An unusual etiology in an 8-year-old girl with chest pain and elevated troponin-I.

Acute post-streptococcal glomerulonephritis (APSGN) is the most common glomerulonephritis of childhood, and clinical presentation can vary widely. This case report presents an atypical manifestation of APSGN in an 8-year-old female patient with pleuritic chest pain and elevated troponin-I, despite lacking classical kidney symptoms. Imaging studies showed cardiomegaly and interstitial lung opacities. Further investigations revealed hematuria and proteinuria, and the diagnosis was confirmed through elevated antistreptolysin-O (ASO) titers and low complement 3 (C3) levels. The patient was successfully managed with fluid restriction, diuretics, and antihypertensives, resulting in the resolution of symptoms and normalization of laboratory values. This case highlights the significance of recognizing atypical manifestations of APSGN for ensuring prompt diagnosis and proper management in the pediatric population.

Does Iron Deficiency Affect the Heart in Children?

INTRODUCTION: It is well known that serum iron parameters are closely related to cardiovascular health. Iron deficiency may lead to significant cardiac problems, such as atrioventricular conduction abnormalities, and systolic and/or diastolic dysfunction. Studies related to the effect of iron deficiency on cardiac functions are limited in children. Therefore, the aim of this study was to evaluate the effect of low iron stores on cardiac repolarization parameters which are used to predict arrhythmia and cardiac functions. MATERIALS AND METHODS: Onehundred thirty-five children 2 to 18 years of age without structural heart disease who were referred to the Pediatric Cardiology Department for various reasons such as chest pain, heart murmur, or evaluation before sports participation were evaluated prospectively. The cases were separated into 3 groups according to ferritin levels (group 1: ferritin <15 ng/mL [n=48], group 2: ferritin 15 to 25 ng/mL [n=51], and group 3: ferritin >25 ng/mL [n=36]). Evaluations were made with detailed 12-lead surface electrocardiography (ECG) and transthoracic echocardiogram. Cardiac repolarization parameters were measured from surface ECG and systolic functions, left ventricular wall diameters were evaluated from transthoracic echocardiogram. RESULTS: Eighty-four (62.2%) girls and 51 (37.8%) boys are included in the study. The average age of the groups was similar. In children with low ferritin levels (group 1); Pw, Pw max, and Pw dis; QT, QTc, and QTc dis; Tp-Te, Tp-Te dis, Tp-Te/QT, and Tp-Te/QTc were significantly longer compared with the group 3 (P<0.05). There was a negative correlation between ferritin level and Pw, P max, and Pw dis; QT, QT dis, QTc, and QTc dis; Tp-Te, Tp-Te dis, Tp-Te/QT, and Tp-Te/QTc (P<0.05). No correlation was found between ferritin level and Pw min. There was no difference between the groups in respect of ejection phase indices, heart wall measurements, and end-diastolic diameter measurements. CONCLUSION: Iron deficiency may be related to the tendency of arrhythmias in children without structural heart disease. Therefore, careful evaluation of ECG parameters of healthy children with low iron stores, and follow up with intermittent monitoring is highly important.

Assessment of Autonomic Nervous System in Children with Celiac Disease: A Heart Rate Variability Study.

OBJECTIVE: We evaluated the activity of autonomic nervous system in children with celiac disease by using heart rate variability (HRV) analysis. METHODS: HRV parameters of 37 children with celiac disease were compared to 36 age- and sex-matched healthy controls. None of the participants had a systemic, central or peripheral neurological disease. RESULTS: Statistically significant differences were present in two parameters; standard deviation of all RR intervals (SDNN) and standard deviation of 5-minute RR interval means (SDANN). Age was negatively correlated with mean, minimum and maximum heart rate. Duration of disease was positively correlated with low frequency power-high frequency power ratio. No correlation was found between anti-tissue transglutaminase IgA level and HRV parameters. CONCLUSIONS: Celiac disease may affect autonomic nervous function in children even if there are no symptoms of dysautonomia.

Echocardiographic assessment of children participating in regular sports training.

