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Objectives: Behçet's disease (BD) is a systemic vasculitis affecting all sizes of arteries and veins. Approximately 5-10% of patients with BD are present during childhood. The chronic nature of the disease may lead to serious cardiovascular complications over time including early atherosclerosis. Increased levels of epicardial adipose tissue (EAT) and carotid intima-media thickness (CIMT) are considered early signs of subclinical atherosclerosis. Ongoing chronic inflammation may cause to increase in both EAT and CIMT. In this study, we aimed to evaluate CIMT and EAT in children with BD and determine their relationship with the clinical manifestations and course of the disease., Methods: This cross-sectional study evaluated 30 patients with juvenile-onset BD and age-sex-matched 20 healthy controls. The CIMT and EAT thickness were measured by the same paediatric cardiologist. The association between clinical features, baseline disease activity, disease duration, EAT thickness and CIMT was also evaluated., Results: Thirty children with BD and 20 age-sex-matched healthy volunteers enrolled in the study. The most common BD-related feature was oral aphthous (n=30), followed by mucocutaneous findings (n=22). Uveitis was observed in 5 patients, vascular involvement in 4, neurological involvement in 4, and gastrointestinal involvement in 2. All patients were inactive at the time of evaluation. The EAT thickness was significantly higher in patients while CIMT levels revealed no significant differences. However, there was no correlation between disease duration, baseline disease activity, and EAT thickness., Conclusions: Increased EAT thickness may be a risk factor for early atherosclerosis in patients with BD. The EAT thickness was found to be significantly higher in paediatric BD patients. Confirmation of results in larger series may provide better insight into early screening for risk factors in these patients.

Background: The present study aims to evaluate possible cardiac involvement in juvenile dermatomyositis (JDM) patients by conventional methods and cardiac magnetic resonance imaging (MRI) along with a systematic review of the literature on cardiac features in JDM., Methods: The study group consisted of JDM patients who underwent cardiac MRI. We conducted a systematic review of the published literature involving JDM patients with cardiac involvement., Results: In the present study, although baseline cardiologic evaluations including electrocardiography and echocardiography were within normal limits, we showed late gadolinium enhancement on cardiac MRI in 3 of 11 JDM patients. In the literature review, we identified 25 articles related to cardiac involvement in JDM. However, none of them, except one case report, included cardiac MRI of JDM patients., Conclusion: Cardiac abnormalities have been reported among the less frequent findings in patients with JDM. Cardiovascular complications during the long-term disease course are a leading cause of morbidity and mortality in these patients. Early detection of cardiac involvement by cardiac MRI in patients with JDM and aggressive treatment of them may improve the clinical course of these patients., Impact: The myocardium in patients with JDM may be involved by inflammation. Myocardial involvement may be evaluated by using contrast-enhanced cardiac MRI. This is the first study evaluating cardiac involvement by cardiac MRI in JDM patients. MRI may show early cardiac involvement in patients whose baseline cardiologic evaluations are within normal limits. Early detection of cardiac involvement by cardiac MRI may improve the long-term prognosis of patients with JDM., (© 2024. The Author(s).)

