Incidence and clinical characteristics of acute rheumatic fever in Turkey: Results of a nationwide multicentre study.

AIM: To evaluate the incidence and clinical features of acute rheumatic fever (ARF) in Turkey, following the revised Jones criteria in 2015. METHODS: This multicentre study was designed by the Acquired Heart Diseases Working Group of the Turkish Pediatric Cardiology and Pediatric Cardiac Surgery Association in 2016. The data during the first attack of 1103 ARF patients were collected from the paediatric cardiologists between 1 January 2016 and 31 December 2016. RESULTS: Turkey National Institute of Statistics records of 2016 were used for the determination of ARF incidence with regard to various cities and regions separately. The estimated incidence rate of ARF was 8.84/100 000 in Turkey. The ARF incidence varied considerably among different regions. The highest incidence was found in the Eastern Anatolia Region as 14.4/100 000, and the lowest incidence was found in the Black Sea Region as 3.3/100 000 (P < 0.05). Clinical carditis was the most common finding. The incidence of clinical carditis, subclinical carditis, polyarthritis, aseptic monoarthritis, polyarthralgia and Sydenham's Chorea was 53.5%, 29.1%, 52.8%, 10.3%, 18.6% and 7.9%, respectively. The incidences of clinical carditis, subclinical carditis, polyarthritis and polyarthralgia were found to be significantly different among different regions (P < 0.05). CONCLUSION: The findings of this nationwide screening of ARF suggest that Turkey should be included in the moderate-risk group.

Serum Nickel and Titanium Levels after Transcatheter Closure of Atrial Septal Defects with Amplatzer Septal Occluder.

INTRODUCTION: There is a concern about release of nickel and titanium after implantation of nitinol-containing devices. OBJECTIVE: To evaluate serum nickel and titanium release after implantation of Amplatzer occluder. MATERIALS AND METHODS: In 38 pediatric patients with no history of nickel sensitivity, blood samples were drawn 24 hours before and 24 hours, 1, 3, 6, and 12 months after implantation. Nickel and titanium concentrations were measured by atomic absorption spectrophotometry. RESULTS: The median serum nickel level which was 0.44 ng/mL before the implantation increased to 1.01 ng/mL 24 hours after implantation and 1.72 ng/mL one month after implantation. The maximum level was detected 3 months after implantation, with a median level of 1.96 ng/mL. During follow-up, the nickel levels decreased to those measured before implantation. Serum nickel levels at the 24th hour, 1st month, and 3rd month following implantation were found to have increased significantly. No patients showed a detectable serum titanium level. DISCUSSION: This is the first study that evaluated both serum nickel and titanium release after implantation of the Amplatzer occluder. Our study shows that nickel is released from the device in the first few months after implantation. Therefore, in patients with nickel allergy, other devices may be considered.

Easy Fenestration Technique for Extracardiac Fontan Operation.

During an extracardiac conduit type of Fontan operation, fenestration is not always technically easy, particularly when the right atrium is small and located posteriorly. We describe here an easy technique for performing an anastomosis of a polytetrafluoroethylene ringed graft to the atrium for fenestration in the setting of an extracardiac conduit Fontan operation. The surgical technique starts with a purse string suture that is customized to fit the atrium and utilizes a ringed graft that is inserted into the right atrium through the purse string, which is then tied.

Combined effect of aerosolized iloprost and oxygen on assessment of pulmonary vasoreactivity in children with pulmonary hypertension.

OBJECTIVE: The evaluation of pulmonary vascular reactivity plays a significant role in the management of patients with pulmonary hypertension. Inhaled nitric oxide in combination with oxygen (O2) has become widely used as an agent for pulmonary vasodilator testing. However, inhaled nitric oxide is not available in many developing countries. Recently, aerosolized iloprost was suggested as an alternative to nitric oxide for this purpose. In the present study, aerosolized iloprost was used together with O2 in the pulmonary vasoreactivity test of children with severe pulmonary hypertension. Thus, the synergistic effect of both vasodilators was utilized without extending the duration of cardiac catheterization. METHODS: The prospective cohort study registered a total of 16 children with severe pulmonary hypertension whose median age was 4.5 years. Hemodynamic parameters were quantified before and after the vasoreactivity test. Increased left-to-right shunt, pulmonary vascular resistance of <6 Woods units (WU)/m(2) and a pulmonary-systemic resistance ratio of <0.3, as well as a decrease >10% in the pulmonary vascular resistance and pulmonary-systemic vascular resistance ratio after the vasoreactivity test were accepted as a positive response. The data were analyzed using Wilcoxon signed-rank and the Mann-Whitney U tests. RESULTS: Eleven children gave a positive response to the vasoreactivity test, while 5 children did not respond. Pulmonary vascular resistance dropped from 9.98 ± 1.39 WU/m(2) to 5.08 ± 1.05 WU/m(2) (p=0.013) and the pulmonary-systemic vascular resistance ratio fell from 0.68 ± 0.08 to 0.32 ± 0.05 (p=0.003) in the children who were responsive. No side effects were observed related to iloprost administration. CONCLUSION: Administration of inhaled iloprost in combination with O2 for pulmonary vasoreactivity testing can be useful for correctly identifying pulmonary vasoreactivity without extending the duration of cardiac catheterization.

