Comorbidity of behavioral problems and parental acceptance-rejection in children diagnosed with chest discomfort, palpitations, vasovagal syncope, and underlying heart disease: a multiple case-control study.

BACKGROUND: Children who experience chest discomfort, palpitations, vasovagal syncope, and underlying heart disease often present a complex clinical picture. Not only are they dealing with potential cardiac issues, but they may also exhibit behavioral problems that can complicate the diagnostic and treatment process. Moreover, parental acceptance or rejection can significantly influence the child's well-being and medical outcomes in such cases. This study aims to explore the comorbidity of behavioral problems and parental acceptance-rejection in children diagnosed with these cardiac symptoms and underlying heart disease. METHODS: In a case-control study, the Parental Acceptance - Rejection Questionnaire and Parental version of Strengths and Difficulties Questionnaire (SDQ) was filled by parents of 314 patients from pediatric cardiology clinic. RESULTS: The control group scored substantially lower overall according to SDQ. The vasovagal syncope subgroup was found to have considerably lower scores on the subscale. The group with chest discomfort scored highly in hostility and aggression in the PARQ. In comparison to the other groups, the vasovagal syncope and chest pain group demonstrated higher scores in undifferentiated rejection and total score. CONCLUSION: This study showed a correlation between children's behavioral and emotional problems and cardiac symptoms. This states that children's relationship with their parents has an impact on their symptoms. It will be necessary to conduct further studies to determine a causal association and devise preventative measures.

Behavioural interventions to increase adherence to palivizumab prophylaxis in children with CHD.

OBJECTIVES: Adherence to palivizumab prophylaxis programmes is crucial to protect infants with CHD against respiratory syncytial virus infections. We analysed the effectiveness of two nudge interventions in increasing adherence. METHODS: Our study included 229 infants, and their caregivers, from five centers in Turkey in the 2020-2021 respiratory syncytial virus season. We randomly allocated caregivers to a control and two intervention groups. Caregivers in all groups were informed about the prophylaxis programme and provided a schedule. Additionally, caregivers in Intervention 1 were called two days before appointments (default bias) and were asked to plan the appointment day (implementation intention), whereas caregivers in Intervention 2 received biweekly text messages informing them about the programme's benefits (availability bias) and current adherence rate (social norm). RESULTS: Caregivers in Intervention 1 had a significantly higher adherence rate than Control (97.3% versus 90.9%) (p = 0.014). Both interventions had a significant effect on participants in their first prophylaxis season (p = 0.031, p = 0.037). Families where the father was employed had a 14.2% higher adherence rate (p = 0.001). Every additional child was associated with a 2.2% decrease in adherence rate (p = 0.02). In control, ICU admission history was associated with an 18.8% lower adherence rate (p = 0.0001), but this association disappeared in intervention groups. CONCLUSION: This is the first prospective interventional study which, in the context of palivizumab prophylaxis, analyses the effectiveness of nudge interventions based on established cognitive biases by comparing randomly generated intervention and control groups. We found that default bias and implementation intention have significant effects on adherence.Clinical trial, in the name and number "Adherence of palivizumab prophylaxis, NCT05778240" registered retrospectively. https://clinicaltrials.gov/ct2/show/NCT05778240.

Acute myocarditis following COVID-19 mRNA vaccination: a paediatric case.

Myocarditis is an inflammation of the heart muscle. In this case, a previously healthy, 17-year-old adolescent with myocarditis after BNT162b2 mRNA vaccination was reported. He was admitted to the hospital with severe chest pain, changes in electrocardiography, and elevation in serum troponin level after fourth day of receiving first dose of vaccine. There was no coronary arterial disease in coronary angiogram. A diagnosis of vaccine-induced myocarditis was made, and supportive treatment was initiated.

Congenital biventricular cardiac diverticula as a part of class III Cantrell's syndrome: case report.

Congenital cardiac ventricular diverticulum is an extremely rare condition that usually occurs as a part of Pentalogy of Cantrell and frequently associated with sternal, pericardial, diaphragmatic, and thoracoabdominal wall defects. The prognosis of the patient depends on the complexity of abnormalities. Herein, we report biventricular diverticula as a part of incomplete Cantrell's syndrome in a 1-day-old newborn.

Cardiovascular anomalies in Seckel syndrome: report of two patients and review of the literature.

Seckel syndrome is a very rare autosomal recessive disorder also known as bird headed dwarfism". It is characterised by proportional short stature, low birth weight, dysmorphic facial appearance, and mental retardation. In addition to its dysmorphic features, skeletal, endocrine, gastrointestinal, haematologic, genitourinary, and nervous system has been involved. Cardiovascular features very rarely associate with Seckel syndrome. We report two patients with Seckel syndrome, one with dilated cardiomyopathy and the other with multiple ventricular septal defects. Dilated cardiomyopathy and isolated ventricular septal defect have not been previously reported in Seckel syndrome. Cardiovascular evaluation should be performed in all patients with Seckel syndrome. Early diagnosis of congenital and acquired heart diseases will reduce morbidity and mortality in these patients.

Are there any novel markers in acute rheumatic fever: neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio.