Background: Atherosclerotic changes can be attributed to early endothelial damage in individuals with hypertension. We aimed to explore the relationship between endothelial dysfunction and hypertension in newly diagnosed children without end-organ damage, considering carotid intima-media thickness (CIMT), flow-mediated dilatation (FMD), and functional capillaroscopy parameters. We also analyzed the differences between dipper and non-dipper patients., Methods: In this cross-sectional study, 20 patients diagnosed with essential hypertension with no target organ damage, and 20 age and sex-matched healthy volunteers were enrolled. The patient group comprised newly diagnosed individuals not receiving antihypertensive treatment. Hypertensive patients were divided into two groups (dipper and non-dipper patients). The measurements of CIMT, brachial FMD, and functional capillaroscopy were performed before starting treatment., Results: Among the patients, 11 were boys, and 9 were girls, with a median age of 16.0 (2.13) years. Of 20 hypertensive patients, 10 were dipper and 10 were non-dipper. Significant differences were observed between the hypertensive patients and controls in terms of CIMT (p = 0.04), brachial artery FMD (p = 0.02), and functional capillary density (p < 0.001). Hypertensive patients exhibited increased CIMT, reduced brachial artery FMD, and lower capillary density. However, there were no differences between dippers and non-dippers regarding age, sex, height SDS, weight SDS, CIMT SDS, brachial artery FMD, and capillary density., Conclusions: Understanding the vascular consequences associated with essential hypertension emphasizes the importance of early detection and management of hypertension. Herein, we have effectively highlighted significant endothelial changes through the analysis of three parameters in newly diagnosed children without apparent target organ damage., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Mediastinal radiotherapy for childhood cancers, particularly Hodgkin disease, has numerous potential adverse effects, including coronary artery disease, pericarditis, cardiomyopathy, valvular disease, and conduction abnormalities. The prevalence of valvular stenosis is relatively low, and regurgitation is more common. Mediastinal radiotherapy-induced valvular disease develops more than 10 years after radiotherapy. Here, we present a case of a 6-year-old boy with moderate to significant mitral stenosis + moderate mitral regurgitation and mild aortic regurgitation that appeared 1.5 months after radiotherapy and showed a progressive course., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Everolimus is a mTOR inhibitor that has been increasingly used in high-risk cardiac rhabdomyomas in recent years. There are questions regarding the optimal dose and duration of therapy with everolimus for cardiac rhabdomyomas. The purpose of this study was to examine retrospectively the dosage-efficacy relationship in seven babies diagnosed with rhabdomyoma treated with different everolimus dose regimens retrospectively. Cardiac rhabdomyoma diagnosis was made in six of seven babies during the prenatal period. Indication of everolimus was an obstruction in six patients and supraventricular tachycardia which is resistant to antiarrhythmic drugs in the remaining one patient. The median age was 8 days (range; 2-105 days) at the time of starting everolimus. It was administered at a dose of 0.25 mg twice a day for two days a week in four patients; 0.1 mg/day in two and 0.4 mg/day in one patient. Serum everolimus level was kept between 5 and 15 ng/ml. All seven cases showed significant regression of cardiac rhabdomyoma within four weeks, and supraventricular tachycardia was controlled in two weeks after everolimus administration.This study demonstrates that everolimus was effective in accelerating regression of the cardiac rhabdomyoma. Dose with 2 × 0,25 mg/day, 2 days a week, seems appropriate. However, lower doses such as 0.1 mg/day are also effective. But dose adjustment should be made according to serum level monitoring.

We aim to evaluate the clinical course and outcome of cases with a prenatal diagnosis of ectopia cordis in our center. In this retrospective study, we analyzed clinical variables including gestational age at diagnosis, maternal age, associated cardiac, extracardiac, genetic anomalies and, outcome in prenatally diagnosed ectopia cordis cases in our tertiary referral center. Eight ectopia cordis cases from seven pregnancies were included in the study. All fetuses had complete type of ectopia cordis and pentalogy of Cantrell. Five multiple pregnancies were found, four were twin pregnancies (three dichorionic diamniotic, one monochorionic monoamniotic) and one was triplet (trichorionic triamniotic). In the monochorionic monoamniotic twin pregnancy, both fetuses have pentalogy of Cantrell. Two cases had intracardiac structural defects including Tetralogy of Fallot and hypoplastic right heart syndrome. Three pregnancies were terminated, four cases delivered alive could not survive beyond the neonatal period. The striking feature in our study is its association with multiple pregnancies., (© 2023 Japanese Teratology Society.)

