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Objective Patients with Behçet's disease (BD) may experience long-term morbidity caused by various forms of cardiovascular disease. This study aimed to assess the risk for cardiovascular comorbidity in paediatric BD patients with and without vascular involvement, independent of the contribution of traditional risk factors. Methods Paediatric patients classified as having BD according to the 2015 Peadiatric BD (PEDBD) criteria were included in the study. Twenty-four-hour ambulatory blood pressure monitoring (ABPM), transthoracic echocardiography, and carotid intima-media thickness (cIMT) measurements were performed. Patients with an active disease or those who have other known risk factors for cardiovascular disease were not included in the study. Results Thirty-one children and adolescents with paediatric BD (16 female, 51.6%; F/M: 1.06) were enrolled in the study. Among the BD patients, 10 patients (34.4%) had abnormal ABPM. Carotid IMT values, mean arterial pressure, systolic and diastolic blood pressure by ABPM and the prevalence of abnormal ABPM, non-dipping, and ambulatory hypertension were similar between patients with and without vascular involvement. The echocardiography measurements showed that BD patients with vascular involvement had a significantly higher velocity and velocity time integral of the left ventricle outflow tract, which may indicate increased stiffness of the aorta. Conclusion Paediatric BD patients with vascular involvement may tend to have more cardiovascular risk factors. However, cardiovascular assessment should be considered in all BD patients, regardless of the involved systems. We suggest that ABPM may accurately define hypertension and cardiovascular risk in BD. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the oral health status and treatment needs of children with congenital and acquired heart disease. Methods: This descriptive study included 301 children aged 5–14 from June 2022 to June 2023. Heart conditions were classified by congenital/acquired status and severity. The children's sociodemographic characteristics, medical and dental history, tooth brushing habits, and non-nutritional habits (bruxism, nail-biting, thumb-sucking, etc.) were evaluated. Oral health assessments including caries, oral hygiene, enamel defects, and dental treatment needs-related indices were recorded. Results: The mean age was 8.95 ± 2.91 years, and 271 (90%) of the children had congenital heart disease. The children with moderate and severe heart disease had significantly higher decayed/missing/filled surfaces (dmfs) (p = 0.038) and pulp exposure (p = 0.015) compared to the children with mild heart disease. According to the International Caries Detection and Assessment System II (ICDAS II) index, which included initial caries lesions, there were no caries-free children and 75.7% had extensive caries. The mean plaque index and gingival index were found to be 1.18 ± 0.38 and 0.69 ± 0.53, respectively. Enamel defects were observed in 15.9%. The Treatment Needs Index (TNI) was 85.8% for the primary teeth and 88.9% for the permanent teeth. The Care Index (CI) was 12.4% for the primary teeth and 10.8% for the permanent teeth. Conclusions: Children with congenital and acquired heart disease exhibit a high prevalence of untreated dental caries, gingivitis, and plaque accumulation, with a high need for dental treatments. Dentists should prioritize addressing these issues to prevent the risk of infective endocarditis (IE) and improve oral health outcomes in this population. [ABSTRACT FROM AUTHOR]

Background: CafeÂ'-au-lait skin macules, Cushing syndrome (CS), hyperthyroidism, and liver and cardiac dysfunction are presenting features of neonatal McCune- Albright syndrome (MAS), CS being the rarest endocrine feature. Although spontaneous resolution of hypercortisolism has been reported, outcome is usually unfavorable. While a unified approach to diagnosis, treatment, and follow-up is lacking, herein successful treatment and long-term follow-up of a rare case is presented. Clinical case: An 11-day-old girl born small for gestational age presented with deterioration of well-being and weight loss. Large hyperpigmented macules on the trunk, hypertension, hyponatremia, hyperglycemia, and elevated liver enzymes were noted. ACTH-independent CS due to MAS was diagnosed. Although metyrapone (300 mg/m2/day) was started on the 25th day, complete remission could not be achieved despite increasing the dose up to 1,850 mg/m2/day. At 9 months, right total and left three-quarters adrenalectomy was performed. Cortisol decreased substantially, ACTH remained suppressed, rapid tapering of hydrocortisone to physiological dose was not tolerated, and supraphysiological doses were required for 2 months. GNAS analysis from the adrenal tissue showed a pathogenic heterozygous mutation. During 34 months of follow-up, in addition to CS due to MAS, fibrous dysplasia, hypophosphatemic rickets, and peripheral precocious puberty were detected. She is still regularly screened for other endocrinopathies. Conclusion: Neonatal CS due to MAS is extremely rare. Although there is no specific guideline for diagnosis, treatment, or follow-up, addressing side effects and identifying treatment outcomes will improve quality of life and survival. [ABSTRACT FROM AUTHOR]

