distances were significantly lower than in Group 2 (p=0.001). Right ventricular MPI is correlated with FEV1, FVC and 6MWT distance in the current study. CONCLUSION: The children with repaired TOF had impaired ventricular and pulmonary functions. Hence, right ventricular MPI along with FEV1, FVC and 6MWT distance may be useful in the follow-up of children with repaired TOF.

Atrial Functions and Aortic Elasticity in Children with Aortic Coarctation.

BACKGROUND: Coarctation of the aorta (CoA) is a chronic vascular disease characterized by a persistence of myocardial and vascular alterations. We aimed to evaluate children who have had successful coarctation surgery or balloon dilatation to evaluate the elasticity of the aorta, left atrial ejection force (AEF) and myocardial performance collectively at midterm follow-up. METHODS: Nineteen patients (7.15 ± 0.9 years of age) and 21 age-sex matched healthy children were included in this study. Left AEF index is defined as the product of mass and acceleration of blood expelled from the left atrium. Aortic stiffness and distensibility were estimated using ascending and descending aorta diameters. RESULTS: The left atrial force index [(g∙cm/s(2))/m(2)] in the patient group was found to be significantly higher (12.69 ± 7.29, 5.74 ± 2.59, respectively, p = 0.001). Distensibility of the ascending aorta (cm(2)/dynes 10(-6)) was significantly lower in the patient group than in the control group (42.13 ± 24.02, 78.79 ± 20.49, respectively, p < 0.001). The stiffness index of the ascending aorta was significantly higher in the patient group (p < 0.001). We also documented that atrial force index is associated with peak E velocity, right arm systolic blood pressure and left ventricular mass index. CONCLUSIONS: Our investigation showed that AEF is higher in children who have had successful coarctation surgery or balloon dilatation, and AEF is associated with systolic blood pressure, peak E velocity and left ventricular mass index. Distensibility of the ascending aorta was lower, and stiffness index was higher in children with corrected CoA than in healthy subjects. KEY WORDS: Atrial ejection force; Balloon dilatation; CoA; Coarctation surgery; Distensibility; Stiffness index.

Cardiac Function in Newborns with Congenital Hypothyroidism: Association with Thyroid-Stimulating Hormone Levels.

OBJECTIVE: The aims of this study were to demonstrate ventricular function changes in patients with congenital hypothyroidism and to investigate whether there is an association between any such changes and thyroid-stimulating hormone (TSH) levels using M-mode and Doppler electrocardiography (ECG) and tissue Doppler imaging (TDI). METHODS: Twenty-seven patients 5-30 days of age with congenital hypothyroidism who were scheduled to receive L-thyroxine treatment and 20 healthy newborns were included in this study. Twelve-lead ECG and M-mode TDI recordings of the patient and healthy groups were obtained. The patient group was divided into two subgroups according to TSH level (>100 uIU/mL or <100 uIU/mL), which were then compared on all parameters. RESULTS: Decreases were observed in the ejection fraction (EF), shortening fraction (SF), and mitral lateral annulus, mitral septal annulus, and tricuspid lateral annulus systolic velocity (Sa) on TDI, whereas left ventricular end-systolic diameter (LVESd) and corrected QT interval (QTc) dispersion were significantly increased in the patient group compared with the control group. No significant differences between the groups were found in left ventricular end-diastolic diameter (LVEDd) or heart rate. When the two patient subgroups (TSH >100 uIU/mL and <100 uIU/mL) were compared, TDI septal annulus Sa wave length and heart rate were significantly lower in the TSH >100 group. CONCLUSION: Impairment in left ventricular systolic function and increased risk of arrhythmia were observed in newborn infants with congenital hypothyroidism. TSH level was associated with heart rate and interventricular septum velocity.

Mid-term assessment of cardiac autonomic functions in children with transposition of the great arteries after arterial switch operation.

