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.

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.

Cardiovascular Screening before Sports Participation: Results of 11487 Children.

AIM: In this study, it is aimed to analyze the data of children who were referred to our clinic for pre-participation sports screening. METHODS: Data, between September 2017 and December 2021, had been analyzed. All these subjects had been questioned for their personal and family medical stories and examined for cardiovascular system findings. Electrocardiography (ECG) and echocardiography were applied to all of them. RESULTS: 11487 children were consulted to the cardiology clinic for pre-sports participation screening. The mean age was 12.7±4,57 (7-18 years). 34/11487(0,29%) subjects weren't allowed to participate in sports activities at first. In 23 of 34 subjects, cardiac arrhythmias were established. 15 had Wolff-Parkinson-White (WPW) syndrome, 3 subjects had ventricular extrasystole, and also ventricular tachycardia in one of them. Four subjects had long QT syndrome and one had ST elevation with the pre-diagnosis of coronary artery disease. ECG screening alone aided in identifying asymptomatic (0.05%) that could have been potentially at risk for sudden cardiac death. CONCLUSION: Positive ECG and echocardiography findings involve a very little ratio.But ECG involves an important tool for screening lethal cardiac arrhythmias in asymptomatic patients.If ECG or echocardiography couldn't be taken, further evaluation should be necessary with symptoms and/or family history.

Cross or Not to Cross-Dilemma of the Pulmonary Arteries.

We aimed to assess the frequency of criss-cross pulmonary arteries and associated intracardiac and vascular anomalies in patients who underwent CT angiography due to suspected congenital heart disease or vascular anomaly at our hospital. We retrospectively evaluated the CT angiography images of 355 patients aged 0-18 years between April 2018 and December 2022. The presence of the criss-cross pulmonary artery anomaly was assessed. Additionally, in patients with a criss-cross pulmonary artery anomaly, accompanying branch pulmonary artery anomalies, aortic arch anomalies, and other vascular-cardiac anomalies were also evaluated. A total of 331 patients' images were evaluated. Criss-cross pulmonary artery anomaly was present in 57 patients (17.2%). Pulmonary artery branch anomaly was present in 16, aortic arch anomaly in 40 patients (70%) with criss-cross pulmonary artery anomaly, while associated intracardiac pathology (by echocardiography) was detected in 43 patients (75.4%). The frequency of criss-cross pulmonary artery was found to be significantly higher in patients with any aortic arch anomaly (p = 0.01). This study represents one of the largest series of patients with criss-cross pulmonary artery anomalies. Our results suggests that it may be more common than previously recognized and potentially overlooked. It is crucial to consider the presence of this anomaly in patients with complex aortic arch anomalies or cardiac pathologies, as it may have implications for surgical approaches and potential complications. Increased awareness of this anomaly among cardiologists and radiologists is necessary for accurate diagnosis and appropriate management.

Acute coronary syndrome due to multi-vessel coronary artery spasm in an Afghan refugee adolescent mimicking recurrent myocarditis.

Vasospastic angina is extremely uncommon for adolescents to experience chest discomfort, which is defined by transitory ST segment elevation or depression and angina symptoms that occur while at rest. It may result in potentially fatal conditions like myocardial infarction, ventricular fibrillation, or even sudden cardiac arrest. To aim of this article is to report a very rare case of a 17-year-old male Afghan refugee who was diagnosed with vasospastic angina after presenting with chest pain, and after receiving calcium channel blocker and nitrates for medical therapy, there were no angina attacks. Our case underlines the value of a thorough evaluation of adolescent's chest pain, the need to diagnose based on the symptoms, and the necessity of performing coronary angiography to rule out coronary causes when there is a high suspicion to a cardiac cause.

Prognostic value of Prognostic Nutritional Index (PNI) for 5-year recurrence-free survival in surgically resected gastrointestinal stromal tumors.