Multisystem inflammatory syndrome in children (MIS-C) is a serious condition characterized by excessive inflammation that can arise as a complication of SARS-CoV-2 infection in children. While our understanding of COVID-19 and MIS-C has been advancing, there is still uncertainty regarding the optimal treatment for MIS-C. In this study, we aimed to compare the clinical and laboratory outcomes of MIS-C patients treated with IVIG plus corticosteroids (CS) to those treated with IVIG plus CS and an additional biologic drug. We used the propensity score (PS)-matching method to assess the relationships between initial treatment and outcomes. The primary outcome was a left ventricular ejection fraction of less than 55% on day 2 or beyond and/or the requirement of inotrope support on day 2 or beyond. We included 79 MIS-C patients (median age 8.51 years, 33 boys) followed in our center. Among them, 50 children (25 in each group) were allocated to the PS-matched cohort sample. The primary outcome was observed in none of the patients in the IVIG and CS group, while it occurred in eight patients in the IVIG plus CS and biologic group (p = 0.04). MIS-C is a disorder that may progress rapidly and calls for extensive care. For definitive recommendations, further studies, including randomized control trials, are required. [ABSTRACT FROM AUTHOR]

Aim: To present the first‐year experience of fetal cardiac interventions (FCIs) in a tertiary referral hospital and to evaluate the outcomes. Methods: This retrospective study consisted of four pregnant women who underwent fetal pulmonary or aortic balloon valvuloplasty between November 2020 and June 2021. The procedures were performed with a percutaneous cardiac puncture under the ultrasonography guidance. Gestational age at intervention, procedural success, complications, and perinatal outcomes were evaluated. Procedural complications defined as fetal bradyarrhythmia requiring treatment, pericardial effusion requiring drainage, balloon rupture, and fetal death. The procedure was considered technically successful if the valve was dilated with a balloon catheter. Ultimately successful procedure was defined as the discharge of infants alive with biventricular circulation. Results: A total of 5 FCIs attempted between 26 + 3 and 28 + 2 gestational weeks. While the procedure was technically successful in 2 cases with pulmonary stenosis, both attempts were unsuccessful in the fetus with pulmonary atresia. Although the procedure was technically successful in the patient with critical aortic stenosis, it ultimately failed. No fetal death occurred in our series and there were no procedure‐related significant maternal complications. However, three interventions were complicated by fetal bradycardia and pericardial effusion necessitating treatment, and balloon rupture cropped up in one case. Conclusion: FCIs may lead to improving the likelihood of a biventricular outcome for selected fetuses. Careful selection of patients and centralization of experience are essential for obtaining favorable outcomes. Operators should be aware of procedural complications. Improved procedural techniques with a lower complication rate will be achieved through advanced medical technology and special balloon catheters. [ABSTRACT FROM AUTHOR]