Objectives: To evaluate the macular and optic nerve head (ONH) vascular density, foveal avascular zone area, and outer retina and choriocapillaris flow in Multisystemic Inflammatory Syndrome in Children (MIS-C) using optical coherence tomography angiography (OCTA)., Methods: Thirty-four eyes of 34 patients with MIS-C and 36 age and sex-matched healthy controls were investigated in this prospective, cross-sectional study. The superficial capillary plexus (SCP) and deep capillary plexus (DCP), ONH, FAZ parameters, the flow area of the outer retina, and choriocapillaris were evaluated using OCTA., Results: All VD parameters in SCP were significantly lower in MIS-C patients. There was no significant difference between the groups in VD parameters of both DCP and ONH, as well as FAZ area and FAZ perimeter. However, foveal density (FD-300) was significantly decreased in the MIS-C group. (p = 0.024). The outer retina flow area at 1 mm, 2 mm, and 3 mm radius and CC flow area at 1 mm and 2 mm radius were significantly lower in the MIS-C group than in the control group. Although CC flow area at 3 mm radius was decreased in the MIS-C group compared to healthy controls, the difference was not statistically significant., Conclusions: We demonstrated a decreased vessel density in SCP, choriocapillaris flow area, and outer retinal flow area in MIS-C patients. Hence, we proposed that OCTA could reveal retinal and choroidal microvascular changes in MIS-C patients who were completely healthy before the diagnosis of MIS-C., (© 2022. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

To evaluate prenatal findings of the right aortic arch (RAA), associated cardiac, extracardiac, and genetic anomalies, its perinatal outcomes and the need for postnatal interventions in cases of isolated RAA with a view to facilitating appropriate counseling. This was a multicenter, cohort study, that was undertaken in two international major cardiac centers between 2009 and 2020. The study subjects were prenatally diagnosed RAA cases with and without other structural cardiac defects. A RAA was identified in 137 fetuses. There were 84 cases of isolated RAA. Associations with additional intracardiac malformations were found in 53 (38.7%) cases. An extracardiac anomaly was observed in 26/137 (18.9%) fetuses, 11/84 (13.0%) fetuses with isolated RAA, and 15/53 (28.3%) fetuses with an additional intracardiac anomaly. The incidence of extracardiac and chromosomal anomalies was significantly higher in cases of RAA with abnormal intracardiac anatomy (28.3-18.8%, respectively), compared with RAA with normal intracardiac anatomy (13.0-5.9%, respectively) (p < 0.05). 22q11.2 microdeletion was found higher in RAA with CHD (4/18 fetuses) than isolated RAA (2/24 fetuses) (22.2% vs. 8.3% respectively). ALSA was present in 19.3% of cases. ALSA was more frequently observed in cases of isolated RAA (23.6%), than in RAA with structural CHD (7.6%) (p < 0.05). The pregnancy was interrupted in six fetuses, and one died in utero. The mortality rate was higher in fetuses with intracardiac anomaly than RAA without cardiac anomaly (11/49 (22.4%) vs. 2/81 (2.4%). Vascular ring formation was revealed in 21/98 cases. The RAA caused symptoms of a vascular ring in only one patient (0.7%) requiring surgery in the follow-up. Overall survival after initial diagnosis in the total cohort was 85.4% with 38 of 53 (71%) RAA with CHD cases and 79 of 84 (94.0%) isolated RAA cases. Chromosomal and extracardiac anomalies are lower in isolated RAA but not negligible hence amniocentesis should be routinely offered in all cases. The requirement for postnatal intervention in the immediate neonatal period is remote, therefore delivery of these fetuses need not be undertaken at a cardiac or surgical center., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: The aim of the study was to evaluate the early myocardial dysfunction detected by strain echocardiography in children with multisystem inflammatory syndrome related to SARS-CoV-2 infection., Methods: This cross-sectional study was conducted with 47 patients diagnosed with MIS-C and 32 healthy age- and gender-matched children. All patients underwent two-dimensional, colour, pulsed, and tissue Doppler, and 2D speckle tracking echocardiography examination at admission, 2 weeks, and 2 months after discharge. The MIS-C patient group was compared with the control group. Echocardiographic changes in MIS-C patients during follow-up were evaluated., Results: Of 47 patients, 30 (63.8%) were male and 17 (36.2%) were female. The mean age at diagnosis was 9.1 ± 4.3 (1.25-17) years. At admission, 25 patients had abnormal findings on conventional echocardiography. Among them, eight patients had left ventricular systolic dysfunction. Ejection fraction and fractional shortening were significantly lower in the patient group at admission compared to controls (p = 0.013, p = 0.010, respectively). While the ejection fraction was <55% in eight patients, and global longitudinal strain was lower than -2SD in 29 patients at admission. Global longitudinal strain z-score <-2SD persisted in 13 patients at 2-month follow-up. Ejection fraction increased above 55% in 3.42 ± 0.53 days in 7 of 8 patients with left ventricular systolic dysfunction, ejection fraction was 51% at discharge in one patient, and left ventricular systolic dysfunction persisted at the 6-month of follow-up., Conclusion: These results confirmed that speckle tracking echocardiography is more likely to detect subclinical myocardial damage compared to conventional echocardiography. In addition, it is a valuable method for follow-up in this patient group.

