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.

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.

Array comparative genomic hybridisation results of non-syndromic children with the conotruncal heart anomaly.

The study aimed to show the chromosomal copy number variations responsible for the aetiology in patients with isolated conotruncal heart anomaly by array comparative genomic hybridisation and identify candidate genes causing conotruncal heart disease. A total of 37 patients, 17 male, and 20 female, with isolated conotruncal heart anomalies, were included in the study. No findings indicated any syndrome in terms of dysmorphology in the patients. RESULTS: Copy number variations were detected in the array comparative genomic hybridisation analysis of five (13.5%) of 37 patients included in the study. Three candidate genes (PRDM16, HIST1H1E, GJA5) found in these deletion and duplication regions may be associated with the conotruncal cardiac anomaly. CONCLUSION: CHDs can be encountered as the first and sometimes the single finding of many genetic disorders in children. It is thought that genetic tests, especially array comparative genomic hybridisation, may be beneficial for children with CHD since the diagnosis of genetic diseases in these patients as early as possible will help to prevent or reduce complications that may develop in the future. Also, it would be possible to detect candidate genes responsible for conotruncal cardiac anomalies with array comparative genomic hybridisation.

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.

Interventions Involving the Use of Covered Coronary Artery Stents for Pseudoaneurysms of Blalock-Taussig Shunts.

One of the most rare and dangerous complications of a modified Blalock-Taussig shunt involves the formation of a pseudoaneurysm. A pseudoaneurysm may rupture or may result in other adverse events including compression of mediastinal structures or collapse of the underlying lung parenchyma. Shunt-related pseudoaneurysm may be associated with shunt occlusion, bacteremia, or pulmonary hemorrhage. We describe two cases complicated by large pseudoaneurysm formation, following systemic-to-pulmonary artery shunt operations. The patients in question had recurrent pulmonary hemorrhage. Both underwent successful placement of covered coronary artery stents.

Holy Grail Deformity of Distal Disc Hubless Atrial Septal Occluders in Pediatric Hearts.

A special deformity associated with the use of left atrial disc hubless atrial septal occluders has been described in 3 children. This chalice-shaped deformity that occurred during the use of Occlutech and Lifetech septal occluders was given the name "Holy Grail Configuration". Despite being easily reversible, reduction of the metal load on the left atrium has been suggested to have some negative effects.

Transcatheter Removal of Embolized Port Catheters from the Hearts of Two Children.

Embolization of a port catheter is a dangerous and serious complication. In this paper, we present two cases of children, aged 4.5 months and 6 years, in whom port catheters had embolized to the right ventricle one month and 1.5 years priorly, respectively; the port catheters were retrieved via snaring.

Transcatheter closure of patent ductus arteriosus in under 6 kg and premature infants.

BACKGROUND: Transcatheter closure of a patent ductus arteriosus (PDA) has always been considered risky for infants weighing <6 kg and preterms. We present our findings regarding transcatheter closures of PDA. METHODS: The inclusion criteria were a weight of <6 kg and the presence of PDA symptoms. The study subjects were divided into two groups: <6 kg and premature infants. RESULTS: A total of 69 infants were included. The mean ages and weights of the <6 kg and the preterms were 5.4 ± 2.7 months and 30.3 ± 19.9 days, and 4.6 ± 0.8 and 1.7 ± 0.3 kg, respectively. Type C PDAs were most frequently observed in the premature group, and type A was in <6 kg. Sixteen of the patients were premature infants, and 81.2% of them had an extremely low birth weight. All of the premature infants had comorbidities, and had been receiving respiratory support therapy. Transcatheter closure was successfully completed in 81.2% of the premature infants and 94.3% of the <6-kg infants. Major complications occurred in 4 patients (one death and three device embolizations). The patient's age was found to be the main risk factor. The most frequently used device was the Amplatzer duct occluder II in additional sizes (84.6%) in the preterms and the Amplatzer duct occluder I (34%) and II (34%) in the <6-kg group. CONCLUSION: The transcatheter closure of PDA is relatively safe and effective in preterms and in infants <6 kg. The selection of a suitable device based on the type of PDA is critical to the success of the procedure.

Bilateral Ductal Stenting for Discontinuity of the Pulmonary Artery via the Femoral and Carotid Arteries in an Infant.

Bilateral ductal stenting should be performed in cases of discontinuity of the pulmonary branches and pulmonary atresia. Performing this procedure via the carotid artery in small infants can be very difficult and challenging. We present a case of bilateral ductal stenting via both the femoral and carotid arteries in a little child with tetralogy of Fallot with pulmonary atresia and a nonconfluent pulmonary artery and bilateral ductus arteriosus.

Evaluation of hearing loss in pediatric celiac patients.