OBJECTIVE: The aim of the present study was to determine the effects of a well-controlled endurance training program on cardiac functions and structures in healthy children and to define whether training hours per week and type of sports affect the training-induced cardiovascular response. METHODS: Echocardiographic recordings were obtained in 126 children who systematically participated in sports training for at least 1 year (study group), and the results were compared with the values obtained in 62 normal children who did not actively engage in any sports activity (control group). The two groups were comparable for age, sex, and body mass index. Study group participants were divided into two groups according to the duration of physical activity (training hours per week, <8 h and >8 h) and five groups according to the cardiovascular demand of sports type. Clinical examination, resting electrocardiogram, two-dimensional, M-mode, and Doppler echocardiography were obtained in all participants. RESULTS: Left ventricle wall dimensions, left atrial diameters, and aortic measurements were significantly higher in the study group. The mean mitral E/A ratio was also significantly higher in the training group than in untrained subjects (p<0.001). Echocardiographic measurements were similar between different sports type participants in the study group. However, aortic root diameter, left atrial diameter, and left ventricle posterior wall diastolic thickness were higher in children training >8 h/week than in children training <8 h/week in the study group. CONCLUSION: The present study showed that the echocardiographic parameters of children participating in regular sports training activities statistically significantly exceeded the parameters of untrained controls. These parameters were mostly dependent on the duration of training hours per week.

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.

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.

Subclinical rheumatic heart disease: A single center experience.

OBJECTIVE: Rheumatic heart disease (RHD) is still a major cause of morbidity and mortality in developing countries. The aim of the present study was to investigate asymptomatic RHD cases diagnosed by echocardiography without any acute rheumatic fever (ARF) history and to present the follow-up results. METHODS: Children who had been admitted to the pediatric cardiology department between 2011 and 2017 for various reasons (e.g., sport participation and palpitation) and diagnosed with RHD by echocardiography without a history of ARF were included the study. Echocardiographic findings of the patients were evaluated retrospectively. RESULTS: A total of 75 (55 girls and 20 boys) patients were included in the study. The median age of the cases was 13.6 (minimum 5 and maximum 18) years. The median follow-up period was 19.2 months, whereas the longest follow-up period was 66 months. At the time of admission, pathological valvular insufficiency was present only in the mitral valve in 69 (89.3%) cases, only in the aortic valve in 2 (2.7%) cases, and in both aortic and mitral valve in 6 (8%) cases. Of the cases, 40 (60%) were diagnosed as borderline RHD at the time of admission, and 30 (40%) as definite RHD according to the World Heart Federation criteria. Of these cases, 88% remained the same as borderline RHD, and the findings of two patients improved from definite to borderline RHD. RHD of four patients deteriorated from borderline to definite RHD, and in two patients, valvular insufficiency completely resolved during the follow-up period. None of the cases needed valvular replacement. CONCLUSION: RHD is still a serious health problem in Turkey. The sensitivity of echocardiography in detecting subclinical mild or asymptomatic cases is well known. For this reason, although it is not yet applied as a routine study, it is important to start the nationwide echocardiographic screening program.

The effects of cigarette smoking on ventricular repolarization in adolescents.

OBJECTIVE: To assess the association between cigarette smoking and ventricular arrhythmias in adolescents. Novel electrocardiographic parameters -Tp-e interval, as well as Tpe/QT and Tpe/QTc ratios - were used to make this assessment. METHODS: The study population consisted of 87 subjects aged between 16-19 years. Fifty-one adolescent smokers with no risk of arrhythmia comprised the Smoker Group, and 36 adolescents who had never smoked cigarettes comprised the Control Group. Smokers were defined as patients smoking more than three cigarettes per day, for at least 1 year. Body mass index, systolic, diastolic and mean blood pressures were measured, and electrocardiograms were performed on all subjects. Heart rate, PR and Tp-e intervals, and Tpe/QT, Tpe/QTc ratio were digitally measured. RESULTS: Adolescents in Smoker Group had smoked cigarettes for 2.9±1.4 years (range 1 to 6 years). The mean age at starting smoking was 13.8±1.4 years. There were no differences between smokers and Control Group as to baseline clinical variables (p>0.05). The PR, QT and QTc intervals were similar in all groups. Tp-e interval (98.4±12.7ms and 78.3±6.9 ms; p<0.001), Tpe/QT (0.28±0.04 and 22±0.03; p<0.01), Tpe/QTc (0.24±0.03 and 0.19±0.01; p<0.001) ratios were significantly higher in Smoker Group. There were no correlations between years of smoking, number of cigarettes per day, Tpe interval, Tpe/QT or Tpe/QTc ratios. CONCLUSION: Cigarette smoking is associated with risk of ventricular arrhytmogenesis with prolonged Tp-e interval and increased Tpe/QT and Tpe/QTc ratios in adolescents.

