Evaluating the requirement of ultrasonography for children with their first urinary tract infection.

BACKGROUND: Management of urinary tract infection (UTI) in children remains important. It may be the first sign for a possible underlying congenital abnormalities for the kidney and urinary tract (CAKUT). This study examined whether performing renal and bladder ultrasonography (RBUS) only for children who have a pathogen other than E. coli during their first urinary tract infection (UTI), or who experience UTI recurrence, would result in more missed diagnoses of kidney anomalies. METHODS: Patients aged between 2 months and 2 years who were seen in a tertiary pediatric hospital during a 2-year period and diagnosed with UTI were included. RBUS and voiding cystourethrography (VCUG) were performed according to American Academy of Pediatrics (AAP) guidelines. Afterwards, we looked back and evaluated how often we found kidney problems when we only did a RBUS on patients who had an atypical cause of their first UTI or who had multiple UTIs. RESULTS: One hundred and seventy-eight patients who were followed up with UTI were included in this study. The isolated pathogen was E. coli in 104 cases (58.4 %) and atypical in 74 cases (41.6 %). VCUG was conducted on 40 patients, and vesicoureteral reflux (VUR) was discovered in 16 cases and ureteropelvic junction obstruction (UPJO) was discovered in 1 case. A different diagnostic approach that required the presence of an atypical pathogen at the first UTI or a fUTI recurrence to perform the RBUS would have missed just two severe kidney anomalies. It was observed that there could be a decrease of 40.4 % in RBUS and at least 20 % in VCUG. CONCLUSIONS: A diagnostic approach that necessitates the presence of an abnormal pathogen during the initial UTI or a second UTI episode for the RBUS to be carried out would lead to fewer negative ultrasounds with minimal risk of overlooking kidney anomalies.

Tricuspid annular plane systolic excursion and mitral annular plane systolic excursion cardiac reference values in 1300 healthy children: Single-center results.

BACKGROUND: Tricuspid annular plane systolic excursion (TAPSE) and mitral annular plane systolic excursion (MAPSE) are two echocardiographic parameters which provide reliable information about the longitudinal myocardial functions of the right and left ventricles in both adult and pediatric population. The aim of this study was to determine the TAPSE and MAPSE reference values in healthy children aged 0-18 years. METHODS AND RESULTS: This prospective study included 1300 healthy children evaluated with two-dimensional echocardiography. In addition to routine echocardiographic examination, the right and left ventricular systolic functions were assessed by TAPSE and MAPSE measurements. Statistical analyses were carried out in the groups of subjects stratified according to age and body surface area. The mean and standard deviation values and z-scores of TAPSE and MAPSE were developed in each group. CONCLUSIONS: The determination of reference values for TAPSE and MAPSE in healthy children will be of guidance in the evaluation of both healthy children and those with congenital or acquired heart diseases in which the right and left ventricular systolic functions are affected. The reference values obtained will contribute to the clinical practice and the future studies.

Anxiety, depression, and behavioural rating scales in children with non-cardiac chest pain.

INTRODUCTION: Chest pain is the second most common reason for referral to paediatric cardiologists after benign heart murmurs. Aetiology frequently depends on non-cardiac reasons. In addition, individuals may experience non-cardiac chest pain which is idiopathic or of unknown origin. The aim of this study is to examine psychological symptoms in children and adolescents with medically unexplained chest pain. METHODS: A total of 76 patients (ages 8-18 years) were included in the study, who were referred to the paediatric cardiology department with the complaint of chest pain but did not have any detected cardiac aetiology or any other organic causes of chest pain. The control group was composed of 51 healthy volunteers. Self-evaluation scales were given to both groups which included Beck Anxiety Inventory and Children's Depression Inventory. Also parents of both groups completed the Conner's Parent Rating Scale for assessment of Attention-deficit/hyperactivity disorder. RESULTS: Anxiety scores of the non-cardiac chest pain group were significantly higher compared to controls. No significant differences were found between patients and controls in terms of attention-deficit/hyperactivity disorder and depression scores. In patient group, patterns were similar for boys and girls and for children and adolescents; except girls scored significantly higher than boys in children's depression inventory. CONCLUSIONS: In children and adolescents, non-cardiac chest pain is associated with increased levels of anxiety. These results show the importance of psychiatric evaluation in non-cardiac chest pain patients. Larger controlled studies are needed to determine the prevalence and impact of attention-deficit/hyperactivity disorder and depression in children and adolescents with non-cardiac chest pain.