OBJECTIVE: The aim of this study was to investigate the relationship between C-reactive protein and erythrocyte sedimentation rate and neutrophil-to-lymphocyte, platelet-to-lymphocyte, and monocyte-to-lymphocyte ratios in acute rheumatic fever in children. METHOD: In this retrospective study, 182 patients with acute rheumatic fever and 173 controls were included. Complete blood count parameters, and neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were recorded for all the patients underwent transthoracic echocardiography. RESULTS: Neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were significantly higher in patients with rheumatic heart disease than patients without cardiac involvement (p < 0.05). C-reactive protein and erythrocyte sedimentation rate levels were found to have a positive correlation with neutrophil-to-lymphocyte (r = 0.228, p = 0.001; r = 0.355, p = 0.001), platelet-to-lymphocyte (r = 0.227, p = 0.01; r = 0.149, p = 0.005), and monocyte-to-lymphocyte ratios (r = 0.117, p = 0.005; r = 0.107, p = 0.044). Cardiac involvement was present in 152 (83.5%) of the patients. Neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios were significantly higher in patients with rheumatic heart disease than patients without cardiac involvement (p < 0.05). Patients with carditis were grouped according to mitral, aortic, or both valve involvement but there was no significant difference between the groups with respect to neutrophil-to-lymphocyte, monocyte-to-lymphocyte, and platelet-to-lymphocyte ratios. In addition, neutrophil-to-lymphocyte and monocyte-to-lymphocyte ratios were significantly higher in patients with Sydenham's chorea than without chorea (p < 0.05). CONCLUSION: Neutrophil-to-lymphocyte, platelet-to-lymphocyte, and monocyte-to-lymphocyte ratios may help make the diagnosis of acute rheumatic fever and its prognosis by serial measurements in follow-up but none of them tell us the severity of carditis. Also, this is the first study showing the positive correlation between Sydenham's chorea and neutrophil-to-lymphocyte and monocyte-to-lymphocyte ratios. Further studies are needed to confirm this hypothesis, as this is the first study in the literature on this topic.

A rare coincidence: the long QT syndrome and cardio-facio-cutaneous syndrome.

Cardio-facio-cutaneous syndrome is a genetic anomaly characterised by craniofacial dysmorphia, developmental retardation, skin lesions, mental retardation/learning disability, and cardiac malformations. Cardio-facio-cutaneous syndrome rarely causes arrhythmias and has not been previously associated with long QT in the literature. With this report, it was aimed to draw attention to a different presentation of the long QT syndrome.

MicroRNA values in children with rheumatic carditis: a preliminary study.

MicroRNAs (miRNAs) are fine regulators of gene expression which participate in the regulation of almost every phase of cell physiology, including development of immune cells and adjustment of immune response. In the studies with in vitro/in vivo model systems, specific miRNAs are revealed to have various roles in cardiovascular development and physiological functions. Furthermore, some studies have been done to understand the role of miRNAs about myocarditis, heart failure and coronary artery diseases. miRNAs crucial role in the pathogenesis of other rheumatic diseases have been investigated, however rheumatic carditis was not studied. The aim of this study is to assess values of miRNAs in children with rheumatic carditis and compare them with healthy children. This study included 36 children with rheumatic carditis (mean aged 12.1 ± 2.1 years) and age-gender matched 35 healthy controls (mean aged 11.1 ± 2.3 years). Conventional echocardiography was performed to all subjects. Using real-time polymerase chain reaction, the expression of some miRNAs (hsamiR-16-5p, hsa-miR-221-3p, hsa-miR-223-3p, hsa-miR-10a-5p, hsa-miR-24-3p, hsamiR-92a-3p, hsa-iR-320a, hsa-miR-21-5p, hsa-miR-155-5p, hsa-miR-132-3p, hsamiR-146a-5p, hsa-miR-499a-5p, hsa-miR-1, hsa-miR-125, hsa-miR-196a-5p, hsa-miR-130b-3p, hsa-miR-133b, hsa-miR150-5p,hsa-miR-204-5p, hsa-miR-203a) were analyzed. hsa-miR-16-5p(-1.46 fold, p < 0.01), hsa-miR-223-3p(-1.46 fold, p < 0.01), and hsa-miR-92a-3p(-1.27 fold, p < 0.05) expressions in the patients were lower than those of controls, whereas other examined miRNAs did not differently express between the groups. Results of the study demonstrated that significant downregulation of hsa-miR-16-5p, hsa-miR-223-3p and hsa-miR-92a-3p in children with rheumatic carditis. Since, this is the first study in children with rheumatic carditis, further studies are needed for lightening whether these miRNAs might be helpful as biomarkers.

Prognostic markers in congenital diaphragmatic hernia: Left ventricular diameter and pulmonary hypertension.

