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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]

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]

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]