Evaluation of long-term cardiac side effects of anthracycline chemotherapy by conventional and non-conventional echocardiographic methods in childhood cancer survivors.

OBJECTIVE: Anthracycline chemotherapeutic agents carry the well-recognised risk of cardiac toxicity. The aim of this study was to determine the long-term effect of anthracycline chemotherapy on the biventricular function in childhood cancer survivors using tissue Doppler imaging and two-dimensional speckle tracking echocardiography. PATIENTS AND METHODS: The study included 45 survivors of childhood cancers and 50 healthy age-matched control patients. Cardiac function was prospectively studied with conventional echocardiography, tissue Doppler imaging, and speckle tracking echocardiography after completion of treatment. The same analysis was performed on matched controls. RESULTS: There was no difference in age, gender, height, and weight between the study and control groups. The mean anthracycline dose was 240 ± 106 mg/m2 and the mean remission duration was 8.2 ± 5 years (1-20 years) in the study group. Conventional echocardiography showed similar ejection fraction, shortening fraction, and left ventricle end-diastolic diameter in both groups. Mitral lateral and septal tissue Doppler imaging showed normal but according to control group relatively sub-normal systolic and diastolic function in patient group. The global longitudinal and circumferential strain and strain rates were significantly lower in the patient group compared to control group. Correlation analysis revealed a negative and significant correlation between total anthracycline dose and global longitudinal and circumferential strain and strain rates. CONCLUSION: Sub-clinical systolic and diastolic dysfunction may not be detected by conventional echocardiographic methods which are frequently used in daily practice. Sub-clinical systolic and diastolic dysfunction may be detected more sensitively by echocardiographic method such as speckle tracking echocardiography in childhood cancer survivors.

Should we start a nationwide screening program for critical congenital heart disease in Turkey? A pilot study on four centres with different altitudes.

BACKGROUND: To investigate the feasibility of critical congenital heart disease (CCHD) screening test by pulse oximetry in four geographical regions of Turkey with different altitudes, before implementation of a nationwide screening program. METHODS: It was a prospective multi-centre study performed in four centres, between December, 2015 and May, 2017. Pre- and post-ductal oxygen saturations and perfusion indices (PI) were measured using Masimo Radical-7 at early postnatal days. The results were evaluated according to the algorithm recommended by the American Academy of Pediatrics. Additionally, a PI value <0.7 was accepted to be significant. RESULTS: In 4888 newborns, the mean screening time was 31.5 ± 12.1 hours. At first attempt, the mean values of pre- and post-ductal measurements were: saturation 97.3 ± 1.8%, PI 2.8 ± 2.0, versus saturation 97.7 ± 1.8%, PI 2.3±1.3, respectively. Pre-ductal saturations and PI and post-ductal saturations were the lowest in Centre 4 with the highest altitude. Overall test positivity rate was 0.85% (n = 42). CCHD was detected in six babies (0.12%). Of them, right hand (91 ± 6.3) and foot saturations (92.1 ± 4.3%) were lower compared to ones with non-CCHD and normal variants (p <0.05, for all comparisons). Sensitivity, specificity, positive and negative predictive values, and likelihood ratio of the test were: 83.3%, 99.9%, 11.9%, 99.9%, and 99.2%, respectively. CONCLUSION: This study concluded that pulse oximetry screening is an effective screening tool for congenital heart disease in newborns at different altitudes. We support the implementation of a national screening program with consideration of altitude differences for our country.

