Multisystem inflammatory syndrome in children during severe acute respiratory syndrome coronavirus-2 pandemic in Turkey: A single-centre experience.

AIM: Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection may result in a life-threatening hyperinflammatory condition named multisystem inflammatory syndrome in children (MIS-C). We aimed to assess demographics, clinical presentations, laboratory characteristics and treatment outcomes of patients with MIS-C. METHODS: We performed a retrospective study of patients with MIS-C managed between August 2020 and March 2021 at Dr. Sami Ulus Maternity Child Health and Diseases Training and Research Hospital in Turkey. RESULTS: A total of 45 patients (23 male, 51%) with a median age of 8.7 years (interquartile range: 5.6-11.7 years) were enrolled to study. The SARS-CoV-2 serology was positive in 43 (95%) patients. Organ-system involvement included the dermatologic in 41 (91%), cardiovascular in 39 (87%), hematologic in 36 (80%) and gastrointestinal in 36 (80%) patients. Acute anterior uveitis was diagnosed in nine (20%) patients. Two patients presented with clinical findings of deep neck infection such as fever, neck pain, trismus, swelling and induration on the cervical lymph node. One patient presented with Henoch-Schonlein purpura-like eruption. Coronary artery dilatation was detected in five (11%) patients. For treatment of MIS-C, intravenous immunoglobulin was used in 44 (98%) patients, methylprednisolone in 27 (60%) and anakinra in 9 (20%) patients. The median duration of hospitalisation was nine days. All patients recovered. CONCLUSIONS: Children with MIS-C might have variable clinical presentations. Acute anterior uveitis might be a prominent presentation of MIS-C and require ophthalmological examination. It is essential to make patient-based decisions and apply a stepwise approach for the treatment of this life-threatening disease.

Intrauterine Growth Retardation is a Risk Factor for Anthracycline Toxicity.

BACKGROUND: Anthracycline chemotherapy is used to treat a variety of cancers. However, late cardiac effects of anthracycline chemotherapy, such as subclinical left ventricular dilatation and/or dysfunction, have been observed in more than half of long-term survivors of childhood cancers. A major risk factor for anthracycline cardiotoxicity is intrauterine growth restriction (IUGR). We assessed the significance of IUGR as an important risk factor for late cardiotoxic effects of anthracycline therapy in asymptomatic long-term survivors of childhood cancers. MATERIALS AND METHODS: The study included 61 survivors of childhood cancers. Cardiac functions were prospectively studied using both conventional and non-conventional echocardiographic methods (two-dimensional speckle tracking echocardiography) after completion of the treatment. The patients were divided into two groups based on their birth weights: Group 1 (patients with IUGR) and Group 2 (patients with normal birth weight). RESULTS: Conventional echocardiography revealed a similar and normal range of left ventricle systolic and diastolic functions in both groups. However, global longitudinal and circumferential strain values demonstrated subclinical left ventricular systolic dysfunction in both groups as compared with normal reference strain values. Furthermore, Group 1 patients had significantly lower global longitudinal and circumferential strain and strain rate values than those in Group 2 patients. CONCLUSION: Asymptomatic long-term survivors of childhood cancers with a history of IUGR may have an increased risk of anthracycline cardiotoxicity due to the low content of mitochondrial DNA (mtDNA). IUGR is a risk factor for late anthracycline cardiotoxicity.

Transcatheter radiofrequency pulmonary valve perforation in newborns with pulmonary atresia/intact ventricular septum: Echocardiographic predictors of biventricular circulation.

