Stent implantation of an unusual morphology patent ductus arteriosus via Glidesheath slender.

The procedure of stenting the patent ductus arteriosus (PDA) is a palliative procedure applied as an alternative to surgery in newborns with ductus-dependent pulmonary circulation. However, it is still a very challenging method in patients with aortic arch anomalies. We describe our experience with a newborn with right atrial isomerism and dextrocardia, complete atrioventricular septal defect, aortic outlet right ventricle with pulmonary atresia, right aortic arch, and a PDA from the left innominate artery. Because the PDA was long and tortuous, we preferred placing three short stents instead of a single long stent. The procedure applied the femoral artery approach with a Glidesheath Slender to decrease arterial injuries. PDA stenting in challenging morphologies can be performed successfully using multiple short stents and via Glidesheath Slenders.

Prevalence of Jervell-Lange Nielsen syndrome in children with congenital bilateral sensorineural hearing loss.

OBJECTIVE: Long QT syndrome (LQTS) is an inherited cardiac ion channel disorder (channelopathy) that is characterized by prolonged QT intervals on the electrocardiography (ECG) and possess the risk of sudden cardiac death (SCD). Jervell-Lange Nielsen syndrome (JLNS) is a specific subtype of LQTS that is accompanied by congenital sensorineural hearing loss, inherited autosomal recessively, and higher risk of SCD. In this study, we aimed to investigate JLNS prevalence in deaf children attending special schools for hearing loss, located in our province. METHODS: An ECG screening program was conducted in 6 special schools for children with hearing loss in Istanbul and a total of 440 students between 6 and 18 years old were included. Corrected QT interval (QTc) was calculated using the Bazett formula. Notably, 51 students, detected with any abnormal finding on ECG, were invited to our center for a comprehensive examination. RESULTS: A total of 8 patients were found with a prolonged QT interval. JLNS was diagnosed in 4 (0.9%) patients. In addition, 2 students had already been diagnosed with JLNS at another center earlier. The other 2 students, being siblings, were newly diagnosed with JLNS; and appropriate treatment was initiated. Genetic testing revealed a pathological homozygous mutation in KCNQ1 gene. The younger sibling (Case 1), who possessed a QTc of greater than 500 ms and a history of syncope, which was very suspicious for SCD, was implanted an implantable cardioverter-defibrillator. Propranolol treatment was initiated for both siblings. CONCLUSION: JLNS should be carefully considered and screened, especially in patients with a history of congenital deafness.

A case of a very large haemorrhagic pericardial effusion in an adolescent patient with COVID-19 infection.

An otherwise healthy 17-year-old adolescent boy presented with upper respiratory tract symptoms and was diagnosed with coronavirus disease 2019 infection. A haemorrhagic pericardial effusion was found in the transthoracic echocardiography. He was treated with pericardiocentesis, ibuprofen, and hydroxychloroquine. A large pericardial effusion may complicate COVID-19 in children and should be considered in acute decompensation.

Evaluation of different lead types and implantation techniques in pediatric populations with permanent pacemakers: Single-center with 10 years' experience.