[Idiopathic dilated cardiomyopathy in children: Prognostic indicators]. / Çocuklarda idiyopatik dilate kardiyomiyopati: Prognostik belirteçler.

OBJECTIVE: Dilated cardiomyopathy (DCM) is a disorder featuring left ventricular dysfunction, heart failure, and a poor prognosis. The etiology is still unclear, despite diagnostic and therapeutic developments. This study was an evaluation of factors affecting the life span of a group of idiopathic DCM patients. METHODS: A total of 79 patients from between October 2005 and October 2017 with a diagnosis of idiopathic DCM were evaluated retrospectively. Demographic characteristics, clinical information, left ventricular function, treatment, and follow-up of the patients were reviewed based on hospital records. Age, gender, parental consanguinity, cardiomegaly on telecardiography, reduced ejection fraction (EF) and shortening fraction (SF), degree of mitral regurgitation, and intracardiac thrombosis were determined to affect prognosis. RESULTS: The patients were aged 20+-60 months, and the male/female ratio was 1.02/1. The patients most frequently presented with heart failure signs and symptoms (n=59, 74.7%). The most common physical examination findings were a murmur (n=53, 67.1%) and tachycardia (n=48, 60.8%). Cardiomegaly was observed on telecardiography in 73.4% of the patients. The EF and SF values were 35.7+-12.6% and 17.3+-6.5%, respectively. In all, 42 (53.2%) patients had mitral regurgitation of grade 2 or higher. The duration of follow-up was between 1 and 156 months (20+-34.9 months). Intracardiac thrombosis was detected in 4 (5.1%) patients. The mortality rate was 36.7%. When the prognostic factors were compared according to survival time, it was determined that survival was reduced in cases of parental consanguinity, low EF, and cardiomegaly. CONCLUSION: The most important negative markers affecting the length of survival of DCM patients were parental consanguinity, cardiomegaly detected on telecardiography, and a reduced EF level.

A pediatric case of Ortner`s syndrome caused by heritable pulmonary arterial hypertension and review of the literature.

Kaya Ö, Yoldas T, Karademir S, Örün UA, Sari E. A pediatric case of Ortner`s syndrome caused by heritable pulmonary arterial hypertension and review of the literature. Turk J Pediatr 2019; 61: 963-966. An 11-year-old male, who complained of hoarseness and fatigue on effort presented to our institution for evaluation. Left vocal cord paralysis and severe pulmonary hypertension was diagnosed. The patient had an enlarged pulmonary artery due to pulmonary hypertension which was responsible for compression to left vocal cord paralysis causing hoarseness. Ortner`s syndrome or cardiovocal syndrome is known as hoarseness due to left vocal cord paralysis secondary to cardiac pathologies. Although hoarseness of voice is frequently encountered in the otorhinolaryngology clinics, pulmonary hypertension related hoarseness is an unusual presentation in childhood.

Sensitive Cardiac Troponins: Could They Be New Biomarkers in Pediatric Pulmonary Hypertension Due to Congenital Heart Disease?

To analyze the role of sensitive cardiac troponin I (scTnI) and high-sensitive troponin T (hscTnT) in the determination of myocardial injury caused by volume and pressure load due to pulmonary hypertension (PH) and to investigate if these markers may be useful in the management of PH in childhood. Twenty-eight patients with congenital heart disease (CHD) with left to right shunt and PH, 29 patients with CHD with left to right shunt but without PH, and 18 healthy children, in total 75 individuals, were included in the study. All cases were aged between 4 and 36 months. Echocardiographic evaluation was performed in all cases, and invasive hemodynamic investigation was performed in 33 cases. Blood samples were obtained from all cases, for the measurement of brain natriuretic peptide (BNP), pro-brain natriuretic peptide (pro-BNP), sensitive cardiac troponin I (scTnI), and high-sensitive troponin T (hscTnT) levels. The mean BNP, pro-BNP, scTnI, and hsTnT levels were statistically significantly higher in patients with PH than in the patients without PH (p < 0.001). A statistically significant positive correlation was determined between pulmonary artery systolic pressure and scTnI and hscTnT levels (r = 0.34 p = 0.01, r = 0.46 p < 0.001, respectively) levels. Pulmonary hypertension determined in congenital heart diseases triggers myocardial damage independently of increased volume or pressure load and resistance, occurring by disrupting the perfusion via increasing ventricular wall tension and the myocardial oxygen requirement. Serum scTnI and hscTnT levels may be helpful markers to determine the damage associated with PH in childhood.