The effects of cigarette smoking on ventricular repolarization in adolescents / O tabagismo altera a repolarização ventricular em adolescentes

ABSTRACT Objective To assess the association between cigarette smoking and ventricular arrhythmias in adolescents. Novel electrocardiographic parameters -Tp-e interval, as well as Tpe/QT and Tpe/QTc ratios - were used to make this assessment. Methods The study population consisted of 87 subjects aged between 16-19 years. Fifty-one adolescent smokers with no risk of arrhythmia comprised the Smoker Group, and 36 adolescents who had never smoked cigarettes comprised the Control Group. Smokers were defined as patients smoking more than three cigarettes per day, for at least 1 year. Body mass index, systolic, diastolic and mean blood pressures were measured, and electrocardiograms were performed on all subjects. Heart rate, PR and Tp-e intervals, and Tpe/QT, Tpe/QTc ratio were digitally measured. Results Adolescents in Smoker Group had smoked cigarettes for 2.9±1.4 years (range 1 to 6 years). The mean age at starting smoking was 13.8±1.4 years. There were no differences between smokers and Control Group as to baseline clinical variables (p>0.05). The PR, QT and QTc intervals were similar in all groups. Tp-e interval (98.4±12.7ms and 78.3±6.9 ms; p<0.001), Tpe/QT (0.28±0.04 and 22±0.03; p<0.01), Tpe/QTc (0.24±0.03 and 0.19±0.01; p<0.001) ratios were significantly higher in Smoker Group. There were no correlations between years of smoking, number of cigarettes per day, Tpe interval, Tpe/QT or Tpe/QTc ratios. Conclusion Cigarette smoking is associated with risk of ventricular arrhytmogenesis with prolonged Tp-e interval and increased Tpe/QT and Tpe/QTc ratios in adolescents.

RESUMO Objetivo Avaliar a associação entre tabagismo e arritmias ventriculares em adolescentes usando novos parâmetros eletrocardiográficos: intervalo Tp-e, e relações Tpe/QT e Tpe/QTc. Métodos A população do estudo incluiu 87 indivíduos de 16 a 19 anos de idade. Cinquenta e um adolescentes fumantes, sem risco de arritmia, formaram o Grupo Fumantes, e 36 adolescentes, que nunca tinham fumado cigarros, formaram o Grupo Controle. Os fumantes foram definidos como pacientes que fumavam mais de três cigarros por dia, há pelo menos 1 ano. O índice de massa corporal, e a pressão arterial sistólica, diastólica e média foram medidos, e foram realizados eletrocardiogramas em todos os participantes. Frequência cardíaca, intervalos PR e Tp-e, e as relações Tpe/QT e Tpe/QTc foram medidas por instrumentos digitais. Resultados Os adolescentes do Grupo Fumante fumavam há 2,9±1,4 anos (variação 1 a 6 anos). A média de idade ao começar a fumar foi 13,8±1,4 anos. Não houve diferença nas variáveis clínicas iniciais entre os Grupos Fumante e controle (p>0,05). Os intervalos PR, QT e QTc foram semelhantes em todos os grupos. O intervalo Tp-e (98,4±12,7ms e 78,3±6,9ms; p<0,001), e as relações Tpe/QT (0,28±0,04 e 22±0,03; p<0,01) e Tpe/QTc (0,24±0,03 e 0,19±0,01; p<0,001) foram significativamente maiores no Grupo Fumantes. Não houve correlação entre anos de tabagismo, número de cigarros por dia, intervalo Tpe e relações Tpe/QT e Tpe/QTc. Conclusão O hábito de fumar está associado ao risco de arritmogênese ventricular, com interval Tp-e prolongado e aumento nas relações Tpe/QT e Tpe/QTc em adolescentes.

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.

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.

Regression of symptomatic multiple cardiac rhabdomyomas associated with tuberous sclerosis complex in a newborn receiving everolimus.

UNLABELLED: Cardiac rhabdomyoma is the most common primary cardiac tumor, is considered to be a hamartoma of developing cardiac myocytes. Cardiac rhabdomyoma is associated with tuberous sclerosis complex (TSC) in 50-86% of cases. Mutations in TSC-1/TSC-2 genes result in increased mammalian target of rapamycin (mTOR) pathway activation responsible for the hamartomatous lesions of tuberous sclerosis complex. Therapy with mTOR inhibitors is currently under investigation as a treatment option for tumors associated with TSC. In this report we present a case with multiple symptomatic rhabdomyomas associated with tuberous sclerosis complex, deemed to be ineligible for surgical removal, treated with everolimus (mTOR inhibitor). CONCLUSION: As we observed in our patient, in cases with inoperable symptomatic rhabdomyomas associated with TSC, everolimus, an mTOR inhibitor, may be the treatment of choice, which should be confirmed with additional studies.