OBJECTIVE: We report a single-institute experience of neonatal transvenous radiofrequency pulmonary valve perforation for pulmonary atresia/intact ventricular septum, with echocardiographic predictors of biventricular circulation. METHODS: Data were reviewed retrospectively for all neonates who underwent primary transvenous pulmonary valve perforation for pulmonary atresia/intact ventricular septum between January 2008 and November 2018 at our institution. We compared patients who need systemic-to-pulmonary shunt or ductal stenting with patients who did not need. RESULTS: During the study period, 31 patients with pulmonary atresia/intact ventricular septum underwent successful radiofrequency pulmonary valve perforation and balloon dilation of the pulmonary valve. There was no procedure-related mortality. Sixteen patients (52%) needed systemic-to-pulmonary shunt or ductal stenting after initial procedure. Among the survivors (follow-up time of 1 to 11.5 years), 15 patients had a biventricular circulation and 6 patients had 1 and 1/2 ventricular circulation. Two patients are awaiting for Fontan operation. Both the TV/MV annulus ratio (>0.85) and tricuspid valve z-score (>-1) were found to be a good predictor of a biventricular outcome in our cohort. CONCLUSIONS: Percutaneous radiofrequency pulmonary valve perforation and balloon valvotomy is an effective and safe primary treatment strategy for neonates with pulmonary atresia/intact ventricular septum. Ductal stenting or systemic-to-pulmonary shunt may be required in the majority of patients who had smaller right heart components. Preselection of patients according to tricuspid valve z-score and TV/MV annulus ratio allows predicting biventricular circulation.

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.

Relationship between non-cardiac chest pain and internalizing problems in pre-school aged children.

OBJECTIVE: Non-cardiac chest pain is a common and persistent problem for children; yet, typically, there is no clear medical cause. To date, no behavioural and/or psychological factors have been studied to explain chest pain in a pre-school paediatric sample. We hypothesized that pre-school children with medically unexplained chest pain would have higher rates of behavioural problems compared to healthy controls. METHODS: We assessed 41 pre-school children with non-cardiac chest pain and 68 age matched children with benign heart murmurs as the control group using the Child Behaviour Check List-1 1/2-5 to evaluate emotional and behavioural problems. RESULTS: Internalizing problem scores comprising emotionally reactive, anxiety/depression, and somatic complaints were higher in children with non-cardiac chest pain than in the control group. Among the possible factors, the factor that is related to behaviour problem scores, in univariate analysis, was a significant and inverse correlation between maternal education and behaviour problem scores. Also, maternal employment status was associated with behavioural problems. Children with a housewife mother were more susceptible to having such behavioural problems. Based on multiple regression analyses, being in the non-cardiac chest pain group was found to be significantly related to internalizing problems in our total sample. CONCLUSIONS: These results suggest that pre-school children with non-cardiac chest pain may experience increased levels of certain behavioural comorbidities. Systematic behavioural screening could increase the detection of behavioural problems and improve care for this population. Future studies of non-cardiac chest pain in pre-school children should include larger samples and comprehensive diagnostic assessments as well as long-term follow-up evaluations.

What is the Significance of Elevated Troponin I in Children and Adolescents? A Diagnostic Approach.

Troponin is a marker that displays cardiac injury quickly and accurately. In adults, troponin elevation is usually associated with coronary artery disease and requires urgent cardiac catheterization. In healthy children, myocardial injury is rare and may develop due to many different causes. Therefore, troponin elevation in children and adolescents does not usually require emergency cardiac catheterization. The aim of this study is to assess the most common causes of troponin elevation in children and adolescents and to show which diagnostic tests are helpful in assessing pediatric patients with elevated troponin. Patients who had been diagnosed with troponin I elevation (> 0.06 ng/ml) at Dr. Sami Ulus Maternity, Children's Health and Disease Training and Research Hospital between 2007 and 2018 were retrospectively evaluated. Patients undergoing cardiac surgery and those with severe congenital heart disease were excluded from the study. The medical records of the patients were examined and age, gender, diagnostic tests, and diagnosis were evaluated. During the study period, the records of 972 patients were obtained. 213 patients were excluded from the study because of heart surgery, congenital heart disease, and neonatal asphyxia or sepsis. Of the remaining 759 patients, 58% were male, 42% were female, and the median age was 4 years (3 days to 17 years). The most frequent causes are myopericarditis (n: 164), drug intoxications (n: 85), carbon monoxide poisoning (n: 74), perimyocarditis (n: 65), and intensive inhalation ß agonist use in acute asthma and lower respiratory tract infections (n: 70). Patients diagnosed with myocarditis and myopericarditis were admitted with a complaint of chest pain, and the diagnosis was made by history, physical examination, ECG, and echocardiographic findings. Unlike adults, troponin I elevation may be associated with many cardiac and non-cardiac pathologies in children. The most common pathologies in cardiac etiology are myopericarditis and perimyocarditis and can be diagnosed by history, physical examination, ECG, and echocardiography. Cardiac catheterization is not necessary except for rare cardiac pathologies and does not alter the prognosis.