Maternal and neonatal micronutrient levels in newborns with CHD.

OBJECTIVE: It is suggested that folic acid and/or multivitamins, taken periconceptionally, have a role in the prevention of many congenital anomalies. The aim of this study was to determine the serum micronutrient levels in mother-infant pairs with CHD compared with those with healthy newborns and their mothers. METHODS: Serum levels of folic acid, homocysteine, zinc, vitamin A, vitamin D, and vitamin B12 were measured from 108 newborns with CHD (study group) and 103 healthy newborns (control group). The mothers' micronutrient levels were also measured simultaneously. RESULTS: When compared with healthy newborns, for both maternal and neonatal data, homocysteine and zinc levels were higher and vitamin D levels were lower in the study group. In multivariate analysis, only maternal high zinc levels were associated with CHD in the newborns (p=0.02, OR: 0.9, 95% CI 0.8-0.9). The results did not change when analysed for truncal anomalies including truncus arteriosus, tetralogy of Fallot, and d-transposition of great arteries. There were positive correlations between maternal and neonatal levels of micronutrients, except vitamin B12. CONCLUSION: We thought that high homocysteine and zinc levels and low vitamin D levels in mother-infant pairs might have a role in the aetiopathogenesis of CHD. Large-scale, prospective studies are needed to clarify the role of micronutrients in CHDs.

Subclinical left ventricular systolic and diastolic dysfunction in type 1 diabetic children and adolescents with good metabolic control.

OBJECTIVE: Cardiac dysfunction is a well-known consequence of diabetes mellitus. This study was designed to assess whether type 1 diabetic children and adolescents with good metabolic control have early echocardiographic signs of subclinical left ventricular dysfunction and whether diabetes duration has any influence, using conventional and nonconventional echocardiographic tools. METHODS: A total of 100 patients with type 1 diabetes mellitus and 80 gender- and age-matched healthy controls were included. The cases underwent standard conventional transthoracic echocardiography, tissue Doppler imaging, and two-dimensional speckle tracking echocardiography. None of the diabetic patients had signs of renal, retinal, or neurological complications of the disease, and all were good metabolic control (mean HbA1c <7.5%). RESULTS: There was no difference among groups in relation to age, sex, body mass index, and blood pressure. Conventional echocardiographic parameters were similar between diabetic and nondiabetic subjects except increased mitral valve peak A-wave and significantly lower mitral E/A ratio in diabetics. Diabetic patients had more advanced diastolic dysfunction with TDI analysis. In the diabetic group, left ventricular global longitudinal, circumferential, and radial strain and strain rate were significantly lower compared with the controls. There was a positive correlation between diabetes duration and cardiac dysfunction. CONCLUSION: The results of this study showed that the diabetic children and adolescents with good metabolic control had diastolic dysfunction when assessed with either conventional or tissue Doppler echocardiography. Also diabetic patients had subclinical LV systolic dysfunction with a normal LVEF which can be detected with 2D speckle tracking echocardiography.

Massive Pericardial Effusion and Tamponade Can Be a First Sign of Familial Mediterranean Fever.

