Evaluation of the reduction of radiation dose received by pediatric patients in new-generation biplane angiocardiography: Randomized controlled study.

Objective: We aimed to evaluate the safety and efficacy of radiation dose reduction with a new-generation biplane angiocardiography system in patients undergoing transcatheter isolated patent ductus arteriosus (PDA) closure. Materials and methods: Fifty pediatric patients who underwent transcatheter PDA closure were randomly divided into two groups as normal radiation dose and low dose. Patients who required additional procedures other than PDA closure were excluded. PDA closure was performed according to the angiographic measurement of the defect. After the procedure, age, weight, sex, PDA measurements, and radiation measurements such as dose-area product (DAP, Gy.cm2) and air kerma (AK, mGy) were compared between the groups. Results: There was no statistically significant difference between the groups in age, sex, weight, PDA diameter, PDA type, device used, and device diameter (p > 0.05). While there was no statistically significant difference between the groups in terms of cine recording, number of recorded images, and fluoroscopy time (p > 0.05), there was a statistically significant difference between the total DAP, cine and fluoroscopy DAP, total AK, frontal and lateral tube AK, and DAP/kg (mGy.m2/kg) measurements (p < 0.05). Conclusion: Transcatheter PDA closure with a low radiation dose is as effective as that with a normal radiation dose. The radiation dose received by the patient during the procedure was significantly reduced. With the vision provided by this study, it seems possible to work with a low radiation dose in other groups of patients.

Percutaneous Closure of Hemodynamically Significant Postoperative Residual Ventricular Septal Defects.

The experience with percutaneous closure of postoperative residual ventricular septal defects (VSDs) is expanding with improved device technology and techniques. To report our experience with percutaneous closure of residual VSDs after cardiac surgeries. Retrospective clinical data review of patients who had percutaneous closure of postoperative residual VSDs at our institution between 2010 and 2022. Patients' demographics, procedural, and follow-up data were looked at. Twelve patients (50% males) with a median age of 9.2 years (range 0.9-22) were identified. Baseline surgeries were 8 tetralogy of Fallot corrections, 2 pulmonary bandings for large muscular VSD (mVSD) including 1 coarctation repair, 1 atrioventricular septal defect repair, 1 sub-aortic membrane resection-induced iatrogenic VSD, 1 isolated VSD closure, and 1 additional mVSD. Median duration between baseline surgery and percutaneous VSD closure was 2.2 years (range 0.2-8.3). Residual VSD shunting was secondary to surgical patch leakage in 8/12 patients. The median angiographic defect diameter was 6.8 mm (range 4.8-14). The defect was balloon-calibrated in 3/12 patients. Defects were tackled retrogradely in 3/9 patients. Amplatzer Membranous VSD occluder (n = 1), Lifetech Multifunctional (n = 5), Membranous (n = 1) and muscular VSD occluders (n = 2) and Occlutech Membranous (n = 1) and Muscular (n = 2) VSD occluders were used. The procedure was successful in 10/12 patients. Two devices embolized to the pulmonary artery and were snare-retrieved. Both patients were referred for surgery. The median follow-up was 1.3 years (range 0.1-12). Six-month ultrasound showed one trivial residual shunt and one mild right ventricular outflow obstruction. One patient is receiving targeted therapy for pulmonary hypertension at 2 years of follow-up. Transcatheter closure of postoperative residual VSDs is a feasible yet challenging intervention. Procedural complications can be encountered.

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.

Evaluation of the relationship between atherosclerosis and Helicobacter pylori infection with measurement of growth differentiation factor 15 and atherosclerosis indicators in adults with no comorbidity.