BACKGROUND: Permanent pacemaker (PM) implantation is performed for various indications and by different techniques in children; however, many problems with lead performance are encountered during follow-up. This study aims to evaluate the possible effects of different lead types and implantation techniques on pacing at early and midterm in children with a permanent PM. PATIENTS AND METHODS: Pediatric patients who underwent permanent PM system implantation at our tertiary cardiac surgery center between January 1, 2010 and January 1, 2020 were evaluated retrospectively. Patients were categorized in the epicardial pacing lead (EP), transvenous pacing lead (TP), and transvenous bipolar lumenless (Select Secure [SS]) lead groups according to the lead implantation technique and lead type with the same manufacturer. Groups were evaluated statistically for demographic features, pacing type and indication for implantation, lead electrical performance, lead failure, complications, and outcome. RESULTS: Over 10 years, 323 lead implantations were performed on 167 patients (96 males, median age 68 months [5 days-18 years]). Of 323 leads, 213 (66%) were EP, 64 (20%) were TP, and 46 (14%) were SS. Of the total, 136 of the leads were implanted in atria, and 187 were implanted in ventricles. Primary pacing indications were postoperative complete atrioventricular (AV) block (n = 95), congenital AV block (n = 71), sinus node dysfunction (n = 13), and acquired complete AV block (n = 1). Additional cardiac diseases were present in 115 patients (69%). No statistically significant difference was observed in gender, syndrome, or pacing indication (P > .05). Atrial and ventricular capture, threshold, sensing, and lead impedance measurements were not significantly different at the initial and follow-up periods (P > .05). The median follow-up duration was 3.3 years (6 months-10 years). Twenty lead failures were determined in 15 patients (EP: 14 lead failures in 10 patients; TP: two lead failures in two patients; and SS: four lead failures in three patients) during follow-up, and no statistically significant difference was found between groups (P = .466). The 5-year lead survival was 98% for TP, 95% for EP, and 90% for SS; the 10-year lead survival was 90% for TP, 70% for EP, and 70% for SS. There was no mortality related to chronic pacing or due to the procedure of implantation. CONCLUSIONS: Despite improvements in technology, lead failure is still one of the most critical problems during these patients' follow-up. Early to midterm lead survival rates of all three lead types were satisfactory.

Munchausen by Proxy Syndrome Associated with Fecal Contamination: A Case Report. / Fekal Kontaminasyon ile Ortaya Çikan Munchausen by Proxy Sendromu: Bir Olgu Sunumu.

Factitious disorder imposed on another, also known as Munchausen Syndrome by Proxy (MSBP), is a serious form of child abuse that is difficult to diagnose. In general, signs and symptoms are fabricated or produced by the mother or the caregiver. Delay in diagnosis may cause serious morbidity and mortality. Here, we present the case of an 18-month-old boy who was admitted to the Paediatric Infection Clinic with a diagnosis of acute gastroenteritis. When on intravenous fluid therapy, he developed high fever and subsequently, polymicrobial growth was determined in his blood. He was later diagnosed with MSBP. Despite being a rare condition, MSBP is a disorder that is often overlooked and may have fatal outcomes. Early diagnosis is very important in this disorder, which is considerably difficult to diagnose. In suspected cases, interdisciplinary team work is necessary to prevent adverse consequences.

Ultrasonographic postoperative evaluation of diaphragm function of patients with congenital heart defects.

BACKGROUND: This study aims to investigate the role of ultrasonography in the postoperative evaluation of diaphragm function in patients with congenital heart defect. METHODS: This prospective study included a total of 360 patients (176 males, 184 females; mean age 2 years; range, 1 month to 8 years) who underwent congenital heart surgery and 44 patients (22 males, 22 females; mean age 1 years; range, 1 month to 4 years) who underwent diaphragm ultrasonography between September 2018 and March 2019. Ultrasonography was performed for the patients who had difficulty in weaning from mechanical ventilation or who were thought to have diaphragm dysfunction due to pathological findings on postoperative chest X-rays. The findings were interpreted as normal, paresis, or paralysis. RESULTS: Diaphragm dysfunction was demonstrated in 23 patients (6.3%), paralysis in 11 patients (3%), and paresis in 12 patients (3.3%). A median sternotomy was performed in 21 patients (91%), and seven of them (30%) were redo cases. Five patients (21%) had single ventricle physiology. Six patients (1.6%) needed an intervention due to diaphragm dysfunction. The interventional procedures were diaphragm plication in three patients (0.8%) and tracheotomy in three patients (0.8%). Three of these patients had a single ventricle and three had biventricular physiology. The median time after surgery for these procedures was 36 days. One patient (0.2%) died in the intensive care unit. The mean length of stay in the intensive care unit and hospital was 36±12 and 48±21 days, respectively. CONCLUSION: Diaphragm dysfunction should be kept in mind in patients undergoing congenital heart surgery and in those who need prolonged intubation during the postoperative period. Ultrasonography is a non-invasive diagnostic tool which can be used to identify diaphragm dysfunction and the best course of management of this clinical condition.