Cardiac involvement is a rare initial presentation of familial Mediterranean fever (FMF). We described 2 children with massive pericardial effusion and cardiac tamponade, who were later diagnosed as having FMF based on clinical and laboratory findings. Therefore, in children presenting with massive pericardial effusion, FMF should be considered as one of the differential diagnoses. In addition, massive pericardial effusion is a serious clinical condition that requires emergency therapeutic approach including pericardiocenthesis. For medical treatment, colchicine is the first line therapy, but in resistant cases, other anti-inflammatory drugs can be used for extra anti-inflammatory effect.

Retinovascular findings in newborns with critical congenital heart disease: A case series. / Signos vasculares retinianos en recién nacidos con cardiopatía congénita crítica: serie de casos.

Critical congenital heart disease (CCHD) may cause changes in retinal vasculature. However, there is lack of data in this issue in newborns. We evaluated retinovascular changes in a series of 43 newborn with CCHD. They were divided into 2 groups according to the type of CCHD; group 1 (n= 18): left obstructive CCHD and group 2 (n= 25): right obstructive CCHD. Retinovascular pathologies were detected in 21 patients (48.8%); it was more frequent in group 1 (p= 0.04). The most common pathology was retinal vascular tortuosity in 6 patients (33.3%) of group 1, and 4 (16.0%) in group 2. None of 21 patients with retinovascular changes required any therapy at follow-up. In multivariate analysis, only having a left obstructive CCHD was associated with the development of any retinovascular pathology (P= 0.03, OR: 2.8, CI95%: 1.1-7.4). Retinovascular changes are frequent in newborn patients with CCHD.

La cardiopatia congènita crítica (CCC) podría provocar cambios en la vasculatura de la retina. Sin embargo, no se dispone de suficientes datos sobre este problema en los recién nacidos. Se evaluaron los cambios en la vasculatura retiniana en una serie de 43 recién nacidos con CCC. Se los dividió en dos grupos según el tipo de CCC; grupo 1 (n= 18): CCC obstructiva izquierda y grupo 2 (n= 25): CCC obstructiva derecha. Se detectaron enfermedades vasculares retinianas en 21 pacientes (48,8%); estas fueron más frecuentes en el grupo 1 (p= 0,04). La patología más común fue la tortuosidad vascular retiniana, observada en seis pacientes (33,3%) del grupo 1 y en 4 (16,0%) del grupo 2. Ninguno de los 21 pacientes con cambios en la vasculatura de la retina requirió tratamiento durante el seguimiento. En un análisis multivariado, solo la CCC obstructiva izquierda estuvo asociada con el desarrollo de enfermedad vascular retiniana (P= 0,03, razón de probabilidades [OR]: 2,8, intervalo de confianza [IC] del 95%: 1,1-7,4). Los cambios vasculares retinianos son frecuentes en los recién nacidos con CCC.

Spontaneous echocardiographic contrast due to ductus venosus.

Spontaneous echocardiographic contrast is defined as a phenomenon of discrete reflections appearing in the cardiac chambers or great vessels. It has been associated with several conditions leading to microbubble formation or low blood flow. This is a report of a neonate with spontaneous echocardiographic contrast related to patent ductus venosus.

Cardiac MRI and 3D contrast-enhanced MR angiography in pediatric and young adult patients with Turner syndrome.

BACKGROUND/AIM: This study aimed to describe the spectrum and frequency of cardiovascular abnormalities in pediatric and young adult patients with Turner syndrome (TS) using cardiac MRI and MR angiography. MATERIALS AND METHODS: This prospective study consisted of 47 female patients of pediatric age and young adults with a karyotypically confirmed diagnosis of TS. All patients underwent cardiac MRI and contrast-enhanced MR angiography. A second examination after 9-26 months was performed for 28 of these patients. RESULTS: Elongation of the transverse aortic arch (ETA) was the most frequent abnormality with a rate of 37%. The rate of partial anomalous pulmonary venous connection (PAPVC) was 21.7%, bicuspid aortic valve (BAV) was 19.6%, coarctation was 6.5%, ascending aorta dilatation was 28.3%, and descending aorta dilatation was 15.2%. The diameters of the aorta and the rate of aortic dilatation per unit of time was greater in the patients with BAV (P < 0.05). ETA was less observed in the patients who were receiving growth hormone therapy (P < 0.05). CONCLUSION: The most common cardiovascular abnormalities in TS patients are aortic arch anomalies such as ETA and coarctation, aortic dilatation, PAPVCs, and BAV. The presence of BAV is an important risk factor for the aortic dilatation.