BACKGROUND: The aim of this study was to investigate presence of subclinical atherosclerosis by measuring carotid intima-media thickness (CIMT) in patients with Helicobacter pylori (HP) and to assess effects of HP on atherosclerosis by evaluating markers of atherosclerosis and blood growth differentiation factor (GDF-15) levels. MATERIALS AND METHODS: This cross-sectional study included 59 patients without comorbid disease who had HP and 30 healthy controls without HP in upper endoscopic biopsy. In order to assess atherosclerosis, the CIMT measurement was performed by sonography. Serum GDF-15 level was measured by ELISA method. In all patients, atherosclerosis markers were recorded. Atherogenic indices were calculated, including Castelli risk index I and II (TG/HDL-c and LDL-c/HDL-c, respectively), plasma atherogenic index (PAI; log TG/HDL-c), non-HDL-c (TH-HDL-c) and atherogenic coefficient (AC; non-HDL-HDL-c). RESULTS: The GDF-15 level and CIMT were significantly higher in HP-positive group when compared to HP-negative group (p≤0.001). There was a significant correlation between serum GDF-15 level and CIMT (r=0.445; p≤0.001). There was no correlation between other atherosclerosis markers and serum GDF-15 level or CIMT. The bacterial intensity on endoscopic specimen was only correlated with CIMT (p<0.001). Vitamin B12 and D levels were comparable among groups. CONCLUSION: This study suggested that there was a correlation between GDF-15 level and subclinical atherosclerosis development in patients with HP. However, GDF-15 level, which was found to be elevated while atherogenic indices were normal, can be an earlier marker for subclinical atherosclerosis.

One-Year Follow-Up Results of MIS-C Patients with Coronary Artery Involvement: A Multi-center Study.

Multisystem inflammatory syndrome (MIS-C) in children is a rare complication of SARS-CoV-2 infection. Knowing the course of the affected or unaffected coronary arteries in the patients under follow-up is important in terms of defining the long-term prognosis of the disease and determining the follow-up plan. This is a multicenter and retrospective study. The data were obtained from nine different centers. Between May 2020 and August 2022, 68 of 790 patients had coronary artery involvement. One-year echocardiographic data of 67 of 789 MIS-C patients with coronary artery involvement were analyzed. Existing pathologies of the coronary arteries were grouped as increased echogenicity, dilatation and aneurysm according to Z scores, and their changes over a 1-year period were determined. The data of all three groups are defined as frequency. SPSS Statistics version 22 was used to evaluate the data. In our study, aneurysm was observed in 16.4%, dilatation in 68.7% and increased echogenicity in 13.4% of the patients. All of the patients with involvement in the form of increased echogenicity recovered without sequelae by the end of the first month. No progression to aneurysm was observed in any of the patients with dilatation. No new-onset involvement was observed in patients with previously healthy coronary arteries during the convalescent period. In addition, from the sixth month follow-up period, there was no worsening in the amount of dilatation in any of the patients. At least 94% of the patients who completed the 12th month control period returned to normal.

Deciphering the host genetic factors conferring susceptibility to severe COVID-19 using exome sequencing.

The COVID-19 pandemic remains a significant public health concern despite the new vaccines and therapeutics. The clinical course of acute SARS-CoV-2 infection is highly variable and influenced by several factors related to the virus and the host. Numerous genetic studies, including candidate gene, exome, and genome sequencing studies, genome-wide association studies, and other omics efforts, have proposed various Mendelian and non-Mendelian associations with COVID-19 course. In this study, we conducted whole-exome sequencing on 90 unvaccinated patients from Turkey with no known comorbidities associated with severe COVID-19. Of these patients, 30 had severe, 30 had moderate, and 30 had mild/asymptomatic disease. We identified rare variants in genes associated with SARS-CoV-2 susceptibility and pathogenesis, with an emphasis on genes related to the regulation of inflammation, and discussed these in the context of the clinical course of the patients. In addition, we compared the frequencies of common variants between each group. Even though no variant remained statistically significant after correction for multiple testing, we observed that certain previously associated genes and variants showed significant associations before correction. Our study contributes to the existing literature regarding the genetic susceptibility to SARS-CoV-2. Future studies would be beneficial characterizing the host genetic properties in different populations.