BACKGROUND: Recent studies have investigated the role of neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), systemic inflammation index (SII), and prognostic nutritional index (PNI) on prognosis for various malignancies. However, the value of these markers in determining the prognosis for gastrointestinal stromal tumors (GIST) remains controversial. We investigated the effect of NLR, PLR, SII, and PNI on 5-year recurrence-free survival (RFS) in patients with surgically resected GIST. MATERIALS AND METHODS: We retrospectively analyzed patients (n=47) who had undergone surgical resection for primary, localized GIST at a single institution between 2010 and 2021. The patients were divided into two groups according to the recurrence status in the 5-year period as 5-year RFS(+) (patients with no recurrence (n=25) and 5-year RFS(-) (patients with recurrence (n=22) groups. RESULTS: In univariate analyses, Eastern Cooperative Oncology Group Performance Status (ECOG-PS), tumor localization, tumor size, PNI, and risk category were significantly different between the RFS(+) and RFS(-) groups while NLR, PLR, SII were not. Multivariate analyses revealed that only the tumor size (HR =5.485, 95% CI: 0.210-143.266, p=0.016), and PNI (HR= 112.020, 95% CI: 8.755-1433.278, p<0.001) were independent prognostic factors for RFS. The patients with a high PNI (≥46.25) had a higher 5-year RFS rate than the patients with low PNI (<46.25) (95.2% to 19.2%, p<0.001). CONCLUSION: A higher preoperative PNI is an independent positive predictor for 5-year RFS for patients with surgically resected GIST. However, NLR, PLR, and SII have no significant effect. KEY WORDS: GIST, Prognostic Nutritional Index, Prognostic Marker.

A rare cause of hydrops fetalis in two Gaucher disease type 2 patients with a novel mutation.

Gaucher disease type 2 is the most progressive and the rarest form of Gaucher disease, defined as the acute neuronopathic type. We presented two GD2 patients who died before three months of age due to severe septicemia, respiratory and liver failure. One was homozygous for a novel GBA variant c.590 T > A (p.197 K), and the second homozygous for the known GBA mutation c.1505G > A (p.R502H). Ichthyosis, hydrops fetalis, apnea, myoclonic seizures, and hepatosplenomegaly occurred in both patients, but hypertrophic cardiomyopathy was observed only in the second and unilateral cataract in the first patient. Due to the disease's early and rapid neurological progression, we did not administer ERT to our patients. It is strongly believed that early diagnosis is essential, and prenatal diagnosis makes genetic counselling possible for future pregnancies.

Femoral venous haemostasis in children and young adults using the 'figure-of-eight' suture technique.

AIM: The aim of our study was to evaluate the safety and efficiency of the 'figure-of-eight' suture among children and young adults with congenital heart defects who underwent interventional procedures, in patients with structurally normal hearts who underwent electrophysiological study and in haemodynamically impaired children and newborns. We also reported a novel femoral haemostasis method in patients with a central catheter by modifying the 'figure-of-eight' suture around the catheter for haemorrhage control. METHOD: Between 2015 and 2018, a total of 100 'figure-of-eight' sutures were performed in 90 patients (48 males, 42 females) where the median age was 12.5 years (minimum 3 days, maximum 22 years). The procedures were diagnostic angiography (n = 6), radiofrequency and/or cryoablation (n = 7) and interventional procedures (n = 87). RESULT: Haemostasis was achieved in 89 of 90 patients. Haemostasis could not be achieved in one malnourished patient due to lack of subcutaneous tissue. There were no major complications. A bullous skin lesion and minor bleeding were the only complications seen in two patients. A central catheter was inserted in eight patients using the modified 'figure-of-eight' suture technique. CONCLUSION: The 'figure-of-eight' suture is a safe and effective method for femoral venous haemostasis in patients who require large sheaths for procedures, in those using high-dose heparin and in haemodynamically unstable children who need cardiac catheterisation.

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.

Bilateral thoracoscopic sympathectomy for cardiac denervation in pediatric population: Does Kuntz nerve cauterization have an impact on success?

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.

Transcatheter closure of patent vertical vein after repair of total anomalous pulmonary venous connection: a case series.

BACKGROUND AND OBJECTIVES: Total anomalous pulmonary venous connection is a rare cyanotic CHD that requires surgical repair. An unligated vertical vein after total anomalous pulmonary venous connection surgery may help to decrease the episodes of post-operative pulmonary hypertensive crises, low cardiac output syndrome, and mortality. The aim was to assess long-term outcome and our post-operative transcatheter vertical vein closure experiences in five patients with repaired total anomalous pulmonary venous connection patients. METHODS: A retrospective study was conducted in five cases with an unligated vertical vein following repair of supra-cardiac total anomalous pulmonary venous connection at our hospital from 2011 through 2018. Patients characteristics, cardiac catheterisation findings, surgical, and transcatheter procedural details were retrospectively analysed. RESULTS: Transcatheter closure of the unligated vertical vein was technically successful in all the patients. Procedure-related complications were not observed in any of the patients. No long-term complication was found. CONCLUSIONS: We suggest that transcatheter closure of the patent vertical vein is an effective and well-tolerated alternative to the surgical approach.