BACKGROUND: The aim of this study was to investigate whether the outcome of congenital diaphragmatic hernia (CDH) in newborns can be predicted using left ventricular (LV) diameter and estimated pulmonary artery pressure. METHODS: Patients in the newborn unit in 2012-2016 were screened retrospectively. Echocardiographic measurements of 35 patients with isolated left lateral CDH and 27 healthy newborns were compared in the first 24 h of life. RESULTS: Mean LV end-diastolic diameter (LVEDD) and end-systolic diameter (LVESD), and LV ejection fraction were significantly lower in the CDH group. Moreover, tricuspid regurgitation (TR) and pulmonary regurgitation (PR) were significantly higher than in the controls (P < 0.001). Six CDH patients died within the next 40 days after birth. Mean LVEDD and LVESD were significantly lower in the CHD patients who died compared with those who were discharged (P < 0.001, P = 0.016). Also, mean TR and PR (P < 0.001) and the frequency of pulmonary hypertension (PH; P = 0.001) were significantly higher in these patients. On receiver operating characteristic analysis of the CDH non-survivors, LVEDD < 11 mm (sensitivity, 100%; 95%CI: 87.9-100; specificity, 100%; 95%CI: 54.1-100) and TR > 3.5 m/s (sensitivity, 89.66%; 95%CI: 72.6-97.7; specificity, 100%; 95%CI: 54.1-100) were associated with poor prognosis. CONCLUSIONS: Decrease in LVEDD and presence of PH are associated with poor prognosis. Also, PH was associated with mortality in CDH patients. Thus, outcome may be predicted on careful echocardiographic evaluation of the LV diameters and pulmonary pressure.

2D-Speckle tracking echocardiography contributes to early identification of impaired left ventricular myocardial function in children with chronic kidney disease.

IntroductionThe aim of this study is to determine early changes in cardiac function of children with chronic kidney disease by using 2D-speckle tracking echocardiography. METHOD: The study included 38 children - 16 girls and 22 boys - diagnosed as having chronic kidney disease in the nephrology department with a glomerular filtration rate of <90 ml/minute/1.73 m2 for at least 3 months. A total of 37 - 15 girls and 22 boys - age- and sex-matched healthy children were included as the control group. 2D-Speckle tracking echocardiography was performed in all subjects. RESULTS: The mean age was 13.45±2.8 years in patients and 12.89±3.07 years in controls. Systolic and diastolic blood pressures and left ventricular mass index were significantly higher in patients (p<0.05). The values of mitral e, mitral a, mitral e/a ratio, and mitral deceleration time were not different between the groups. Tricuspid annular plane systolic excursion values were lower in patients (p<0.01). Global strain values in apical long-axis 3-chamber and 2-chamber views were significantly lower in patients (p<0.05). Longitudinal, radial, and circumferential peak systolic strain values were lower in patients, but the difference was statistically significant in all segments of longitudinal view and basal segment of circumferential view (p<0.05). Radial and circumferential systolic strain rates were significantly lower in patients in all three segments (p<0.05). Moreover, early diastolic strain rate was significantly lower in longitudinal and radial apical segments and in all segments of circumferential measurements in patients. Besides, strain rate e/a ratio was significantly lower in all longitudinal segments of patients (p=0.01). CONCLUSION: The study concluded that 2D-speckle tracking echocardiography method can determine cardiac involvement earlier than conventional echocardiography in children with chronic kidney disease having preserved ejection fraction.

Can whole-blood parameters be used in follow-up of children with rheumatic valvular heart disease?

OBJECTIVES: The aim of the present study was to investigate the relationships between red blood cell distribution width, platelet distribution width, and mean platelet volume and the presence and severity of valvular involvement in patients with rheumatic heart disease. METHODS: Between April, 2012 and December, 2015, 151 patients who were admitted to the Pediatric Cardiology Unit with diagnosis of rheumatic heart disease and 148 healthy children were included to our study. Transthoracic echocardiography for all children was performed, and the values of red blood cell distribution width, platelet distribution width, and mean platelet volume, besides other blood count parameters, erythrocyte sedimentation rate, and C-reactive protein levels were recorded. RESULTS: Red blood cell distribution width, platelet distribution width, mean platelet volume, and C-reactive protein levels were significantly higher in patients with rheumatic heart disease when compared with healthy controls (p0.05). CONCLUSION: This is the first study in children with rheumatic heart disease that demonstrated significantly increased red blood cell distribution width, platelet distribution width, and mean platelet volume levels, as well as evaluated all three parameters together. Furthermore, red blood cell distribution width values in the chronical period of acute rheumatic fever, due to the positive correlation with the other chronic inflammatory markers, may help make the diagnosis in children.

Familial hypertrophic cardiomyopathy: A case with a new mutation in the MYBPC3 gene.

Familial hypertrophic cardiomyopathy is a genetically heterogeneous disease with variable clinical features that is inherited as autosomal dominant with variable penetrance. Recent developments in genetics of hereditary cardiomyopathy have not only enlightened many points about pathogenesis, but have also provided great benefit to diagnostic approaches of clinicians. Heterozygous mutation of c3691-3692insTTCA in MYBPC3 gene was identified in a pediatric patient with diagnosis of hypertrophic cardiomyopathy at clinic. Hypertrophy was observed in sister and father of the patient in echocardiography screening, and it was subsequently determined that they also had same mutation. This mutation has not previously been defined and reported previously in the literature as cause of hypertrophic cardiomyopathy.