Objectives: To describe fetal spectrum and echocardiographic characteristics of interrupted inferior vena cava (IIVC) with azygos/hemiazygous continuation without other structural heart defects and to evaluate its association with visceral heterotaxy and isomerism, extracardiac and genetic anomalies, and to review neonatal outcome., Methods: This was a retrospective study of 14 fetuses with a confirmed diagnosis of IIVC with normal intracardiac anatomy. The following variables were collected; indication for referral, gestational age at diagnosis; associated isomerism and visceral heterotaxy, heart rhythm, genetic and extracardiac abnormalities, and fetal/neonatal outcome., Results: Among 36 fetuses with IIVC, 14 cases (38.8%) had normal intracardiac anatomy. These IIVC cases correspond to 0.19% (14/7250) of all fetal cardiac examinations, and to 1.5% (14/922) of all cardiac abnormalities. Six patients had visceral abnormalities. Atrial appendage morphology was clearly depicted in three fetuses, both appendages were left. One fetus had bradyarrhythmia revealing atrial ectopic rhythm. Six fetuses did not have any concomitant cardiac or visceral abnormalities, therefore regarded as isolated. All babies were delivered at term with a good prognosis., Conclusion: Our study has shown that almost half of the IIVC cases without intracardiac structural anomalies displayed other findings of isomerism while the other half was isolated benign vascular variant. Therefore, prenatal diagnosis of IIVC should prompt a comprehensive evaluation for cardiac, situs, and visceral anomalies. The outcome is favorable., (© 2022 Wiley Periodicals LLC.)

Background: The aim of this study was to evaluate the outcomes of patients with the multisystem inflammatory syndrome in children (MIS-C) according to phenotypes of disease and define the prognostic factors for the severe course., Methods: This cross-sectional study included 293 patients with MIS-C from seven pediatric rheumatology centers. A two-step cluster analysis was performed to define the spectrum of disease and their outcomes were compared between each group., Results: Four subgroups were identified as follows: cluster I, predominantly Kawasaki-like features ( n = 100); cluster II, predominantly MAS-like features ( n = 34); cluster III, predominantly LV dysfunction ( n = 47); cluster IV, other presentations ( n = 112). The duration of fever was longer in cluster II and the length of hospitalization was longer in both clusters II and III. Laboratory findings revealed lower lymphocyte and platelet counts and higher acute phase reactants (APRs) in cluster II, while patients in cluster IV showed less inflammation with lower APRs. The resolution of abnormal laboratory findings was longer in clusters II and III, while it was shortest in cluster IV. Seven patients died. Among them, four belonged to cluster II, while three were labeled as cluster III. Patients with severe course had higher levels of neutrophil-lymphocyte ratio, mean platelet volume, procalcitonin, ferritin, interleukin-6, fibrinogen, D-Dimer, BNP, and troponin-I, and lower levels of lymphocyte and platelet counts., Conclusion: As shown, MIS-C is not a single disease presenting with various clinical features and outcomes. Understanding the disease spectrum will provide individualized management.