Aim: In children, a broad range of paroxysmal events, including syncope and arrhythmias, may mimic true epileptic seizures. When a definitive diagnosis could not be established, long-term video electroencephalogram monitoring (LTVEM) should be taken into consideration. Furthermore, epilepsy patients have a higher rate of cardiac comorbidities. The purpose of this study is to evaluate the rationale and results of the pediatric cardiology consultations in patients admitted to the LTVEM unit. Materials and Methods: We retrospectively analyzed the files of children who were admitted to LTVEM unit and consulted with the pediatric cardiology department between January 2006 and May 2014. The patients who had both echocardiography and 24-hour ambulatory electrocardiogram monitoring were included. Results: Among 70 children, 74.3% (n: 52) were classified as having epilepsy, 21.4%. (n: 15) with nonepileptic events, and 4.3% (n: 3) could not be classified. In epilepsy group, 21 children (40.4%) were consulted with pediatric cardiology due to rhythm disturbances detected during LTVEM, the remaining consultations (59.6%) were due to history of known cardiac diagnosis (arrhythmias n: 2, structural/congenital heart disease n: 5), tuberosclerosis (n: 6), drop attacks (n: 5), murmur (n: 5), and other reasons. The cardiac evaluation revealed previously undetected arrhythmia (n:3) and mitral valve prolapse (n:1) in four patients with epilepsy. In addition to the pre-existing long QT syndrome, one child experienced his typical attack, subsequently he was diagnosed as epilepsy. The remaining group consisted of 18 children, with syncope being the most common diagnosis for consultation (n: 10, 55.5%). Conclusion: Our study revealed that a subgroup of children with epilepsy had cardiovascular comorbidities. Additionally, epilepsy was confirmed in some patients who already had cardiac problems. Pediatricians should be aware of potential mimickers of epilepsy and note that epilepsy and cardiac problems may also co-exist. Correct diagnosis and appropriate treatment are crucial in this patient group. [ABSTRACT FROM AUTHOR]

Conflicting results have been reported in small non-homogenous groups of children with chronic renal failure in terms of casual blood pressure and ambulatory blood pressure monitoring (ABPM) parameters and left ventricular hypertrophy (LVH). The aim of our study was to assess the value of ABPM and hematological and biochemical parameters in predicting LVH in children on chronic peritoneal dialysis (CPD). Echocardiography and 24-h ABPM were performed in addition to routine biochemical and hematological evaluations in 47 children on CPD (26 male, 21 female; mean age 14.74 [+ or -] 3.52 years). Mean daytime systolic blood pressure (SBP) and mean daytime diastolic blood pressure (DBP) values were found to be higher than the mean casual SBP and DBP (p=0.001) values. Thirty-three (70.2%) children had LVH. The correlations between the left ventricular mass index and ABPM variables were good. Stepwise multiple regression analysis revealed daytime SBP load ([beta]=0.652; /K0.01) and hematocrit ([beta] = -0.282; p15% and hematocrit value 31% and daytime SBP load Keywords Blood pressure load * Children * Chronic peritoneal dialysis * Hematocrit * Left ventricular hypertrophy, Introduction Ambulatory blood pressure monitoring (ABPM) can be used to precisely characterize the variations in blood pressure (BP) that occur during daily activities [1]. It is also superior to casual [...]

Introduction: Cardiac sympathetic denervation (CSD) is an effective procedure for the treatment of inherited channelopathies. Left CSD has traditionally been recommended as neuromodulation to prevent arrhythmia episodes; however, it is thought that bilateral sympathectomy in combination with Kuntz ablation may have additional effects effective due to the anatomical variability of preganglionic sympathetic fibers. The aim of the study was to share our single‐center clinical experience with bilateral thoracoscopic sympathectomy for cardiac denervation in different groups of pediatric patients with malignant arrhythmias. Methods: Fourteen patients (seven with CPVT, five with LQTS, one with resistant ventricular tachycardia as a sequela of myocarditis, and one with cardiomyopathy and atrial tachycardia) underwent bilateral thoracoscopic sympathectomy for cardiac denervation. Results: In all patients, arrhythmia episodes persisted despite medical therapy, and patients with implantable cardioverter‐defibrillator received appropriate therapies. The rate of appropriate therapies was 3.25 per year (1–5 per year) in the year before sympathectomy. No major complications related to the procedure were observed in any of the patients. The QTc interval of LQTS patients decreased from 506.2 ± 16.9 ms before the procedure to 476 ± 28.8 ms after the procedure. The mean duration of follow‐up after the procedure was 23.3 months (11–47 months). Only two patients received single episodes of therapy 12 and 22 months after CSD, and one patient had arrhythmic events due to noncompliance to medical therapy. Conclusion: Due to the anatomical variability of preganglionic fibers bilateral CSD with Kuntz nerve ablation effective treatment and is a safe option. [ABSTRACT FROM AUTHOR]

