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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]

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]

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.)

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]

The article presents a case study of a 06-month-old boy with recoarctation of aorta (CoA). Topics include two-dimensional transthoracic echocardiography revealing severe aortic regurgitation and aortic cusp prolapsus; and computed tomography angiography (CTA) showing multiple aneurysms in different parts of the ascending and descending aorta.

Extracorporeal life support (ECLS) is instituted for the management of life threatening pulmonary or cardiac failure (or both), when no other form of treatment has been or is likely to be successful. It has being used in developed countries for many years. ECLS has come into practice for a while in Turkey, in order to ECLS recommendations. However, most of them are postcardiotomy cases. However, this therapeutic option has more indications including its reason that brought it into practice. All equipment of the system has being changed with the developing technology. The aim of this paper is to point out this modality, to introduce equipment, and to give information about organization and management to bring this therapeutic option in Turkey in order. [ABSTRACT FROM AUTHOR]

Extracorporeal life support (ECLS) is instituted for the management of life threatening pulmonary or cardiac failure (or both), when no other form of treatment has been or is likely to be successful. It has being used in developed countries for many years. ECLS has come into practice for a while in Turkey, in order to ECLS recommendations. However, most of them are postcardiotomy cases. However, this therapeutic option has more indications including its reason that brought it into practice. All equipment of the system has being changed with the developing technology. The aim of this paper is to point out this modality, to introduce equipment, and to give information about organization and management to bring this therapeutic option in Turkey in order. [ABSTRACT FROM AUTHOR]

The article presents a case stud on the hybrid transcatheter pulmonary valve implantation (PVI). It highlights the case of three patients who had severe pulmonary valve regurgitation (PVR) after the transannular patch repaired of tetralogy of Fallot (TOF). It also provides discussion of the result which indicates that hybrid PVI plays a significant role in patients who are not suitable for percutaneous PVI.

The article presents the case study of 4.5-month-old boy who underwent hybrid Norwood stage 1, bilateral pulmonary artery banding and ductal stenting with the diagnosis of hypoplastic left heart syndrome (HLHS) who underwent stent implantation into the atrial septum.

Background: This study aims to evaluate early postoperative arrhythmias in children undergoing congenital cardiac surgery., Methods: A total of 670 pediatric patients (355 males, 315 females; median age: 4 months; range, 1 day to 18 years) who underwent cardiac surgery due to congenital heart defects between December 2018 and November 2019 were included. The rate of postoperative arrhythmias, diagnosis, potential risk factors, and management strategies were evaluated. Multivariate regression analysis was used to identify significant factors of development of postoperative arrhythmias., Results: Tachyarrhythmia was detected in 54 patients (8.1%), and the most common tachyarrhythmia was junctional ectopic tachycardia. Medical treatment was required in 25/38 (66%) of junctional ectopic tachycardia patients. Amiodarone was initiated in 18, dexmedetomidine in five, and flecainide + amiodarone in two of the patients. Different degrees of atrioventricular block were observed in 30 patients (4.5%). In 12 patients, permanent pacemakers were implanted during hospitalization. Age at the time of surgery under one-year-old, high inotropic scores, prolonged operation time, and high Aristotele"s scores were independent risk factors associated with early postoperative arrhythmia (p<0.05). The most common operations associated with early postoperative arrhythmia were left ventricular outflow tract, (6/20, 30%), complete atrioventricular septal defect (13/53, 24%), and tetralogy of Fallot (20/134, 14%) surgeries., Conclusion: Cardiac arrhythmias are common in the early period after congenital heart surgery in children. The diagnosis and frequency of arrhythmias may vary according to different surgical procedures., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2021, Turkish Society of Cardiovascular Surgery.)

Background: In this study, we aimed to evaluate the patient and surgical factors affecting prolonged hospital stay and major adverse events after surgical repair of tetralogy of Fallot and to identify the predictors of a complicated course after surgical repair., Methods: A total of 170 consecutive patients (96 males, 74 females; median age 12 months; range, 1 to 192 months) who underwent surgical repair of tetralogy of Fallot between January 2015 and April 2018 were retrospectively reviewed. A mechanical ventilation duration of >24 h, an intensive care unit stay of >3 days, and a hospital stay of >7 days were considered as prolonged. Unplanned reoperation, complete heart block requiring a permanent pacemaker implantation, renal failure, diaphragmatic paralysis, neurological deficit, sudden circulatory arrest, need for extracorporeal membrane oxygenation, and death were considered as major adverse events., Results: The median time to hospital discharge was 8.0 (range, 5.0 to 40.0) days. Higher preoperative hematocrit levels prolonged the length of hospital stay (odds ratio: 1.12, 95% confidence interval 1.1-1.2, p<0.001). A total of 28 major adverse events were observed in 17 patients (10%). Lower pulmonary artery annulus Z-score (odds ratio: 0.5, 95% confidence interval 0.3-0.9, p=0.01) and residual ventricular septal defects (odds ratio: 54.6, 95% confidence interval 1.6-1,874.2, p=0.03) were found to increase mortality. Residual ventricular septal defect was also a risk factor for major adverse events (odds ratio: 12.4, 95% confidence interval 1.5-99.9, p=0.02)., Conclusion: Preoperative and operative factors such as high preoperative hematocrit, low preoperative oxygen saturation, low pulmonary annulus Z-score, Down syndrome, residual ventricular septal defects, and the use transannular patch were found to be associated with prolonged length of hospital stay, prolonged mechanical ventilation, prolonged intensive care unit stay, and increased development of major adverse events., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2020, Turkish Society of Cardiovascular Surgery.)

Background: This study aims to evaluate the surgical results of our clinic according to presumption systems of Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity score, Aristotle Comprehensive Complexity score, and Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories and to compare the efficiency of these systems in predicting morbidity and mortality., Methods: In the study, classification and the risk scoring were performed with the four different systems for 1,950 patients (1,038 males, 912 females; mean age 5.5 months; range, 1 day to 18 years) who were administered congenital heart surgery between 1 October 2012 and 31 December 2016. The hospital mortality and morbidity were calculated for each category from the four models. The discriminatory ability of the models was determined by calculating the area under the receiver operating characteristic curve and the receiver operating characteristic curves of the four models were compared., Results: Median weight of the patients was 7.2 kg (range, 1.8-80 kg). Among the patients, 53% were males and 47.5% were younger than one year of age. Of totally 1,950 operations, mortality was observed in 149 (7.6%) and morbidity was observed in 541 (27.7%). Areas under the receiver operating characteristic curve for mortality were 0.803, 0.795, 0.729, and 0.712 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Aristotle Comprehensive Complexity, Risk Adjustment in Congenital Heart Surgery, and Aristotle Basic Complexity scores, respectively. Areas under the receiver operating characteristic curve for morbidity were 0.732, 0.731, 0.730, and 0.685 for the Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Comprehensive Complexity, and Aristotle Basic Complexity scores, respectively., Conclusion: Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories, Risk Adjustment in Congenital Heart Surgery, Aristotle Basic Complexity, and Aristotle Comprehensive Complexity score systems were effective in predicting the morbidities and mortalities of patients who underwent congenital heart surgery and evaluating the performance of the surgical centers. Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery mortality categories were on the forefront due to high feasibility and performance. Aristotle Basic Complexity score system had the lowest performance. Combinations of systems will provide the most benefit during evaluation of results., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2018, Turkish Society of Cardiovascular Surgery.)