Search

Objective: Aortopulmonary window (APW) is an uncommon congenital cardiac abnormality marked by a septation defect between the ascending aorta and pulmonary artery. This study aimed to define the clinical characteristics, diagnostic features, treatment strategies, and follow-up outcomes of pediatric patients diagnosed with APW. Material and Methods: We retrospectively reviewed children diagnosed with APW from 2010 to 2023. Morphological APW typing of our patients was based on the classification that is settled by the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database Committee. The patients' demographic data, symptoms at admission, transthoracic echocardiography, cardiac computed tomography, management modalities, and follow-up data were evaluated. Results: Twenty-five children were diagnosed with APW over the study period. Thirteen patients were male (52%), and the median age at presentation of the patients was three months (8 days-7.5 years). Two patients were diagnosed with coronary fistula by echocardiography at the first admission and were diagnosed with APW after catheterization. APW was detected in one patient while being operated on for large ventricular septal defect. According to the STS classification, 32% (n=8) of the patients were type III, 32% (n=8) were type I, 16% (n=4) were intermediate type, 12% (n=3) were type II, and 4% (n=1) were APW with aortic interruption. Associated cardiovascular malformations were in 76% (n=19) of the patients. Fifteen patients (60%) underwent surgery. Transcatheter closure of APW was performed in four patients (16%). Conclusion: Detection of the APW requires careful and systematic investigation. Transcatheter closure can be performed in selected cases where the defect is suitable. Although rare, this defect, which can cause severe left-right shunting, should be kept in mind as a cause of pulmonary hypertension and unexplained cardiac dilation and should be investigated in patients whose cause cannot be determined. [ABSTRACT FROM AUTHOR]

Copyright of Ümraniye Pediatri Dergisi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article reports that the isolated congenital complete atrioventricular block is an uncommon disease linked to SSA/Ro and SSB/La autoantibodies that come from the mother. Topics include examines several patients are diagnosed prenatally or in the first month have a congenital heart condition and the diagnosis is considered to be childhood CAVB.

Purpose: This study was planned to assess the application of three-dimensional (3D) cardiac modeling in preoperative evaluation for complex congenital heart surgeries.Methods: From July 2015 to September 2019, 18 children diagnosed with complex congenital heart diseases (CHDs) were enrolled in this study (double outlet right ventricle in nine patients, complex types of transposition of the great arteries in six patients, congenitally corrected transposition of the great arteries in two patients, and univentricular heart in one patient). The patients' age ranged from 7 months to 19 years (median age, 14 months). Before the operation, 3D patient-specific cardiac models were created based on computed tomography (CT) data. Using each patient's data, a virtual computer model (3D mesh) and stereolithographic (SLA) file that would be printed as a 3D model were generated. These 3D cardiac models were used to gather additional data about cardiac anatomy for presurgical decision-making.Results: All 18 patients successfully underwent surgeries, and there were no mortalities. The 3D patient-specific cardiac models led to a change from the initial surgical plans in 6 of 18 cases (33%), and biventricular repair was considered feasible. Moreover, the models helped to modify the planned biventricular repair in five cases, for left ventricular outflow tract obstruction removal and ventricular septal defect enlargement. 3D cardiac models enable pediatric cardiologists to better understand the spatial relationships between the ventricular septal defect and great vessels, and they help surgeons identify risk structures more clearly for detailed planning of surgery. There was a strong correlation between the models of the patients and the anatomy encountered during the operation.Conclusion: 3D cardiac models accurately reveal the patient's anatomy in detail and are therefore beneficial for planning surgery in patients with complex intracardiac anatomy. [ABSTRACT FROM AUTHOR]

