Piccolo in transcatheter PDA closure multi-centre study from premature to adolescent children.

In this multi-centre study, the mid- to long-term efficacy and safety of the Amplatzer Piccolo Occluder in patent ductus arteriosus closure in premature and term infants as well as children were discussed. Methods. Between 2016 and 2021, 645 patients, 152 of whom were less than 1 month old, underwent ductus closure with the Piccolo device from five different centres in Turkey. The median age of the patients was 2.2 years, and the mean narrowest point of duct diameter was 1.8 mm. Sixty-two patients weighed ≤ 1.5 kg, 90 patients 1.5-3 kg, and the mean follow-up was 20.4 months. In 396, the duct was closed by the retrograde route. Ductal anatomy was Type A in 285, C in 72, E in 171, and F in 64 patients. Fluoroscopy duration was 6.2 min. The procedure success rate was 99.1%. Device embolisation occurred in 13 patients (2%), and 11 were retrieved with a snare. Cardiac perforation and death developed in one premature baby. The left pulmonary artery and the descending aorta stenosis were observed in 3 (0.4%) and in 5 patients (0.5%). Results. Piccolo device is safe and effective in closing ductus in all age groups. It has low profile for use in premature and newborn babies, a small embolisation risk, and a low residual shunt rate after closure. Conclusion. The Piccolo device can be considered as close an ideal occluder. The lower profile, smaller delivery catheter size, and symmetry of this device allow for a venous or arterial approach.

Ductus arteriosus diameters in fetuses with early- and late-onset fetal growth restriction.

PURPOSE: Fetal growth restriction (FGR) is a common pregnancy complication that can be associated with several adverse perinatal outcomes. One of these negative outcomes is ductus arteriosus, especially in preterm babies. In this study, intrauterine heart function and ductus diameter were evaluated in babies with FGR. METHODS: Thirty-seven fetuses with FGR were compared with 37 normal-weight fetuses at the same gestational week. In our study, ventricular diameters, aorta, pulmonary artery, ductus arteriosus (DA), aortic arch diameter, and flow traces were examined. In addition, the aorta and aortic isthmus diameters were proportioned to the ductus diameter, and the left ventricular myocardial performance index (MPI) [(ICT + IRT)/ET] was evaluated. RESULTS: There was no difference in DA diameters between the patient and control groups. The intragroup comparison of the cases with early- and late-onset FGR revealed no statistically significant difference between DA diameters. However, the ratios of the aortic annulus diameter/ductus diameter (AOD/DAD) and aortic isthmus diameter/ductus diameter (AID/DAD) were significantly lower in early-onset FGR because the diameter of the DA was greater. In addition, the mod-MPI values were higher in the patient group. CONCLUSIONS: In our study, although the ductal diameters did not change significantly in the patient group, the ductal diameter was greater in the early-onset intrauterine growth restriction (FGR) group compared with other cardiac measurements. The mod-MPI value, a cardiac function indicator, was higher in fetuses with FGR. These findings may be useful for evaluating postnatal cardiac functions in FGR.

Cardiac Inflammatory Myofibroblastic Tumor Causing Pulmonary Artery Obstruction: A Rare Case Report.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor of the heart. In the literature, cardiac IMT is often described as an endocardial-based cavitary mass originating from the right side of the heart in infants and adolescents. In this article, we present a 5-year-old boy with a rare cardiac IMT who had no complaints and was diagnosed with murmur during his routine examination. Transthoracic echocardiography showed a homogeneous polypoid mass originating from the pulmonary valve, extending into the main pulmonary artery during systole and causing obstruction of the pulmonary artery and right ventricular outflow tract. Surgical resection of the tumor was performed successfully. There was no tumor recurrence in the control echocardiography at the postoperative first month.

Symptom Frequency in Children with Congenital Heart Disease and Parental Care Burden in Predicting the Quality of Life of Parents in Turkey.

PURPOSE: This study was conducted to investigate the symptom frequency and parental care burden of the children with congenital heart disease in predicting the quality of life of parents in Turkey. DESIGN AND METHOD: The study subjects for the present study included 124 parents whose children were suffering from heart disease. The data for the study were collected from these parents using parent information form, the questionnaire of the frequency of heart disease-related symptoms of the child, the caregiver burden scale, and the quality of life scale. The level by which the heart disease-related symptoms of the child and caregiver burden predicted the quality of life was analyzed using the regression analysis. RESULTS: The most common symptoms in children were found to be anorexia, difficulty in activities, palpitations, shortness of breath, weakness, and fatigue. The number of symptoms observed in children and the care burden of the parents were determined to explain 27.1% of the psychological health sub-dimension, 14.4% of the social relations sub-dimension, and 34.9% of the environment sub-dimension. When the variables were examined separately, the number of symptoms was found to significantly predict only social relations sub-dimensions. CONCLUSION: Increased number of symptoms increased parental care burden. Increased number of symptoms and care burden of parents reduced the quality of life of parents. PRACTICE IMPLICATIONS: Symptom management is one of the important responsibilities of nurses in improving care burden and quality of life for parents of children with heart disease.