OBJECTIVE: It has been documented that impaired heart rate variability (HRV) is related to life threatening arrhythmias in children with surgically repaired congenital heart disease. We aimed to analyze the balance of the cardiac autonomic functions by assessing HRV in children with arterial switch operation (ASO). METHODS: In this observational cohort study, HRV analysis using 24-h Holter electrocardiography recordings was examined in 22 patients (mean age: 59.5±38.7 months, 18 male, 4 female) who had undergone ASO during the newborn period and 22 healthy children (mean age: 65.1±39.4 months, 18 male, 4 female). After Kolmogorov-Smirnov testing for normality, Student t-test and Mann-Whitney U test were used when appropriate. Chi-square was used for categorical data. RESULTS: In 24-h HRV analysis showed that SDANN and VLF were significantly higher in patient group. Awake SDNN, rMSSD, pNN50, TP and VLF levels of patient group were significantly higher than those of control subjects. Awake LF/HF ratio in patient group was significantly higher than their counterpart in asleep group. In the patient group, awake rMSSD, pNN50, TP, LF and HF were significantly lower than their counterpart in the asleep group. CONCLUSION: Children with transposition of the great arteries (TGA) following ASO have not decreased levels of time and frequency HRV parameters in the mid-term follow-up period. All HRV parameters reflecting vagal tone were increased in the patient group. It is suggested that vagal tone is more predominant than sympathetic tone for children with ASO.

Carotid artery intima-media thickness in pediatric type 1 diabetic patients.

OBJECTIVE: To compare the carotid artery intima-media thickness in pediatric type 1 diabetic patients with that in healthy control subjects matched for age, sex, height, weight, body mass index (BMI) and waist circumference. METHODS: Fifty diabetic patients and forty-five control subjects were enrolled into this observational, cross-sectional, controlled study. Carotid artery intima-media thickness (cIMT), flow-mediated dilation (FMD) and carotid stiffness index were measured by using a carotid Doppler and real-time ultrasound. Student's t, chi-square and Kolmogorov-Smirnov tests and Pearson's correlation coefficient were used for the statistical analysis. RESULTS: There were no significant differences in the groups for age, sex, height, weight, BMI and waist circumference (mean age 12.10 ± 2.02 vs. 11.49 ± 1.90 years, weight 41.14 ± 11.28 vs. 40.88 ± 11.68 kg, height 149.78 ± 20.3 vs. 145.62 ± 20.14 cm, BMI 18.49 ± 2.64 vs. 18.26 ± 2.59 kg/m2, waist circumference 69.72 ± 8.6 vs. 66.05 ± 7.47 cm, respectively). A significantly higher cIMT was found in the patients with type 1 diabetes (0.49 ± 0.05 vs. 0.44 ± 0.03 mm; p<0.001). A higher carotid stiffness index was found in the diabetic group when compared with control group (3.11 ± 0.46 vs. 2.6 ± 0.29 mm; p<0.001). Carotid IMT was not affected by mean HbA1c level and median HbA1c level (r=0.112, p=0.437 and r=0.249, p=0.082). CONCLUSION: Type 1 diabetes is associated with higher cIMT and carotid stiffness index in a pediatric population.

P-wave dispersion: an indicator of cardiac autonomic dysfunction in children with neurocardiogenic syncope.

Neurocardiogenic syncope is the most frequent cause of fainting in childhood and adolescence. Although head-up tilt table testing (HUTT) was previously considered as the reference standard in the diagnosis of syncope, in children with a typical history of reflex syncope, normal physical examination, and electrocardiogram (ECG) are sufficient to cease investigation; however, according to recent reports, TT is indicated in patients in whom this diagnosis cannot be proven by initial evaluation. The hypothesis of this study is that P-wave dispersion (PWD) can be a useful electrocardiographic predictor of cardiac autonomic dysfunction in children with vasovagal syncope (VVS). The study was designed prospectively and included 50 children with positive and 50 children with negative HUTT who presented with at least two previous unexplained episodes of syncope as well as 50 sex- and age-matched healthy children as the control group. All standard 12-lead ECGs were obtained in patients and controls, and the difference between maximum and minimum durations of the P wave was defined as the PWD. A total of 100 children with VVS and 50 healthy controls were evaluated for the study. The P maximum values of HUTT-positive (HUTT[+]) patients were significantly greater than those in the HUTT-negative (HUTT[-]) and control groups(p < 0.05). In addition, mean PWD values were 50.2 ± 18.5, 39.6 ± 11.2 and 32.0 ± 11.2 ms in the HUTT(+), HUTT(-), and control groups, respectively. The difference between groups was statistically significant (p < 0.05). We suggest that PWD is an early sign of cardiac autonomic dysfunction in children with neurally mediated syncope and can be used as a noninvasive electrocardiographic test to evaluate orthostatic intolerance syndromes.