Background: To investigate the diagnostic accuracy of CXCL10/IP10 for left ventricular (LV) dysfunction in multisystemic inflammatory syndrome (MIS-C)., Methods: This cross-sectional, longitudinal study included 36 patients with MIS-C. Patients were classified as follows: (1) patients presenting with Kawasaki-like features (group I = 11); (2) patients presenting with LV systolic dysfunction (group II = 9); and (3) other presentations (group III = 3). CXCL10/IP10 levels were measured upon admission and on days 3 and 7 of treatment., Results: Twenty patients were male and 16 were female. The median age of patients at diagnosis was 7.5 (1.5-17) years. All patients had a fever lasting for a median of 4 (2-7) days. Ten patients had LV systolic dysfunction. The duration of hospitalization was longer in group II. Lymphocyte and platelet counts were lower, whereas NT-pro-BNP, troponin-I, D-dimer, and CXCL10/IP10 levels were higher in group II. Baseline levels of CXCL10/IP10 were weakly negatively correlated with ejection fraction (r = -0.387, p = 0.022). Receiver operator characteristic curve analysis yielded a cutoff value of CXCL10/IP10 to discriminate patients with LV dysfunction was 1839 pg/mL with sensitivity 88% and specificity 68% (Area under curve (AUC) = 0.827, 95% CI 0.682-0.972, p = 0.003)., Conclusion: Having a good correlation with cardiac function, CXCL10/IP10 is a potential biomarker to predict LV dysfunction in MIS-C patients.

Aim: When encountering adolescents with chest pain and a high troponin level but with no underlying coronary artery illness, it is advisable to consider myopericarditis. Though myopericarditis is a self-limiting, benign condition, it nevertheless causes anxiety in the patient and the family., Methods: Thirty-nine patients diagnosed with myopericarditis were included. We retrospectively analysed the demographic and clinical features, laboratory tests, echocardiography, electrocardiograms, MRI findings, coronary CT angiography, and conventional angiography findings in these patients., Results: Of the 39 patients (female/male = 4/35) aged 7-17 years, 66.6% had viral infection in the 2 weeks preceding presentation. Eleven patients were tested for high-sensitivity cardiac troponin I, 28 for high-sensitivity cardiac troponin T, and 10 patients were tested for both biomarkers. The median hs-TnI and hs-TnT values were 6.3 (0.05-29.9) ng/mL and 586 (51-9398) ng/L, respectively. Twenty-three patients showed ST changes on electrocardiography, of whom 11 had ST-elevation in the leads supporting left ventricular involvement. Coronary CT angiography and catheter angiography evaluations performed for differential diagnosis of coronary anomaly and acute coronary syndrome were normal. Cardiac MRI was conducted on 28 patients, and the results in 10 (35.7%) were suggestive of myopericarditis., Conclusions: Myopericarditis is common in the adolescent age group and is generally benign but should be carefully monitored for differential diagnosis and possible complications. Cardiac MRI, which has been used more frequently in recent years, has an important role in differential diagnosis and the follow-up of patients.

We described a very rare case of aorto-pulmonary communication with right aortic arch and crossed pulmonary artery that cannot be placed in the typical anatomic classification of aortopulmonary window. At 23 weeks gestation, fetal echocardiography revealed a large tunnel-like communication connecting the great vessels proximal to the main pulmonary artery bifurcation, rather than a classic aortopulmonary window between the ascending aorta and the main pulmonary artery.

We report isolated extramedullary relapse in a 14-year-old boy, sequentially presenting with intestinal and cardiac myeloid sarcoma (MS). Acute myeloblastic leukemia M5 was diagnosed 41 months ago. On the 14th month of the second HSCT, he presented with ileus and underwent surgical treatment. After 2 weeks, arrhythmia, bradycardia, complete heart block, and atrial flutter developed and echocardiography revealed multiple cardiac masses. There was no bone marrow relapse but pathology of the intestinal biopsy showed leukemic infiltration. Patient was successfully treated with a permanent pacemaker and salvage chemotherapy. To the best of our knowledge, this is the first pediatric cardiac MS developed after HSCT., (© 2021 Wiley Periodicals LLC.)