Inflammatory myofibroblastic tumour usually has a benign course and is very rarely associated with the heart. It can have life-threatening consequences, depending on its position or the presence of aggressive and metastatic complications. A 3-month-old boy presented with pericardial tamponade and was diagnosed with intrapericardial inflammatory myofibroblastic tumour associated with Coronavirus OC43. A large tumour attached to the left ventricle was completely removed by surgical resection and he made a full recovery. ALK: anaplastic lymphoma kinase; CMV: cytomegalovirus; CRP: C-reactive protein; EB: Epstein–Barr virus; ESR: erythrocyte sedimentation rate; IM: inflammatory myofibroblastic tumour; NSAI: non-steroidal anti-inflammatory drugs; PTFE: polytetrafluoroethylene; SMA: smooth muscle actin [ABSTRACT FROM AUTHOR]

Background Despite concerns about complications with the implantable cardioverter defibrillator (ICD), it is effective for the prevention of sudden cardiac death (SCD). We aimed to analyze our midterm experience with ICD in children and young adults. Methods This retrospective study included patients who were implanted with an ICD between 2001 and 2014. Demographic characteristics, clinical information, shock features, and complications for all patients with ICD were analyzed. The study population was divided into two groups: early-era patients implanted before 2008, and late-era patients implanted after 2008. Results Sixty-nine patients (median age: 12 years, median follow-up: 52 months) were implanted with an ICD. Diagnostic categories were channelopathy (56.6%), cardiomyopathy (36.2%), congenital heart disease (5.8%), and other (1.4%). We performed implantation for primary prevention in 66.6% (39.3% in early-era patients and 85.4% in late-era patients). Thirty-one (44.9%) received 139 appropriate shocks (66% of total shocks) while 14 (20.2%) received 71 inappropriate shocks. However, there was no statistically significant difference in the use of appropriate shocks in the primary (66.7%) versus the secondary (72.2%) prevention groups. The incidence of appropriate and inappropriate shock was 66.7% and 33.3% in the primary prevention group, and 72.2% and 27.8% in the secondary prevention group, respectively. Two patients died, although only one death was the result of a lead problem. Conclusions Although lead integrity problems, inappropriate shocks, and infections are significant issues, ICD therapy appears to be a safe, effective, and necessary option for the prevention of SCD in both children and young adults. [ABSTRACT FROM AUTHOR]

Objective: Numerous investigations have demonstrated that phototherapy (PT) directly or indirectly causes ductal patency by photorelaxation effect. In this observational study, we aimed to assess the effect of PT on the incidence of patent ductus arteriosus (PDA) together with prostaglandins (PGE2) and (PGI2) levels in preterm infants. Methods: Preterm infants whose gestational age < 34 weeks and who required PT in the first 3 d of life were enrolled in this prospective study. The clinical signs of PDA, the data of detailed echocardiographic study were recorded and plasma PGE2 and PGI2 levels were measured before and after PT. The outcome measures were the status of ductus arteriosus and alterations of PGE2 and PGI2 levels under the effect of PT. Results: A total of 44 preterm infants were enrolled in the study, of these 21 (47.7%) were in Group 1 (Non-PDA Group) and 23 (52.3%) were in Group 2 (PDA Group). After PT, ductal reopening occurred in three infants (14.3%) in Group 1, while ductus closed in four infants in Group 2 (17.3%). PT does not seem to effect ductal patency for both groups ( p = 0.250 and p = 0.125, respectively). PGE2 levels were not different before and after PT for both groups ( p = 0.087, p = 0.408, respectively). However, PGI2 levels were significantly decreased after PT in both groups ( p = 0.006, and p = 0.003, respectively). Conclusion: There was no effect of PT on ductal patency. We can conclude that PGs were eliminated simultaneously with ductal closure and photorelaxation effect did not influence PG levels. [ABSTRACT FROM AUTHOR]