Symptomatic Fetal-Type Cardiac Rhabdomyoma.

Rhabdomyomas are the most common primary cardiac tumors, especially seen during early periods of childhood. Fetaltype rhabdomyoma is a benign tumor described almost always in extracardiac locations. Although the natural history of the cardiac rhabdomyoma is to regress, the behaviour of the fetal-type rhabdomyomas when present in the heart is unknown with respect to its infrequency. Herein, we report a hemodynamically unstable female neonate with a single large intra-cardiac mass unresponsive to medical treatment, who underwent surgery. The neonate could not survive the operation due to ventricular fibrillation. The mass was diagnosed as fetal-type cardiac rhabdomyoma on autopsy.

Aneurysm of the muscular septum associated with Wolf-Parkinson-White syndrome presenting as dilated cardiomyopathy; A report of two cases.

Muscular septal aneurysms are extremely rare without a ventricular septal defect and are diagnosed accidentally in most cases. Reported cases generally have rhythm disturbance or electrocardiographic findings of Wolf-Parkinson-White (WPW) syndrome. Presently described are 2 cases of ventricular septal aneurysm associated with WPW syndrome, which presented as dilated cardiomyopathy. Pre-excitation disappeared gradually in first patient. There was also concurrent decrease in degree of bulging of the interventricular septum and improvement of left ventricular systolic function. Second patient had complaints of palpitation and was referred for ablation of accessory pathway. Our findings suggest that presence of pre-excitation may lead to ventricular dyssynchrony and abnormal ventricular septal movement, resulting in appearance of aneurysm.

Persistent left superior vena cava accompanying congenital heart disease in children: Experience of a tertiary care center.

BACKGROUND: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). METHODS: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed. RESULTS: The median age of the patients was 9.5 months, and 46 of the 88 (52.3%) patients were female. The most common concomitant CHD were ventricular septal defect, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF). When the patients were compared according to their CHD, persistent left SVC frequency was significantly higher in those with DORV (P<.001), TOF (P=.04), patent ductus arteriosus (P=.01), and atrial septal defect (P=.03). Eighty-three of the 88 (94.3%) patients with persistent left SVC had right SVC, and 5 (5.7%) had absence of the right SVC. Twenty-seven of the 83 (32.5%) patients with double SVC had connected normal innominate vein. In all cases, right aortic arch association was seen in 14 (15.9%) patients. Eighty-four (95.4%) of the patients were diagnosed by echocardiography prior to catheter angiography. Persistent left SVC drained to the coronary sinus in all cases. CONCLUSION: Increased awareness about the association of certain CHD with persistent left SVC and a careful echocardiographic examination can facilitate the diagnosis of persistent left SVC. In addition, precise prior diagnosis of persistent left SVC can prevent complications during surgery.

A case of tracheal bronchus associated with right aortic arch and partial anomalous pulmonary venous connection.

Tracheal bronchus includes a variety of bronchial anomalies arising in the trachea or main bronchus and directed toward the upper-lobe territory. Reported incidence varies from 1-3% in the pediatric population. It is generally associated with other congenital malformations, including costovertebral anomalies, congenital airway and lung anomalies, vascular anomalies, and congenital heart defects. Presently described was the case of a 14-year-old female with tracheal right-upper-lobe bronchus, right aortic arch with mirror image, and abnormal left upper pulmonary venous return to innominate vein.