Antenatal diagnosis of left atrial isomerism and heterotaxy syndrome in fetus with Meckel-Gruber syndrome.

We aimed to present a fetus with Meckel-Gruber syndrome (MKS) who had left atrial isomerism, heterotaxy syndrome and complete heart block. A 26-year-old healthy female was referred to our clinic in the 23rd week of her pregnancy. The fetus had multiple systemic anomalies including fetal heart. Fetal echocardiography revealed a horizontal liver, left-sided stomach and vena cava interruption with azygos continuation. There was also an apical trabecular ventricular septal defect, aorta and pulmonary artery arising from the left ventricle, pulmonary artery hypoplasia, pulmonary valve stenosis and left atrial isomerism. The heart rate was 46/min, consistent with third-degree atrioventricular block. Multiple anomalies including occipital encephalocele, bilateral polycystic kidneys, cleft lip, cleft palate, and polydactyly were also detected in the obstetric ultrasonography. The pregnancy was terminated in the 23rd gestational week based on the consensus of perinatology council. The autopsy examination confirmed the diagnosis of MKS, left atrial isomerism and heterotaxy syndrome. Although some cardiac defects have been reported previously in MKS fetuses, here we expand the cardiac spectrum of anomalies associated with MKS to include left atrial isomerism and heterotaxy syndrome.

Evaluation of left atrial functions in children with chronic renal failure.

OBJECTIVE: One-quarter of deaths in children with chronic renal failure is due to cardiovascular complications. Conventional echocardiographic methods are insufficient for evaluating systolic functions in children with chronic renal failure. The aim of the present study was to investigate cardiac functions in children with chronic renal failure by evaluating left atrial volume and functions. METHODS: The present cross-sectional observational study included 44 children undergoing dialysis, 16 children with chronic renal failure but not yet on dialysis, and 20 healthy control subjects. Transthoracic echocardiography was performed for all children. Variables regarding to left ventricle and atrium (left atrial systolic force, left atrial systolic force index, left atrial volume, left ventricular mass index, and relative wall thickness) were measured using two-dimensional and M-mode echocardiography. RESULTS: Left atrial systolic force index was negatively correlated with systolic blood pressure and left ventricular mass (p=0.01, r=0.266 and p=0.02, r=0.347, respectively). However, it was positively correlated with both early and late diastolic mitral inflow velocity (r=0.518, p=0.001 and r=0.828, p=0.001, respectively). There were no significant difference among the groups in terms of left atrial systolic force index and left atrial volume. However, left atrial systolic force index was higher in children with chronic renal failure but not yet on dialysis. CONCLUSION: Left atrial systolic force was negatively correlated with systolic blood pressure and left ventricular mass. These findings suggested that evaluating left atrial systolic force and left atrial volume were useful to determine diastolic dysfunction and the necessity of dialysis in patient with chronic renal failure.

Coarctation of the aorta with infracardiac total anomalous pulmonary venous drainage: a rare combination.

A 7-day-old female neonate was referred to the pediatric emergency department due to cyanosis and respiratory distress. Her arterial oxygen saturation was 65%. The Coarctation of the aorta, infracardiac total anomalous pulmonary venous drainage without obstruction and pulmonary artery hypertension were diagnosed by echocardiography. Emergency corrective surgery was done without confirming the diagnosis with cardiac magnetic resonance imaging (MRI) or cardiac catheterization due to her poor clinical status. The postoperative course was uneventful and the patient was extubated after the sixth day of surgery and discharged after 14 days.