Objective: Multisystem inflammatory syndrome in children (MIS-C) associated with the coronavirus disease 2019 (COVID-19) is a new concern emerging as a severe presentation of COVID-19 in children. We aimed to describe the characteristics and short-term outcomes of children diagnosed with MIS-C., Material and Methods: A retrospective study was conducted on 24 patients who were diagnosed with MIS-C between June 1, 2020 and December 1, 2020. A total of 24 (14 male and 10 female) patients were included in the study., Results: The median age at the diagnosis was 111 (10-180) months. A total of 17 patients had a history of contact with a patient with COVID-19. Among the 24 patients, the most common findings were gastrointestinal involvement (n=20), followed by conjunctivitis (n=12), erythematous rash (n=11), and oral changes (n=10). Cardiovascular involvement was detected in 12 patients, of whom six had systolic dysfunction, four had mild coronary artery involvement, four had pericardial effusion, and three had mitral insufficiency. All patients received intravenous immunoglobulin, and 14 patients were treated with methylprednisolone in addition. Anti-interleukin-1 was given to two patients. The median duration of hospitalization was 8 (5-15) days. A total of 23 patients were discharged and evaluated on the median of 68.5 (52-140) days after discharge. The remaining one patient with dilated cardiomyopathy died after 2 months in the intensive care unit., Conclusion: Increasing the knowledge on MIS-C will provide clinicians with information on early recognition, evaluation, and management of these patients., Competing Interests: Conflict of interest: The authors have no conflicts of interest to declare., (Copyright © 2021 Turkish Pediatric Association.)

Objective: This study aimed to determine the possibility of subclinical myocardial dysfunction detected by strain echocardiography in the late period of children with Kawasaki disease., Material and Methods: The study enrolled 30 patients with Kawasaki disease with a follow-up period of at least 12 months and 30 healthy age- and gender-matched children. During the follow-up period, standard echocardiography, pulsed and tissue Doppler, and strain echocardiography were recorded for both groups., Results: The mean age at the time of the diagnosis was 2.6±2.3 years (2 months-11 years). The mean follow-up period after the diagnosis was 3.55±2.20 years. Conventional echocardiography, M mode, pulsed and tissue Doppler values, and myocard performance index did not reveal significant differences. Left ventricle strain and strain rate parameters obtained by apical four-, three-, and two-chamber views did not show statistical differences between patients and controls. There was a positive correlation between the duration of follow-up and global four- and three-chamber longitudinal strain and global longitudinal strain values (r=0.465, p=0.010; r=0.414, p=0.023; r=0.492, p=0.006, respectively), whereas global radial strain showed negative correlation (r=-0.517, p=0.003)., Conclusion: The analysis of systolic strain and strain rate did not detect a subclinical myocardial dysfunction in the long-term follow-up of Kawasaki disease. However, strain values showed variability with the follow-up periods, which indicates that Kawasaki disease might cause left ventricular dysfunction in the later phases. Therefore, a follow-up of children with a diagnosis of Kawasaki disease is of capital importance., Competing Interests: Conflict of Interest: The authors have no conflicts of interest to declare., (Copyright © 2021 Turkish Pediatric Association.)

Objective: Survivors of childhood cancer treated with anthracyclines carry the risk for developing late-onset cardiotoxicity. The purpose of this study was to evaluate left ventricular (LV) function in this patient group and compare it with healthy controls by means of conventional and speckle tracking echocardiography (STE) after exposure to chemotherapy., Material and Methods: Conventional and STE were performed in 45 childhood cancer survivors (mean age 11 ± 4.6; 26 male) treated with anthracyclines (median cumulative dosage 240 mg/m 2 ; range, 100-460) and compared with age, gender and body surface area matched healthy controls. Follow-up period after chemotherapy was 21.9 ± 17.8 months. Blood samples were taken from survivors and controls to determine brain natriuretic peptide (BNP)., Results: Following anthracycline exposure, pediatric cancer survivors had lower longitudinal, radial anteroseptal, and radial anterior strain values compared to controls (P < .05). The calculated global longitudinal and global radial strain values were lower compared to the control group (P < .05). Both groups had normal ejection fraction (EF) and fractional shortening (FS). Brain natriuretic peptide (BNP) levels of both groups were in the normal range., Conclusion: Despite normal EF and FS, children exposed to anthracycline therapy may have late-onset subtle changes of LV strain values measured by STE. Whether these changes of strain can predict future risk of developing heart failure needs to be explored in further studies., (© 2017, Wiley Periodicals, Inc.)