Tissue Doppler, strain, and strain rate measurements assessed by two-dimensional speckle-tracking echocardiography in healthy newborns and infants.

OBJECTIVES: To evaluate cardiac maturational and haemodynamic alteration in healthy newborns and infants and determine reference values in this period using tissue Doppler, strain, and strain rate echocardiography. MATERIAL AND METHODS: The study included 149 healthy subjects. Babies from 1 day to 3 months were selected from the well-baby nursery department, and infants were selected from paediatric clinics during routine visits for health maintenance. Subjects were allocated to four groups: preterm (36-37 weeks, n = 32), term (≥ 38 weeks, n = 32), 1 month of age (n = 47), and 3 months of age (n = 38). Standard echocardiographic evaluations, pulsed wave Doppler, tissue Doppler echocardiography, strain, and strain rate studies were applied by the same person using a MyLab50 echo machine. Longitudinal and circumferential systolic strain and strain rate measurements were assessed by two-dimensional speckle-tracking echocardiography in all subjects. RESULTS: The longitudinal systolic velocity, strain, and strain rate values derived from left ventricle apical four-, three-, and two-chamber images, and circumferential systolic velocity, strain, and strain rate values derived from left ventricle short-axis images decreased from the base to the apex in all subjects (p < 0.001). CONCLUSION: Significant cardiac haemodynamic alterations occurred during the newborn and early infancy periods and were detected by tissue Doppler, strain, and strain rate echocardiography. Although two-dimensional speckle-tracking echocardiography is useful and can produce improved, reliable results in clinical practice, it has some limitations. Therefore, more studies on this issue are required.

Subclinical diastolic dysfunction in children with Wilson's disease assessed by tissue Doppler echocardiography: a possible early predictor of cardiac involvement.

BACKGROUND: The aim of this study was to evaluate the left ventricular systolic and diastolic functions and cardiac rhythm problems for the early detection of myocardial dysfunction in children with Wilson's disease. METHODS: We compared patients who had Wilson's disease (n = 22) witl Wilson's disease was based on clinical symptoms and laboratory tests (serum ce cardiographic examination, as well as pulsed-wave Doppler, tissue Doppler ech Holter monitoring was also performed in all subjects. RESULTS: All patients were asymptomatic on cardiological examination an tion, fractional shortening, wall thickness and left ventricular mass were similar disease had significantly lower mitral E velocity, mitral E/A ratio (P= 0.046, P= 0.0 as estimated by pulsed wave Doppler echocardiography. Wilson patients had lo pler echocardiography (P=0.006) compared to the controls. On 24-hour ECG cardiac arrhythmia. CONCLUSION: Our study showed results that might be consistent with disease children which probably represents an early stage of cardiac involvem the patients.

Evaluation by N-terminal prohormone of brain natriuretic peptide concentrations and ross scoring of the efficacy of digoxin in the treatment of heart failure secondary to congenital heart disease with left-to-right shunts.

This study aimed to evaluate the effectiveness of digoxin in children with heart failure secondary to left-to-right shunt lesions and normal left ventricular systolic function. The study registered 37 such patients (ages 10 days to 24 months, groups 1 and 2) and used 20 healthy children as a control group (group 3). Left ventricular systolic function, as assessed by conventional echocardiography, was normal in all the subjects. Congestive heart failure was diagnosed by clinical evaluation and modified Ross scoring. Plasma N-terminal prohormone of brain natriuretic peptide (NT-proBNP) concentrations and complete blood counts were assessed in all the children. Group 1 was treated with digoxin, enalapril, and furosemide and group 2 with enalapril and furosemide. Approximately 1 month after starting treatment, the patients were reevaluated by physical and echocardiographic examinations, modified Ross scoring, plasma NT-proBNP concentrations, and complete blood counts. The pre- and posttreatment Ross scores of group 1 (p = 0.377) and group 2 (p = 0.616) did not differ significantly. The NT-proBNP values in both groups decreased after treatment (p = 0.0001). The pre- and posttreatment NT-proBNP values did not differ significantly in group 1 (p = 0.094)) and group 2 (p = 0.372). The pretreatment NT-proBNP values in groups 1 and 2 (p = 0.0001) were significantly higher than in the control group (p = 0.003). A smaller difference was observed between posttreatment NT-proBNP values in group 1 and the control group (p = 0.045). We found no significant difference between the posttreatment NT-proBNP values of group 2 and those of the control group (p = 0.271). The study showed that both treatments currently used to treat heart failure secondary to congenital heart disease with left-to-right shunts and preserved left ventricular systolic function are effective and do not differ significantly. Thus, digoxin does not provide any extra benefit in the treatment of such patients.

