Search

Background: Transposition of the great arteries is a severe CHD that affects term neonates. The presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients is rare. This study investigated the clinical and haemodynamic implications of the presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients who underwent an arterial switch operation., Materials and Methods: The study was a retrospective analysis conducted on neonates diagnosed with transposition of the great arteries who underwent arterial switch operation within the period from 1 May 2020 to 1 January 2023 at two high-patient-volume paediatric cardiac surgery centres in Turkey. The patients' demographic characteristics, echocardiographic features, and clinical data were analysed. Additionally, the possible clinical effects of the presence of major aortopulmonary collateral arteries were statistically evaluated., Results: Two hundred cases of neonatal transposition of the great arteries were included in this study, with 55% of the cases male. All the patients underwent arterial switch operation. The median age at the time of arterial switch operation was 5 days (interquartile range 3-7), with a median weight of 3,100 g (interquartile range 2,900-3,400). The median pre-operative saturation level was 76% (interquartile range 70-82%). Prior to arterial switch operation, 32 patients underwent balloon atrial septostomy.In all the patients, the interatrial septum was checked to determine if the atrial septum was intact. A patent foramen ovale (≤ 3 mm) was found in 112 patients, and a non-restrictive atrial septal defect (> 3 mm) was found in 88. Forty-eight patients had ventricular septal defects, and 72 had coronary anomalies. Major aortopulmonary collateral arteries were found in 4 patients pre-operatively and in 12 patients after arterial switch operation (echocardiography, n = 8; angiography, n = 4). Of the patients with post-operative detection of cumulative number of major aortopulmonary collateral arteries were on post-operative day 1 in 2 patients, on post-operative day 3 in 5 patients, on post-operative day 7 in 6 patients, and on post-operative day 14 in 11 patients.Transcatheter closure was performed in 3 cases due to recurrent extubation failure. Major aortopulmonary collateral artery shrinkage was observed in one case under medical treatment. The length of paediatric cardiac intensive care unit stay (10 days versus 8 days; p < 0.005), mechanical ventilator time (4 days versus 2 days; p = 0.02), and inotrope use time (5 days versus 3 days; p = 0.04) were higher in the major aortopulmonary collateral artery cases than patients without major aortopulmonary collateral artery., Conclusion: Major aortopulmonary collateral arteries are frequent in transposition of the great arteries patients and may have clinical effects. The presence of major aortopulmonary collateral arteries should be investigated in patients who do not have a favourable post-operative course after arterial switch operation.

Introduction: Echocardiography (echo) is the primary non-invasive imaging modality for the assessment of congenital heart disease (CHD). Computed tomography angiography (CTA) also has potential to examine the anatomy of complex heart anomalies as well as extracardiac involvement., Objectives: The aim of this study is to determine the impact of new CTA technology in the diagnosis of CHD and to compare echo and CTA in terms of diagnostic accuracy., Methods: Forty-five patients who underwent preoperative echo and CTA assessment in the intensive care unit were included in this study. The results were assessed for three main types of CHD (cardiac malformations, cardiac-major vessel connections and major vessels). The main groups were also divided into subgroups according to surgical features in order to assess them more objectively. Imaging methods were compared for diagnostic accuracy, sensitivity and specificity, while surgical findings were accepted as the gold standard., Results: Patients' median age and weight were two months (three days-eight years) and 12 kg (2.5-60 kg), respectively. In 45 operated cases, 205 subgroup malformations were assessed. Diagnostic accuracy was significantly greater in echo (echo vs. CTA: 98.4% and 96.2% [chi-square=6.4, p=0.011]). During surgery, 84 cardiac malformations (echo vs. CTA: 97.4% and 95.1% [chi-square=4.9, p=0.03]), 47 cardiac-major vessel connections (echo vs. CTA: 98.3% and 95.4% [chi-square=7.5, p=0.03]), and 74 major vessel malformations (echo vs. CTA: 96% and 98% [chi-square=1.8, p=0.48]) were confirmed., Conclusion: Echocardiography and CTA are imaging methods with high diagnostic accuracy in children with CHD. The use of echocardiography together with CTA, especially for the visualization of extracardiac anatomy, provides additional information for clinicians., (Copyright © 2021 Sociedade Portuguesa de Cardiologia. Published by Elsevier España, S.L.U. All rights reserved.)

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.

Objectives: In recent years, cardiac resynchronization therapy (CRT) has also started to be performed in the paediatric and CHD population. This study aimed to evaluate the efficacy of CRT in children with CHD., Patients and Methods: Patients with CHD who underwent CRT treatment in our paediatric cardiology clinic between January, 2010 and January, 2020 were included in the study. Demographic findings, 12-lead electrocardiograms, echocardiograms, clinical characteristics, management strategies, and outcomes were reviewed systematically., Results: The study population consisted of 18 CHD patients who had been treated with CRT for 10 years in our institution. The median age was 11 years (2.2-18 years) and the median weight was 39 kg (10-81 kg). Systemic ventricle was left ventricle in 13 patients, right ventricle in 4 patients, and 1 patient had single-ventricle physiology. CRT implantation indications were as follows: dysfunction after permanent pacemaker in 11 patients, dysfunction after left bundle branch block in 4 patients, and systemic ventricular dysfunction in 3 patients. CRT implantation techniques were epicardial (n = 13), hybrid (n = 4), and transvenous (n = 1) methods. QRS duration significantly decreased after CRT implantation (160 versus 124 m/second, p < 0.05). Median systemic ventricle ejection fraction (EF) significantly increased after the procedure (30 versus 50%, p < 0.05). Fourteen patients (78%) were responders, two patients (11%) were superresponders, and two patients (11%) were non-responders after the CRT treatment. One patient deceased during follow-up. Median follow-up duration was 40 months (6-117 months)., Conclusion: When electromechanical dyssynchrony occurs in paediatric cases with CHD and developing heart failure, patients should be evaluated in terms of CRT to improve ventricular function. Alternative CRT therapy will be beneficial in these cases that do not improve clinically despite optimal medical treatment.

We investigated the effects of intraoperative parameters measured during pulmonary artery banding operations and pre-discharge parameters on the completion of Fontan procedures. Fifty consecutive patients with single-ventricle anomalies and unrestricted pulmonary blood flow who underwent a PAB operation in and were discharged from our hospital were retrospectively analyzed. Patients who underwent a Fontan operation, a Glenn shunt operation, or who were eligible for a Fontan procedure were defined as the "successful group." Patients who needed rebanding prior to a bidirectional Glenn shunt, patients who were not eligible for a Glenn shunt, and those underwent a takedown due to high pulmonary arterial pressure after implantation of a Glenn shunt were defined as the "failure-to-progress group." The successful group included 34 (68%) patients and the failure-to-progress group included 16 (32%) patients. The median age was 2 months (IQR 1-4 months). There was a statistically significant difference between the groups in terms of systolic pulmonary arterial pressure, mean pulmonary arterial pressure, and pulmonary arterial pressure/systemic arterial pressure after PAB (P = 0.01, 0.03, and 0.03, respectively). While the median gradient before discharge was 60 mm Hg (IQR 50-70 mm Hg) in the successful group, it was 47.5 mm Hg (IQR 45-63.7 mm Hg) in the failure-to-progress group (P = 0.05). Mortality was observed in one (2.9%) patient in the successful group and five (31.2%) patients in the failure-to-progress group (P = 0.04). Successful pulmonary arterial banding increases long-term survival. Adequate targets should be determined, efforts should be made to achieve these targets, and patients should be followed up closely in terms of rebanding when the targets are not reached.

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.

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.