We report here the successful treatment of a 16-year-old female who ingested 20 tablets of digoxin each containing 0.25mg (total dose ingested equivalent to 0.1 mg/kg), 32 tablets of warfarin each containing 5mg (equivalent to 3.2 mg/kg), and approximately 15 tablets of propafenone each containing 300mg (equivalent to 90 mg/kg). The patient developed hypotension and sinus bradycardia necessitating external cardiac pacing 17 hours after drug ingestion. In addition to gastric lavage, activated charcoal, blood alkalinisation, administration of vitamin K and temporary cardiac pacing, the authors performed plasma exchange for drug removal and administered rifampicin in order to increase the metabolism of digoxin, propafenone and warfarin. The patient was discharged without any sequelae. Plasma exchange may be lifesaving in drug ingestions where there is a low volume of distribution and high plasma protein binding. Rifampicin, an inducer of cytochrome p450, may be used in intoxications for elimination of drugs with inactive metabolites. [ABSTRACT FROM AUTHOR]

The Autocapture function controls and optimizes the amplitude of the pacing pulse and saves energy. The manufacturer recommends using a special low polarization, low threshold bipolar Pacesetter lead for the Autocapture function. The purpose of this study was to evaluate the compatibility of Autocapture with previously implanted pacing leads. The study included 15 patients (mean age 13.6 ± 3.4 years) who needed pulse generator replacement and received the VVIR pacemaker Regency SR + or the DDDR pacemakers Affinity DR or Integrity DR with the Autocapture function. The new pulse generators connected to previously implanted ventricular leads. At the time of implantation the pacing threshold was 1.0 ± 0.35 V at 0.5 ms, the lead impedance was 580 ± 80 Ω, and the spontaneous R wave amplitude was 7.89 ± 4.89 m V. The polarization signal (PS) was 3.8 ± 3.04 m V, and evoked response (ER) was 8.15 ± 4.57 mV at the predischarge testing. Follow-up telemetry was done at months 1, 3, 6, 12, and 18. The follow-up duration was 9.4 ± 5 months (range 1–18 months). If the results of PS and ER measurements were acceptable for Autocapture, it turned on at the 1-month visit. In six (40%) patients the results were found acceptable for Autocapture function. Age, lead impedance, pacing threshold, intrinsic R wave measurement, lead age, fixation mechanism, and ER measurements were not statistically different in Autocapture suitable and not suitable groups. The main reason not to activate Autocapture had been increased PS. Any significant fluctuations were not observed in pacing threshold, lead impedance, ER, and PS during follow-up. In conclusion, previously implanted pacing leads may be compatible with the Autocapture function. [ABSTRACT FROM AUTHOR]

AbstractPurposeMaterials and methodsResultsConclusions\nIMPLICATIONS FOR REHABILITATIONThe study aimed to assess frailty in adults with congenital heart disease (ACHD) and to compare muscle strength, functional capacity, activities of daily living (ADL), and cognition between frail and non-frail ACHD patients.A cross-sectional study design was used. Sixty people with ACHD aged between 18 and 45 years were included. Frailty was determined according to the Fried criteria. Peripheral muscle strength was assessed with a digital dynamometer, functional capacity with the 6-min walk test (6MWT), ADL with the Glittre ADL test, and cognition with the Montreal Cognitive Assessment (MoCA) test.Frailty was seen in 38.33% (frail = 23 and non-frail = 37) of the participants. In the frail patients, dominant knee extensor strength (p = 0.002), shoulder abductor strength (p = 0.005), 6MWT distance (p = 0.021), and MoCA score (p = 0.005) were significantly lower than those in the non-frail patients. Glittre ADL test (p = 0.002) was significantly higher in the frail patients.Muscle strength, functional capacity, ADL, functional mobility, and cognition were lower in the frail participants with ACHD. Early assessment of frailty in ACHD and planning individualized exercise training programs for frail individuals may be a strategy to reduce the impact of frailty on adverse clinical outcomes.We found that muscle strength, functional capacity, ADL, functional mobility, physical activity, and cognition were lower in the frail ACHD patients than in the non-frail ones.Early assessment of frailty in individuals with ACHD is important for determining health status.Planning individualized exercise training programs for individuals with ACHD may be a strategy to reduce the impact of frailty on adverse clinical outcomes.We found that muscle strength, functional capacity, ADL, functional mobility, physical activity, and cognition were lower in the frail ACHD patients than in the non-frail ones.Early assessment of frailty in individuals with ACHD is important for determining health status.Planning individualized exercise training programs for individuals with ACHD may be a strategy to reduce the impact of frailty on adverse clinical outcomes. [ABSTRACT FROM AUTHOR]