Association of cardiac changes with serum adiponectin and resistin levels in obese and overweight children.

OBJECTIVES: To investigate serum adiponectin and resistin levels in childhood obesity and their relationship with cardiac changes and insulin resistance. METHODS: Seventy-one obese and 24 overweight children and 40 healthy children and adolescents were selected for the study. Height and weight measurements, BMI values and BMI SD score values were obtained for each individual. After blood pressure measurement, left ventricular wall thickness, left ventricular mass, stroke volume, cardiac output, systolic and diastolic functions of the left ventricle were measured using an M-mode, two dimensional color-coded echocardiography device. Blood samples of the individuals were obtained for fasting blood sugar, total blood cholesterol, triglyceride, low-density lipoprotein (LDL) cholesterol, very low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, insulin, C-peptide, adiponectin and resistin values. RESULTS: Cholesterol and LDL values, homeostasis model assessment of insulin resistance, fasting insulin and fasting C-peptide values of the obese and overweight groups were higher (P<0.01). Adiponectin level (P<0.001) and resistin level (P<0.05) of the obese and overweight groups were lower than those of the control group (P<0.05). Echocardiographic evaluation showed diastolic dysfunction in addition to increased left ventricular wall thickness and left ventricle mass values in the obese and overweight children. We also detected a significant positive correlation among left ventricular mass, interventricular septum systolic diameter and resistin in obese children. Among the factors, resistin level was determined as an independent predictor of left ventricular mass in obese children. CONCLUSION: In this study, even in asymptomatic obese and overweight children, cardiac structural and functional changes, such as increased left ventricular mass and diastolic dysfunction, were demonstrated. Although decreased adiponectin level was not related to cardiac changes, it was shown that decreased serum resistin levels in the obese cases lead to left ventricle hypertrophy.

Presence of a D8/17 B lymphocyte marker and HLA-DR subgroups in patients with rheumatic heart disease.

OBJECTIVE: The aim of our study was to investigate the association of HLA antigens and a non-HLA protein D8/17 with rheumatic heart disease and its pattern of cardiac involvement. METHODS: This cross- sectional observational study included 35 children and 12 adult patients who have rheumatic heart disease and 35 healthy children and 12 healthy adult controls. After physical examination, all patients and control group members were evaluated with 2D and color-coded echocardiography. B- lymphocyte D8/17 expression was tested by a flow cytometry assay. HLA genotyping was performed using polymerase chain reaction sequence-specific primers. In statistical analysis, Chi-square, unpaired t and Mann-Whitney U tests were used for comparison groups. RESULTS: The percentage of the D8/17-expressing B lymphocytes of the patient group was significantly higher than of the control group (77.3±15.6% vs. 67.7±20.0%, p=0.013). When compared with the control group, the HLA DRB5 (38.6% vs. 13.6%, p=0.007) and HLA DRB1*15 (31.8% vs. 9.0%, p=0.008) expression levels of the patient group were significantly higher and the DRB4 expression of the patient group was significantly lower (29.5% vs. 50.0%, p=0.049). CONCLUSION: Our findings support the association between HLA Class 2 subgroups and rheumatic heart disease, and an association between D8/17 expression and rheumatic heart disease. Further studies including higher number of patients and control group members should be performed for the confirmation of our results.

Evaluation of the left ventricular function with tissue tracking and tissue Doppler echocardiography in pediatric malignancy survivors after anthracycline therapy.