Cyclosporin treatment improves skin findings in omenn syndrome.

Omenn syndrome is a combined immunodeficiency characterized by a generalized erythematous skin rash, enlarged lymph nodes, hepatosplenomegaly, severe susceptibility to infections, eosinophilia, and hyperimmunoglobulinemia E. A 3-month-old girl was admitted to our hospital with a history of recurrent sepsis. Physical examination revealed severe erythroderma, hepatosplenomegaly, lymphadenopathy, and failure to thrive. Laboratory findings revealed leukocytosis, lymphocytosis with high CD3 T-cells, a high CD4:CD8 ratio, absence of CD19 B-cells, high eosinophil count, and low immunoglobulin levels. A heterozygote RAG1 gene mutation was found. She had itchy, scaling, ichthyosiform erythroderma and protracted diarrhea. Cyclosporin treatment up to 10 mg/kg effectively resolved erythroderma and lowered total eosinophil counts, and she gained weight during treatment. Since extensive erythroderma with generalized itching causes patient discomfort in Omenn syndrome, cyclosporin treatment can be considered while waiting for treatment with hematopoietic stem cell transplantation.

Transcatheter Patent Ductus Arteriosus Closure in Children With Different Devices and Long-Term Results.

INTRODUCTION: With the development of transcatheter interventional techniques and the introduction of next-generation duct occluder devices, transcatheter closure has become the first treatment option for patent ductus arteriosus (PDA) in pediatric patients. In this study, we compared the effectiveness and safety of different devices for transcatheter PDA closure in pediatric patients, focusing on long-term outcomes. METHODS: A total of 235 patients aged 0-18 years who underwent transcatheter PDA closure at a tertiary care center between January 2005 and February 2020 were included. The medical records of the cases were retrospectively evaluated. RESULTS: The median age of the patients was 2.3 years (range: 3.5 months to 17 years), with a mean weight of 12.8 kg (range: 5.7-43.2 kg). The mean PDA diameter at its narrowest point was 2.9 mm (range: 2.2-5.1 mm). Ductal anatomy was as follows: Type A in 98 (41.7%) patients, Type E in 36 (15.6%) patients, Type C in 32 (13.5%) patients, Type F in 27 (11.4%) patients, Type D in 23 (9.7%) patients, and Type B in 19 (8.1%) patients. Arterial access was used in 138 (57.1%) patients, venous + arterial access in 58 (24.6%) patients, and venous access only in 39 (16.5%) patients. Closure was performed with Amplatzer Duct Occluder (ADO; AGA Medical Corp., Golden Valley, MN, USA) II in 151 (64.2%) cases, ADO I in 43 (18.2%) cases, and coils in 41 (17.4%) cases. The mean fluoroscopy time and mean procedural time were 10.3 ± 4.2 minutes and 41 ± 7.2 minutes, respectively. The mean radiation dose was 1364 ± 497 cGy/min. The early closure rate after the procedure was 92%, while residual shunting on the first day post-procedure was observed in 1.8% of cases, decreasing to 0.1% at the one-month follow-up. The overall procedural success rate for all cases was 96.0%. The mean follow-up duration was 9.7 years (range: 2.9-13.8 years). CONCLUSION: For percutaneous PDA closure, ADO I devices are preferred for larger defects, whereas ADO II devices are prioritized for small- to medium-sized defects instead of coils.

Safety and efficacy of Amplatzer duct occluder II and konar-MF™ VSD occluder in the closure of perimembranous ventricular septal defects in children weighing less than 10†kg.

Introduction: Device closure of perimembranous ventricular septal defects (pmVSD) is a successful off-label treatment alternative. We aim to report and compare the outcomes of pmVSD closure in children weighing less than 10 kg using Amplatzer Duct Occluder II (ADOII) and Konar-MF VSD Occluder (MFO) devices. Methods: Retrospective clinical data review of 52 children with hemodynamically significant pmVSD, and sent for transcatheter closure using ADOII and MFO, between January 2018 and January 2023. Baseline, procedural, and follow-up data were compared according to the implanted device. Results: ADOII devices were implanted in 22 children with a median age of 11 months (IQR, 4.1-14.7) and weight of 7.4 kg (IQR, 2.7-9.7). MFO devices were implanted in 30 children with a median age of 11 months (IQR, 4.8-16.6) and weight of 8 kg (IQR, 4.1-9.6). ADOII were implanted (retrograde, 68.1%) in defects with a median left ventricular diameter of 4.6 mm (IQR, 3.8-5.7) and right ventricular diameter of 3.5 mm (IQR, 3.1-4.9) while MFO were implanted (antegrade, 63.3%) in defects with a median left ventricular diameter of 7 mm (IQR, 5.2-11.3) (p > 0.05) and right ventricular diameter of 5 mm (IQR, 2.0, 3.5-6.2) (p < 0.05). The procedural and fluoroscopy times were shorter with the MFO device (p < 0.05). On a median follow-up of 41.2 months (IQR, 19.7-49.3), valvular insufficiency was not observed. One 13-month-old child (6.3 kg) with ADOII developed a complete atrioventricular heart block (CAVB) six months postoperative and required pacemaker implantation. One 11-month-old child (5.9 kg) with MFO developed a CAVB 3 days postoperative and the device was removed. At 6 months post-procedure, only one child with MFO still experiences a minor residual shunt. There was one arterio-venous fistula that resolved spontaneously. Conclusion: Both the MFO and ADOII are effective closure devices in appropriately selected pmVSDs. CAVB can occur with both devices. The MFO is inherently advantageous for defects larger than 6 mm and subaortic rims smaller than 3 mm. In the literature, our series represents the first study comparing the mid-term outcomes of MFO and ADOII devices in children weighing less than 10 kg.