Osteopontin as an early predictor of atherosclerosis in attack-free Familial Mediterranean fever patients.

Familial Mediterranean fever (FMF) is an autoinflammatory disease that is associated with endothelial dysfunction and atherosclerosis. Osteopontin which is a multifunctional protein involved in the modulation of inflammatory processes may contribute to the development of atherosclerosis in FMF patients. Therefore, this cross-sectional study investigated the relationship of osteopontin with carotid intima media thickness (CIMT) and atherogenic indices in patients with FMF. Serum osteopontin levels, CIMT, Castelli risk index I and II, plasma atherogenic index (PAI), non - high-density lipoprotein cholesterol, and atherogenic coefficient (AC) in 64 attack-free FMF patients were compared with levels in 23 healthy control subjects. The serum osteopontin level, CIMT, Castelli risk index I, AC and PAI were significantly higher, and high-density lipoprotein cholesterol was significantly lower in FMF patients (P < .001, P < .001, P = .045, P = .016, P = .045, and P = .024; respectively). There were significant positive correlations between osteopontin and CIMT, PAI, AC, and Castelli risk index I (R = 0.580, R = 0.259, R = 0.233, R = 0.277; respectively) and there was significant negative correlation between osteopontin and high-density lipoprotein cholesterol (r= -0.309). Patients who had homozygote mutations had significantly higher osteopontin, PAI, Castelli risk index I and II level. The current study is the first to demonstrate significantly increased serum osteopontin levels in attack-free FMF patients compared with healthy controls. It was also associated with CIMT and many atherogenic indices. This finding provides a new experimental basis to understand the pathogenesis of inflammation-induced atherosclerosis in FMF patients. Furthermore, patients who had homozygote mutations had worse atherogenic indices than those with heterozygote mutations.

Unknown origin of acute myocardial infarction and lifesaving percutaneous coronary intervention in a 10-year-old girl.

Myocardial infarction is extremely rare in children. However, chest pain suspected to be cardiac in origin by the parents is a very common presenting symptom in pediatric emergency services. In this case, an electrocardiogram is very important in the differential diagnosis of cardiogenic complaints and should be carefully considered.

Iatrogenic perforation of atrial appendage and successful closure with Amplatzer Piccolo Occluder, in a 1-year-old patient.

We present the successful transcatheter closure of the perforation of the cardiac wall from atrial appendage in a 1-year-old girl. Although open-heart surgical repair has been the primary option, percutaneous device closure should always be considered before surgery.

Consensus Guidelines for the Prevention and Management of Periprocedural Complications of Transcatheter Patent Ductus Arteriosus Closure with the Amplatzer Piccolo Occluder in Extremely Low Birth Weight Infants.

Transcatheter closure of patent ductus arteriosus (PDA) in premature infants is a feasible, safe, and an effective alternative to surgical ligation and may be performed with an implant success rate of 97%. Major procedural complications related to transcatheter PDA closure in extremely low birth weight (ELBW) infants are relatively infrequent (< 3%) ,but may be associated with a fatality if not optimally managed. Operators performing transcatheter PDA closures should be knowledgeable about these potential complications and management options. Prompt recognition and treatment are often necessary to avoid serious consequences. With strict guidelines on operator training, proctoring requirements, and technical refinements, transcatheter PDA closure in ELBW infants can be performed safely with low complication rates. This article summarizes the consensus guidelines put forward by a panel of physicians for the prevention and management of periprocedural complications of transcatheter PDA closure with the Amplatzer Piccolo Occluder in ELBW infants.

The Effect of Transcatheter Atrial Septal Defect Closure on Left Heart Function in Pediatric Patients.