Element profiles in blood and teeth samples of children with congenital heart diseases in comparison with healthy ones.

BACKGROUND: Some elements were claimed to play a role in the pathogenesis of congenital heart defects (CHD) and influence the general well-being and health of these children. OBJECTIVES: We aimed to assess the levels of some elements simultaneously in the blood and teeth samples of children with cyanotic and acyanotic CHD compared with healthy children. METHODS: A total of 39 children with CHD (11 with cyanotic and 28 with acyanotic CHD) and 42 age- and sex-adjusted controls were enrolled. Levels of 13 elements, including magnesium, phosphorus, calcium, chromium, manganese, iron, copper, zinc, strontium, cadmium, lead, mercury, and molybdenum, were assessed using inductively coupled plasma mass spectrometry. RESULTS: Children with cyanotic and acyanotic CHD had significantly lower teeth calcium and calcium/phosphorus ratio as compared to the controls after adjusting for confounders. The mean blood iron level was found to be significantly higher in the cyanotic CHD group compared to the other groups. In addition, children with acyanotic CHD had significantly higher teeth copper levels, higher blood molybdenum and lower blood magnesium levels compared to the healthy control group. Blood cadmium and mercury levels were found to be significantly elevated in both the cyanotic and acyanotic CHD groups compared to the healthy control group. There were no differences in toxic metal levels of teeth in cases with CHD. CONCLUSION: Monitoring adequate and balanced gestational micronutrient intake might support not only maternal health but also fetal cardiac development and infant well-being. Supplementation of magnesium should be evaluated in patients having CHD.

Intrapericardial inflammatory myofibroblastic tumour in a 3-month-old infant associated with Coronavirus OC43 presenting with pericardial tamponade.

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. ABBREVIATIONS: 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.

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.

Palivizumab compliance in congenital heart disease patients: factors related to compliance and altered lower respiratory tract infection viruses after palivizumab prophylaxis.

BACKGROUND: Lower respiratory tract infections caused by respiratory syncytial virus can be severe during infancy, which requires admission to the hospital. These infections may be more severe especially in patients with congenital heart disease. Passive immunisation with palivizumab, a monoclonal antibody, is recommended in high-risk infants. We tried to determine the compliance rates, factors affecting compliance, and also other microorganisms responsible for lower respiratory tract infections after palivizumab prophylaxis in these patients. METHODS: We evaluated patients' compliance to prophylaxis with palivizumab in two consecutive respiratory syncytial virus seasons from pharmacy records. We also investigated factors affecting compliance and the frequency of hospitalisations for lower respiratory tract infections. We investigated the causative microorganisms detected in hospitalised patients. RESULTS: In this study, 86.7% of the desired number of injections was achieved in 176 patients in two seasons. Out of these, 117 patients (66.4%) received all the doses they were prescribed. Although not statistically significant, compliance to prophylaxis was higher in male patients, cyanotic patients, those who started under 1 year old, and who lived in the city centre. Human metapneumovirus, parainfluenza type 3, and bocavirus were detected in the hospitalised patients. CONCLUSION: Patients with congenital heart disease can survive the period of infancy with less problem by making palivizumab prophylaxis more effective, and awareness about non- respiratory syncytial virus factors may be a guide for the development of new treatments.

Acute coronary syndrome in an adolescent with aortic root replacement (Bentall).

Acute coronary syndrome is a cause of cardiac-induced chest pain and rarely seen among childhood. It should be kept in mind in patients presenting with typical chest pain with a history of CHD and/or surgery, as this will lead to life-threatening complications and death. We present an adolescent with a history of Bentall operation who had acute coronary syndrome owing to coronary thrombosis as a result of inappropriate drug use.

Assessment of cardiac function and electrocardiographic findings in patients with Wilson's disease.

BACKGROUND: This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson's disease. METHOD: Asymptomatic patients with a diagnosis of Wilson's disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched. RESULTS: The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias. CONCLUSION: Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.

Anomalous single trunk coronary artery from the right pulmonary artery.

Anomalous single coronary artery from pulmonary artery is a very rare congenital heart anomaly. Anomalous single coronary artery from pulmonary artery has high mortality rates and poor surgical outcome despite advanced surgical techniques. We report a 4-month-old infant presented by congestive heart failure findings and diagnosed with anomalous single trunk coronary arteries arising from right pulmonary artery.