OBJECTIVES: Celiac disease (CD) is a chronic immune-mediated enteropathy caused by the ingestion of gluten in genetically predisposed individuals. In some reports, sensorineural hearing loss (SNHL) has been identified as an extraintestinal symptom of CD. We aimed to further investigate the possible association between CD and SNHL by examining a greater number of pediatric CD patients. METHODS: The study was carried out from March to September 2014 and included 110 pediatric patients with biopsy-confirmed CD (220 ears) and 41 age- and sex-matched controls (82 ears); participants were evaluated by tympanometry and pure tone audiometry (frequency, 250-8000Hz frequency). RESULTS: Audiometric bone conduction thresholds were significantly different between the CD patients and the controls (p<0.05), but there were no significant differences in pure tone averages for air conduction (p>0.05). When the results for CD patients were analyzed according to duration of disease (≤36 months and >36 months), a significant difference in bone conduction thresholds (p<0.05) was noted, with significant increments at the later stages of disease. However, this difference was not sufficient to define clinical hearing loss, as the pure tone average thresholds remained below 20dB. CONCLUSION: These results indicate that subclinical hearing loss may be present in children with CD, which could presage more serious hearing impairments at older ages and later stages of the disease. Hearing screenings should be recommended for children with CD in order to prevent the potentially unfavorable effects of hearing loss on the emotional, behavioral, cognitive, and sensorimotor development of these patients.

Comparison of the efficacy of different-sized Amplatzer duct occluders (I, II, and II AS) in children weighing less than 10 kg.

The transcatheter closure of patent ductus arteriosus (PDA) may cause more complications in small children. Amplatzer (St. Jude Medical, Plymouth, MN) has produces three types of devices for ductal occlusion: the Amplatzer duct occluder I (ADO I) and II (ADO II) and the recently introduced ADO II additional sizes (ADO II AS). We performed this study to determine the efficacy and complication rates in children who weigh <10 kg for the three types of devices used in our clinic. Between February 2007 and March 2012, 77 patients weighing <10 kg had their PDAs occluded with ADOs. The mean age of the patients was 0.76 ± 0.44 years (range 17 days-2 years), and their mean weight was 6.73 ± 2.05 (range 1.2-9.9) kg. In total, 54 girls (70.1 %) and 23 boys (29.9 %) with a mean pulmonary ductus diameter of 2.55 ± 1.0 (1.08-5.94) mm were included in the study. The ADO I was used in 26 patients (33.8 %); the ADO II was used in 43 patients (55.8 %); and the ADO II AS was used in 8 patients (10.4 %). The mean ages of patients with the ADO I, ADO II, and ADO II AS were 1.07 ± 0.48, 0.66 ± 0.31, and 0.28 ± 0.17 years (p < 0.05), respectively. Their mean weights were 7.86 ± 1.45, 6.50 ± 1.85, and 4.36 ± 2.49 kg (p < 0.05), respectively. Their mean narrowest ductal diameters were 3.11 ± 0.96, 2.25 ± 1.06, and 2.33 ± 1.01 mm (p < 0.05), respectively. The use of the ADO II and ADO II AS was found to be more common in type C defects. One patient with the ADO I and 5 patients with the ADO II (7.8 %) developed varying degrees of left pulmonary artery stenosis or iatrogenic aortic coarctation. In 1 patient, the ADO II AS was replaced with the ADO II due to a significant residual shunt observed during the procedure. Each of the ADOs has its own advantages and disadvantages. Although the ADO I is convenient for medium- and large-sized defects, the ADO II and ADO II AS can be used both anterogradely and retrogradely. The ADO II AS is safe and efficient to use in small infants.

Midazolam or diazepam administration during electroencephalography helps to diagnose subacute sclerosing panencephalitis (SSPE).

Although diagnostic contribution of intravenous diazepam administration during electroencephalography (EEG) recording in subacute sclerosing panencephalitis has been known, no another drug with less potential side effects has been studied in this procedure. In this study, diazepam is compared with midazolam in 25 subacute sclerosing panencephalitis-diagnosed children and 10 children with subacute sclerosing panencephalitis-suggesting symptoms, normal EEG findings and no certain diagnosis. Neither midazolam nor diazepam affected typical periodic slow-wave complexes. However, in the patients with atypical EEG abnormalities, midazolam, like diazepam, attenuated sharp or sharp-and-slow waves, and therefore made the identification of periodic slow-wave paroxysms easier. In the patients with normal EEGs, both midazolam and diazepam revealed typical periodic complexes on EEG recording in the same 3 patients. Cerebrospinal fluid examination verified the diagnosis of subacute sclerosing panencephalitis. The findings suggest that midazolam or diazepam administration increases the contribution of EEG recording in atypical cases with subacute sclerosing panencephalitis.