OBJECTIVE: The aim of this study was to use tissue Doppler imaging to evaluate the left atrial systolic and the left ventricular (LV) diastolic function as well as the left atrial ejection force in children who underwent transcatheter closure of a secundum atrial septal defect (ASD). METHODS: Tissue Doppler measurements of the left atrial ejection force, the mitral valve, and left atrial volume were performed before the ASD closure procedure, and on the 1st day, 10th day, and 1st and 3rd months after the procedure in 56 patients and in 28 healthy controls. RESULTS: There was a significant decrease in the septal and lateral a' velocities on the first day (p<0.05). There was a statistically significant increase in the septal e'/a' parameters at the third month compared with the initial measurements. The left atrial ejection force was lower in patients with an ASD than in the healthy group (10.69±4.94 kdyn, 12.31±4.05 kdyn, respectively), but there was no significant difference (p=0.053). The left atrial ejection force was significantly greater in the patient group 3 months after the procedure, and there was no significant difference compared with the control group. CONCLUSION: Improvement in the LV diastolic and left atrial systolic functions was observed in children who underwent transcatheter closure of an ASD. There was no negative effect related to the devices used.

Use of Lifetech™ Konar-MF, a device for both perimembranous and muscular ventricular septal defects: A multicentre study.

BACKGROUND: The objective of this study was to evaluate the safety and efficacy of transcatheter closure of ventricular septal defects (VSD) using the LifeTech™ multifunctional occluder device (MF-Konar). METHODS: Clinical features and demographic characteristics and follow-up findings were evaluated retrospectively from three centers. RESULTS: MF-Konar was used in 98 patients. The median age and weight of the patients were 3.8 years (range 5.4 months-50 years) and 15.3 kg (range 5.5-80 kg), respectively. The mean fluoroscopy time was 13.7 ± 8.2 min (range 3.4-42.6 min). Procedural success was obtained for 96 out of 98 patients (98%). In 54 out of 98 patients, closure was performed via the antegrade route. Major complications occurred in four patients (embolization in two, complete heart block in one, and device dislocation needing surgical treatment in one). All of the complications were treated successfully, and there was no mortality. Mild residual flow in eight patients (8%), new onset tricuspid valve insufficiency in one (moderate), and new onset aortic valve insufficiency in one (mild) were observed during a mean follow-up duration of 224 ± 149 (10-515) days. Minor rhythm disturbances were observed in eight patients. CONCLUSIONS: Transcatheter closure of VSDs in selected patients using the LifeTech MF-Konar device seems effective. Its advantages are softer design, use of both an antegrade and retrograde approach, and an advanced smaller delivery system. Increasing the number of usage and the experience will provide more accurate data and low complication rates.

Percutaneous ASD closure of children weighing less than 10 kg.

Background: Traditionally the procedure of percutaneous ASD closure is carried out in children weighing more than 15 kg. The aim of this study was to discuss the success, efficacy and safety of the percutaneous closure of symptomatic ASD in children weighing less than 10 kg.Material and methods: This study was performed in two centres. A total of 44 patients were included. Demographic and angiographic data of these patients were gathered retrospectively from patients' records. Main indications for ASD closure were: failure to thrive, recurrent respiratory infections, bronchopulmonary dysplasia and genetic syndromes.Results: Median weight of patients was 9.0(8.12-9.50) kg. Bodyweight of 22 patients was less than 3 percentiles. In the follow-up, this number was lowered to 9 patients at 12 months. Median age of patients was 18.0(12.0-285) months. Minimum age and weight of patients was 3 months and 4.5 kg, respectively. Median mean pulmonary pressure was 24.0(20.0-29.5). The values of median defect size were measured in Cath lab as 13.0(10.75-15.3) mm. Median device size was 13(9-15) mm. Defect size was evaluated according to body weight and body surface area. The ratio of weight per defect size was 0.65(0.54-0.84) also the ratio of body surface area per defect size was 0.032(0.028-0.04). The ratio of total septum per device diameter was 2.5(2.1-3.1). Types of devices used were Amplatzer Septal Occluder, Cera Flex Septal Occluder, Figulla Flex II Atrial Septal Occluder, Memopart Septal Occluder. All cases were closed successfully, but the device had to be retrieved in one patient after successful positioning because it was detected that device compressed the aorta. No major complications were seen.Conclusion: In the experienced centres, percutaneous ASD closure can be done effectively and safely in symptomatic children weighing less than 10 kg.