Although the anthracyclines have gained widespread use in the treatment of childhood hematological malignancies and solid tumors, cardiotoxicity is the major limiting factor in the use of anthracyclines. The aim of this study was to assess the mitral annular displacement by tissue tracking in pediatric malignancy survivors who had been treated with anthracycline groups chemotheraphy and compare with the tissue Doppler and conventional two dimensional measurements and Doppler indices.In this study, 32 pediatric malignancy survivors and 22 healthy children were assessed with 2D, colour-coded echocardiography. Left ventricular ejection fraction, fractional shortening, stroke volume, cardiac output, cardiac index and diastolic functions were measured. All subjects were assessed with tissue Doppler echocardiography, mitral annular displacements, and also with tissue tracking method.We detected that peak velocity of the early rapid filling on tissue Doppler (E') was lower (p < 0.05) and the ratio of early peak velocity of rapid filling on pulse Doppler to tissue Doppler (E/E') values were statistically higher in patient group than control group (p < 0.05). Myocardial performance index values were also higher in patient group than the control group (p < 0.01). It appears that MPI is a useful echocardiograghic method than tissue tracking of mitral annular displacement in patients with pediatric cancer survivors who had subclinical diastolic dysfunction.

Is edema in minimal change disease of childhood really hypovolemic?

OBJECTIVES: In this study, we aimed to find out whether children with minimal change disease can be classified as hypervolemic by objective measures. METHODS: Eighteen children with minimal change disease diagnosed at our department between November 2005 and May 2007 were included in this study. All patients were newly diagnosed or relapsed but were steroid free for at least 6 months. In the first week of edema and when edema resolved (5-7 days after initiation of therapy), weight, height and blood pressure were obtained from all patients. Serum and plasma samples were taken following a starvation period of 12-14 h. The volume load of all patients was evaluated, measuring the inferior vena cava indices in each stage by echocardiography. RESULTS: Average weight at presentation was 8.5% higher than the ideal (dry) weight. There were significant differences between the first and post-treatment body weights, abdomen circumference, and systolic and diastolic blood pressure values (P<0.05 for each). The inferior vena cava index (IVCI) values decreased significantly after diuretic treatment (P<0.001), while inferior vena cava collapsibility index (IVCCI) values increased in the post-treatment period (P<0.001). CONCLUSION: We believe that a close follow-up of hypervolemic children with MCD, treated solely with easy-to-handle diuretics instead of I.V. albumin and diuretics may properly solve the edematous state in these patients.

Prevalence and outcome of congenital heart disease in patients with neural tube defect.

A prospective clinical study was designed to establish the risk factors, the prevalence, and the progress of congenital heart defects in children with neural tube defects. Study included 90 children with a mean age of 13.5 +/- 30.4 months. There were 53 (59%) patients with spina bifida occulta and 37 (41%) patients with spina bifida aperta. The overall prevalence of congenital heart disease was 27.8% (40.5% in spina bifida aperta and 18.9% in spina bifida occulta; P = .024). There was no statistically significant difference for maternal age, usage of periconceptional folate, and maternal diabetes between the patient and control groups. The authors conclude that congenital heart defects are more common than reported in neural tube defects, and screening echocardiograms are warranted. This should be kept in mind especially in patients requiring minor or major surgical procedures. Furthermore, routine obstetric examination and therefore the use of periconceptional folic acid during pregnancy is still lacking in our country.

Brain MR spectroscopy in children with a history of rheumatic fever with a special emphasis on neuropsychiatric complications.

PURPOSE: To investigate whether there are metabolite changes in basal ganglia of children with complete healing of rheumatic fever (RF), history of Syndenham chorea (SC) and obsessive compulsive-tic disorder (OCTD) developed after RF when compared with healthy controls and each other. MATERIAL AND METHODS: A total of 49 children with history of RF and 31 healthy controls were included into the study. All patients and control group underwent a detailed neuropsychiatric evaluation. Children with the history of RF were classified into three groups as; group 1: with history of RF without neuropsychiatric complications (NCRF), group 2: only with history of SC (HSC), group 3: with HSC and OCTD (OCTD). After MR imaging, single voxel MR spectroscopy was performed in all subjects. Voxels (15 x 15 x 15 mm) were placed in basal ganglia. N-acetyl aspartate (NAA)/creatin (Cr), and choline (Cho)/Cr ratios were calculated. RESULTS: OCTD were detected in 13 children with HSC. NAA/Cr ratio was found to be decreased in these children when compared with NCRF (n:29), HSC without OCTD (n:7) and control groups (n:31). No significant difference was found in metabolite ratios of children with HSC without OCTD when compared with NCRF and control groups. There were no significant differences in Cho/Cr ratio between patient and control groups. CONCLUSION: Although MR imaging findings was normal, MR spectroscopy findings (decreased NAA/Cr ratio) in our study support the neuronal loss in basal ganglia of children with OCTD and could indicate the development of permanent damage.