Four ectopia cordis cases surgically managed with different strategies.

Ectopia cordis is a rare congenital disorder in which the heart is partially or completely located outside the chest cavity. In this article, we present four cases of ectopia cordis accompanied by cardiac abnormalities with either thoracic and/or abdominal placed heart, managed with strategies ranging from follow-up without any intervention to complete surgical closure.

Does Familial Mediterranean Fever Provoke Atherosclerosis in Children? Evaluation of Arterial Stiffness and Serum Endocan Levels.

OBJECTIVES: This study aimed to evaluate the risk for atherosclerosis by using echocardiographic arterial stiffness (AS) parameters and serum endocan levels, as a biomarker of endothelial dysfunction (ED) in children with FMF. METHODS: Seventy-nine children with FMF (12-18 years) and 41 healthy children were included, and clinical features (age at the first attack, age at the time of diagnosis, diagnosis delay time, colchicine dose, biological agent usage, MEFV mutations, and symptoms of attacks) of patients were noted. Arterial stiffness parameters were calculated by using echocardiographic aortic measurements with blood pressure monitoring. Hemogram parameters, acute phase reactants, blood glucose and lipid levels of 12 hours of fasting, and serum endocan levels were evaluated for all participants. RESULTS: There were no statistically significance regarding demographic features, acute phase reactants, and hemogram parameters. Blood glucose and lipid levels were similar, except for HDL (lower in FMF group, p=0.029). Serum endocan levels did not differ in two groups (p=0.906). Only stiffness of descending aorta was lower in FMF group (p=0.028), and the other AS parameters were similar between two groups (p>0.05 for each parameters). CONCLUSION: Good disease control could be preventive for atherosclerosis in children with FMF. On the other hand, screening for cardiovascular diseases is essential, particularly for uncontrolled cases. Distribution of MEFV gene mutations KEY POINTS: • Exaggerated inflammation is the prominent feature of FMF attacks; moreover, it is shown that subclinical inflammation might also continue in attack-free periods. • Chronic inflammation contributes to atherosclerotic process in almost all stages by activating endothelial cells, producing reactive oxygen species, and accelerating foam cell and atherosclerotic plaque formations. • However, the results of this study showed that there was no difference in terms of atherosclerotic markers such as serum endocan levels and arterial stiffness parameters between pediatric FMF patients and healthy peers. • Good disease control in pediatric FMF patients may prevent early atherosclerotic changes during childhood, which then may lead a probable decreased risk of subsequent CVD in adulthood.

Risk factors of intravenous immunoglobulin resistance and coronary arterial lesions in Turkish children with Kawasaki disease.

Kawasaki disease (KD) is the most common cause of childhood coronary artery disease. The incidence of coronary artery lesions (CALs) has declined with the routine use of intravenous immunoglobulin (IVIG) treatment, but there is still considerable risk for resistance to IVIG treatment and development of CALs. The present study was aimed to determine the risk factors in Turkish children with IVIG resistant KD and coronary artery involvement. Clinical, laboratory and echocardiographic data were retrospectively analyzed in 94 Kawasaki patients. IVIG resistant and responsive groups were compared. The IVIG resistant group had a higher rate of CALs compared to the IVIG responsive group (p < 0.05). Duration of fever ≥ 9.5 days, C-reactive protein (CRP) ≥ 88 mg/L and Neutrophil/lymphocyte ratio (NLR) ≥1.69 were the best cutoff values for predicting IVIG resistance before treatment. The criteria for at least two of these three predictors were considered to be statistically significant risk factors for detecting IVIG resistance in KD before treatment (76.47% sensitivity, 71.05% specificity and 95% CI were 50.1-93.19% and 59.51-80.89%, respectively). Based on the clinical and laboratory features, we established a new risk-scoring system for predicting IVIG resistance in a cohort of Turkish children with KD. This may be useful for choosing optimal treatment for KD to prevent coronary artery involvement.