Kawasaki disease is an acute, self-limited vasculitis of infants and children that is now the most common cause of acquired heart disease in the pediatric age group in the United States and Japan. This report presents a case of classic Kawasaki disease with reversible sensorineural hearing loss that was treated with steroid. In conclusion, Kawasaki disease caregivers must be aware of possible sensorineural hearing loss, which is reversible by early intervention and thus improves quality of life. [ABSTRACT FROM AUTHOR]

Abstract: Perigraft seroma is a collection of non-secretory fibrous pseudomembrane surrounding a vascular graft, which is a collection of clear, sterile fluid. Thoracal computed tomography, Magnetic Resonance Imaging, and thoracal ultrasonography have been proposed for diagnosis of this pathology. A consecutive modified Blalock–Taussig (m-BT) shunt complicated by perigraft seroma formation, which is diagnosed using a Doppler ultrasonography (US), is presented here. [Copyright &y& Elsevier]

In this report, we describe a successful percutaneous transcatheter n-butyl 2-cyanoacrylate embolization of a coronary fistula originating from the left anterior descending artery in an adolescent with unexpected recurrent attacks of myocardial ischemia. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 2.5-month old who was admitted to the center with the diagnosis of coronary artery fistula. It states that coronary artery fistulas (CAF) are rare congenital or acquired malformations in which a direct vascular connection from coronary artery to a cardiac chamber or a great vessel occurs. It indicates that the patient's large CAF was successfully closed with Amplatzer Duct Occluder-II.

Objective: Numerous investigations have demonstrated that phototherapy (PT) directly or indirectly causes ductal patency by photorelaxation effect. In this observational study, we aimed to assess the effect of PT on the incidence of patent ductus arteriosus (PDA) together with prostaglandins (PGE2) and (PGI2) levels in preterm infants. Methods: Preterm infants whose gestational age < 34 weeks and who required PT in the first 3 d of life were enrolled in this prospective study. The clinical signs of PDA, the data of detailed echocardiographic study were recorded and plasma PGE2 and PGI2 levels were measured before and after PT. The outcome measures were the status of ductus arteriosus and alterations of PGE2 and PGI2 levels under the effect of PT. Results: A total of 44 preterm infants were enrolled in the study, of these 21 (47.7%) were in Group 1 (Non-PDA Group) and 23 (52.3%) were in Group 2 (PDA Group). After PT, ductal reopening occurred in three infants (14.3%) in Group 1, while ductus closed in four infants in Group 2 (17.3%). PT does not seem to effect ductal patency for both groups ( p = 0.250 and p = 0.125, respectively). PGE2 levels were not different before and after PT for both groups ( p = 0.087, p = 0.408, respectively). However, PGI2 levels were significantly decreased after PT in both groups ( p = 0.006, and p = 0.003, respectively). Conclusion: There was no effect of PT on ductal patency. We can conclude that PGs were eliminated simultaneously with ductal closure and photorelaxation effect did not influence PG levels. [ABSTRACT FROM AUTHOR]