True aneurysmal dilatation of a valved bovine jugular vein conduit after right ventricular outflow tract reconstruction: a rare complication.

Valved bovine jugular vein conduit is considered a suitable choice for paediatric population with congenital heart defect requiring right ventricle to main pulmonary artery connection. However, complications related to the use of this device have been reported, with conduit failure occurring mainly as a consequence of stenosis, conduit thrombosis, and valve regurgitation. We present a case of aneurysmal conduit failure of a valved bovine jugular vein conduit used to reconstruct the right ventricular outflow tract.

Single right coronary artery with right ventricular fistula and congenital absence of left coronary artery: an extremely rare combination.

We report an extremely rare case of a 14-month-old girl who was diagnosed with a single right coronary artery with coronary artery fistula communicating with the right ventricle and congenital absence of left coronary artery. Angiography showed a dilated and tortuous single right coronary artery draining into the right ventricle, absence of left coronary system, and left ventricular coronary circulation supplied via collateral vessels.

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.

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.

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.

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.

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.

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.

Pediatric Electrocardiography Interpretation: Where We Are Now?

OBJECTIVE: Electrocardiography (ECG) is an important diagnostic tool used in the early diagnosis and treatment of arrhythmias and conduction disorders. Pediatricians need to interpret the ECGs correctly and be able to decide the situations that require pediatric cardiology consultation. In this study, we aimed to determine the ECG evaluation competencies of pediatricians. The obtained data will serve as a guide for the development of a training module. MATERIALS AND METHODS: Pediatric residents and specialists were included in the study. A questionnaire was sent to the participants via Google Forms. In this questionnaire, participants were asked to evaluate whether 10 ECG samples were normal or abnormal, detect the abnormality in the ECGs, define the specific diagnosis, and indicate how confident they were in their evaluation. A total of 242 participants, 160 of whom were pediatric residents and 82 were pediatricians, were included in the study. RESULTS: The mean knowledge level of the participants was 17 ± 4.1 out of 30 points. Electrocardiography knowledge score increased with increasing age, increasing experience after graduation from medical school, increasing seniority during residency training, and increasing self-confidence in ECG interpretation. A significant difference was found between the ECG knowledge scores of those who attended an ECG course after graduation and those who did not. The rate of correctly defining the distinction between normal and abnormal was 93.7%. The rate of detecting pathologies in ECGs accurately and correct identification of specific diagnosis was 56.7%. CONCLUSION: The accuracy level of pediatricians in ECG evaluation is low. Electrocardiography trainings are needed to develop ECG assessment skills.

Supraventricular tachycardia after respiratory syncytial virus infection in a newborn.

Respiratory syncytial virus (RSV) is a common cause of lower respiratory tract infections in newborn infants. RSV can cause cardiac arrhythmias. We present a case of a term newborn with supraventricular tachycardia that developed during RSV infection and was successfully treated with amiodarone. RSV infection increases the risk of cardiac arrhythmia due to its course and the agents used in the treatment; therefore, close cardiac monitoring is required.

A pediatric case of anaphylaxis due to octreotide.

Octreotide is an octapeptide that mimics natural somatostatin pharmacologically. It is a potent inhibitor of growth hormone, glucagon and insulin, which is used for treatment of acromegaly, symptomatic treatment of carsinoid tumours, and vasoactive intestinal peptide secreting tumors. It is also used for chylothorax, chemotherapy induced diarrhea and, as it inhibits the exocrine production of pancreatic enzymes, for acute and chronic pancreatitis. Gallbladder stones, diarrhea, nausea, vomiting, hypoglycemia/hyperglycemia, headache, and abdominal discomfort are some of the common adverse effects of octreotide and it may rarely cause anaphylaxis. We present here a child who had chronic pancreatitis and had an anaphylactic reaction to octreotide. To our knowledge this is the first pediatric case of anaphylaxis with octreotide who was successfully desensitized.