Objective: Long QT syndrome (LQTS) is an inherited primary arrhythmia syndrome associated with life-threatening ventricular arrhythmias and sudden death. This study aimed to report the clinical and genetic characteristics and outcomes of children diagnosed as having LQTS in a tertiary pediatric cardiology center in Turkey. Methods: This was a retrospective review of pediatric patients diagnosed as having LQTS at our center from January 2011 to April 2020. Results: A total of 145 patients (76 males) were included, with a mean age of 9.2±4.5 years and a mean weight of 35.7±18.5 kg; 38 (26.2%) were identified as having LQTS during family screening, whereas a significant proportion of patients were asymptomatic at presentation, 15 patients (10.3%) were diagnosed after previous cardiac arrest, and 26 patients (18%) had syncope. The mean Schwartz score was 4.5 points (range, 3-7.5 points). Furthermore, 107 patients (82%) were confirmed to have a pathogenic mutation for LQTS genes. All patients received beta-blockers. Implantable cardioverter-defibrillator insertion was performed in 34 patients (23.4%). Left or bilateral cardiac sympathetic denervation was performed in 9 patients (6.2%). Median follow-up time was 35.6±25.8 months. Five (3.4%) patients died during the follow-up. Statistical analyses of risk factors for major cardiac events revealed that the QTc was >500 ms and that T wave alternans, high Schwartz score, and Jervell and Lange-Nielsen syndrome were strong and significant predictors of cardiac events. Conclusion: LQTS has a variety of clinical manifestations. Patients' symptoms ranged between asymptomatic and sudden cardiac death (SCD). By raising the awareness of physicians regarding the disease, SCD might be prevented in the early period. [ABSTRACT FROM AUTHOR]

The main aim is to improve the outcomes of extracorporeal life support (ECLS) systems and cardiopulmonary bypass (CPB) in pediatric patients. For priming of the CPB circuit, they preferred blood (containing erythrocyte suspension and fresh frozen plasma) prime solutions (if the patient's weight is greater than 15 kg, then they preferred prime solutions without blood products). Patient selection is still an uncertain area, and further studies should be done according to Yildiz et al.[3] ECMO should be started in patients when conventional treatments fail, before the onset of multiple organ failure, and for potentially reversible conditions. Patients were unable to successfully terminate CPB; postoperative low cardiac output syndrome, postoperative cardiac arrest, and respiratory failure were the indications for ECMO. [Extracted from the article]

Copyright of Journal of Pediatric Emergency & Intensive Care Medicine / Çocuk Acil ve Voğun Bakım Dergisi is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Vacuum‐assisted closure (VAC) has been widely used to treat mediastinitis after congenital cardiac surgery, which is associated with a high risk of morbidity and mortality. The aim in this study is to review our 14 cases of mediastinitis treated with VAC therapy after congenital cardiac surgery. We retrospectively reviewed the medical records of 14 congenital heart patients with mediastinitis from January 2012 to March 2017. Patients with fever, wound discharge, sternal dehiscence, a positive wound culture or abscess diagnosed with computed tomography are accepted as mediastinitis. A VAC was applied to all our patients without irrigation or dressing the wound because of sterility concerns. The vacuuming of the wound was either 50 mm Hg or 75 mm Hg according to the sternal intactness. We gradually decreased the pressures and changed the VAC systems once every three days, after wound healing was seen and a negative culture was obtained and VAC was terminated. There were 14 patients (8 male and 6 female) with mediastinitis and all of them were treated using VAC. The mean age of the patients was 6.96 months (ranging from 0.5‐26 months). The mean weight was 5.16 kg (2.8‐12 kg). Three patients needed extracorporeal membrane oxygenation after the surgery. Mean onset of mediastinitis was 25.3 days. The wound cultures showed methicillin resistant coagulase negative streptococcus and methicillin‐sensitive staphylococcus aureus in most cases. Acinetobacter, serratia, pseudomonas, and klebsiella were the other bacterial species seen in cultures. Two patients had mediastinitis symptoms, but their cultures were negative. VAC systems were changed 3.85 times on average. Mean duration of hospital stay was 49.9 days (21‐104 days). One patient needed a muscle flap to close the thoracic cavity after mediastinitis. Two patients did not survive. Mediastinitis is a serious postoperative condition in pediatric cardiac surgery patients. Classical wound dressing and irrigation methods are not suitable in mediastinitis for the pediatric age group. Therefore, VAC therapy can be an effective way to successfully treat the situation. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Harran University Medical Faculty / Harran Üniversitesi Tıp Fakültesi Dergisi is the property of Harran University Medical Faculty and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: An implantable cardioverter defibrillator (ICD) storm involves very frequent arrhythmia episodes and ICD shocks, and it is associated with poor short‐term and long‐term prognosis. Radiofrequency catheter ablation can be used as an effective rescue treatment for patients with an ICD storm. To our knowledge, this is the first report of an infant with hypertrophic cardiomyopathy presenting with an ICD storm and undergoing successful radiofrequency catheter ablation salvage treatment for the fast left posterior fascicular ventricular tachycardia. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 12-year-old male patient with a murmur and pectus carinatum and dysmorphic features of dolichocephaly, palpebral fissures and scoliosis. It mentions a computed tomography evaluation which revealed aneurysmatic dilatation from the aortic root to the right truncal brachiocephalic branch. It also states detection of mutation of heterozygous missense in the TGFBR2 gene.