Transcatheter Treatment of a Symptomatic Giant Iliac Artery Aneurysm with a BeGraft Peripheral Stent in a 2-Year-Old Child.

A 2-year-old girl presented with severe abdominal pain due to a giant iliac artery aneurysm. We embolized the internal iliac artery with microcoils and then eliminated the aneurysmal sac using a BeGraft peripheral stent without any complications. To the best of our knowledge, there are no previous reports of a transcatheter giant iliac artery intervention.

Serum prolidase level in patients with brucellosis and its possible relationship with pathogenesis of the disease: a prospective observational study

Background/aim: Changes in collagen metabolism and fibroblastic activity may play a role in the pathogenesis of brucellosis. The prolidase enzyme plays an important role in collagen synthesis. We aimed to investigate the association of prolidase levels with brucellosis. Materials and methods: Serum prolidase levels in 20 patients newly diagnosed with brucellosis were compared with levels in 30 healthy control subjects. Patients with brucellosis were reassessed 3 months later for prolidase, other laboratory measurements, and response to treatment. Results: The levels of serum prolidase were significantly higher in brucellosis patients compared with those of healthy controls. Prolidase, sedimentation, and C-reactive protein levels were significantly lower after antibrucellosistreatment than before treatment. Conclusion: The current study is the first to demonstrate significantly increased serum prolidase levels in patients with brucellosis compared with healthy controls. Prolidase levels also significantly decreased with antibrucellosis treatment. This finding provides a new experimental basis to understand the pathogenesis of brucellosis in relation to collagen metabolism. The increase in serum prolidase levels might be related to several factors such as tissue destruction, increased fibroblastic activity, and granuloma formation, all of which are involved in the natural history of brucellosis.

Giant aortic aneurysm due to fibulin- 4 deficiency: case series.

Cutis laxa tip1b is a rare autosomal recessive disorder caused by FBLN 4 mutation and primarily characterized by vascular anomalies. Herein, we present five patients who are the members of the same family. The primary cardiac findings of these patients were giant aortic aneurysms. One 2.5-year-old patient with a massive aneurysm of the ascending aorta died as a result of compression to the heart chambers, trachea, and bronchi. The bentall procedure was performed in three of our patients who are under follow-up. One patient is still under clinical follow-up without surgery. After the diagnosis of the first patient, a genetic study was performed in which FBLN 4 mutations were investigated. Four new patients were detected during genetic screening of the family. Other 29 family members were screened bur were negative in physical examinations and echocardiography. Pedigree is important for early diagnosis of genetic diseases in asymptomatic individuals.

"Cutis laxa" tip1B ender görülen FBLN 4 mutasyonunun neden oldugu, ön planda damar anomalileri ile seyreden otozomal çekinik bir hastaliktir. Burada ayni ailenin üyesi olan bes olgumuzu sunduk. Olgularimizin asil kalp bulgusu çikan aort anevrizmasi idi. Bir olgumuz 2,5 yasinda çikan aorttaki dev anevrizmanin trakea, bronslar ve kalp odaciklarina basisi nedeni ile kaybedildi. Üç olgumuza Bentall operasyonu yapildi ve izlemleri devam etmektedir. Bir olgumuz halen klinik olarak izlemdedir. Ilk olgunun tani almasindan sonra genetik çalismasi yapildi ve FBLN 4 mutasyonu saptandi. Aile taramasi sirasinda belirtisiz dört olgu daha saptandi. Yogun akraba evliliklerinin oldugu ailenin taranabilen 29 üyesinde fizik baki ve ekokardiyografik incelemelerde sorun saptanmadi. Aile agacinin çikarilmasi, belirtisi olmayan bireylerde genetik hastaliklarin erken tanisi açisindan önemlidir.