Objective: Extracorporeal membrane oxygenation (ECMO) is a lifesaving intervention for pediatric patients with respiratory and/or cardiovascular failure. In this study, we evaluated the cardiac catheterization results of pediatric patients on ECMO support. Methods: Between January 2012 and October 2016, 98 patients (5.2% of all surgery patients) needed ECMO support during perioperative cardiac surgery. We retrospectively reviewed the clinical data of 16 patients who underwent cardiac catheterization under ECMO support. Results: The median age at catheterization was 6.5 months (range, 3.3-60 months), and the median weight was 6.0 kg (range, 3.7-16 kg). Eight of the catheterizations were diagnostic, and the remaining eight were interventional. Five out of these eight patients underwent surgical palliation after diagnostic catheterization. Right pulmonary artery (RPA) stenting, right ventricular outflow tract (RVOT) stenting, combined left pulmonary artery (LPA) and RVOT stenting, combined LPA and modified Blalock-Taussig shunt stenting, bilateral pulmonary artery balloon angioplasty, and bilateral pulmonary artery stenting were each performed once, whereas LPA stenting was performed in two different patients. In one patient undergoing RVOT stenting, a complete atrioventricular block developed, resulting in hypotension; however, this was overcome with an ECMO flow increase. In another patient, the ECMO tubing disconnected from the arterial line. Minor vascular complications were seen in three patients. Twelve patients (75%) were successfully weaned from ECMO after the procedure and ten (63%) were discharged. Conclusion: Diagnostic and interventional cardiac catheterization can be safely and effectively performed in patients on ECMO. If the patient cannot be weaned from ECMO support, clinicians should consider performing an early angiogram either to treat or clarify the underlying problem. [ABSTRACT FROM AUTHOR]

Aim: The aim of the study is to evaluate extracorporeal life support system (ECLS) employed in neonates in pediatric cardiac intensive care unit.Material and Methods: Twenty-five neonates that required ECLS in between November 2010 and November 2015 were evaluated.Results: The median age was 12 days (range 3-28 days) and the median body weight was 3 kg (range 2.5-5 kg). Venoarterial ECLS was performed in all of the cases. Ascendan aorta-right atrial cannulation in 22 patients and neck cannulation in three patients were performed. The reason for ECLS was E-CPR in two patients, inability to wean from cardiopulmonary bypass (CPB) in seven patients, respiratory insufficiency and hypoxia in nine patients, low cardiac output (LCOS) in seven patients. Median duration of ECLS was four days (range 1-15). Hemorrhagic complications developed in 15, renal complications in 13, pulmonary complications in 12, infectious complications in 11, neurologic complications in three and mechanical complications in two of the patients. Weaning was successful in 15 of the patients. Eleven patients were successfully discharged.Conclusion: ECLS is an important treatment option that is performed successfully in many centers around the world to maintain life support in patients unresponsive to medical treatment. The utilization of this modality especially in newborns with congenital heart disease should be taken into consideration. [ABSTRACT FROM AUTHOR]