Successful ductal stent implantation for initial palliation in two patients with aortic atresia and interrupted aortic arch.

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other. Initial palliation was successfully performed by bilateral pulmonary artery banding and transcatheter ductal stent implantation in both cases.

Coronary sinus ablations in pediatric patients with supraventricular arrhythmias.

BACKGROUND: Intracoronary sinus ablations have been performed for various arrhythmical substrates. The aim of this study is to report our experience on pediatric patients of the safety and efficacy of ablations in the coronary sinus. METHODS: This is a retrospective study of all patients who underwent ablations in the coronary sinus from October 2013 to October 2021 at a single center. Clinical presentation, type of arrhythmia causing tachycardia, ablation procedure, and outcome were recorded. RESULTS: A total of 27 patients were included in the study. Nineteen (69%) of those followed up received a diagnosis of Wolff-Parkinson-White syndrome (WPW), 4 (15%) were cases of supraventricular tachycardia with concealed accessory pathway (AP), 2 (8%) were cases of focal atrial tachycardia, and 2 (8%) were cases of permanent junctional reciprocating tachycardia. Negative delta wave was noteworthy especially in lead II in 11/19 (58%) cases and coronary sinus diverticulum was detected in the WPW cases. Of those with manifest AP (19 cases), 15 (79%) had a high-risk AP and the AP in all WPW cases was adenosine unresponsive. Radiofrequency (RF) catheter ablation was performed in 25/27 (93%) cases during the procedure, and 16/25 (64%) of these were irrigated RF catheters. No complications were observed in the follow-up, including coronary artery injury. CONCLUSIONS: Catheter ablation of supraventricular tachyarrhythmias can be accomplished effectively and potentially safely within the coronary sinus. Coronary sinus diverticula should be suspected in patients with manifest posteroseptal APs who have a previous failed ablation and typical electrocardiographic signs.

Clinical effects of major aortopulmonary collateral arteries in term neonates diagnosed with transposition of the great arteries.

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.

Rapid reversal of left ventricular enlargement after percutaneous closure of pulmonary sequestration in a newborn with prune belly syndrome.

In this article, we describe a newborn with Prune belly syndrome who presented with left ventricular dilation due to an extensive intralobar sequestration of the left lung. Pulmonary sequestration was combined with congenital cystic adenomatoid malformation and also had coarctation of the aorta. Percutaneous closure of the anomalous aberrant artery feeding the sequestrated lung and balloon angioplasty for coarctation resulted in prompt regression of the left ventricular enlargement in the catheterization lab.

Predictors of prolonged pleural effusion after Fontan operation.

Prolonged pleural effusion is a fairly common condition which has considerable impact on complicated and longer hospital stays after Fontan surgery. Identifying the patient population prone to have pleural effusions is still seeking for an answer. This study is to determine the variables that may predict prolonged pleural effusion according to the data of 69 patients who underwent Fontan operation between June 2018 and December 2020 and survived to date. Prolonged pleural effusion was defined as the need for a chest tube for more than 7 days. Two patient groups, with and without prolonged effusion, were compared in terms of pre-, peri-, and post-operative variables. The patients were subdivided into "high-risk" and "low-risk" groups based on the pre-operative catheterisation data. The most frequent main diagnosis was tricuspid atresia (n: 13, 19%). Among 69 patients, 28 (40%) had prolonged pleural effusion whereas 11 (16%) had effusions that lasted longer than 14 days. Ten patients among prolonged effusion group (35%) had pulmonary atresia coexistent with the main diagnosis. Fontan operation was performed in 6 patients (8.7%) over the age of 10, and 4 of these patients (67%) had prolonged pleural effusion. Among numerous variables, statistical significance between the two groups was achieved in pre-operative mean pulmonary artery pressure, post-operative albumin, C-reactive protein levels, length of hospital stay, duration of chest tube drainage, and amount of effusion per day. Early recognition and treatment strategies with routine medical protocol use remain to be the cornerstone for the management of post-operative prolonged pleural effusions after Fontan surgery.

Isolated coarctation repair through a left thoracotomy in children.

INTRODUCTION: Isolated aortic coarctation performed through a left thoracotomy resection and end-to-end anastomosis results in low mortality and morbidity rates. Recoarctation and late hypertension are among the most important complications after such repairs. In this study, we reviewed the results of children who underwent left-side thoracotomy to correct an isolated aortic coarctation. METHOD: A consecutive sample of 90 patients who underwent resection and extended end-to-end anastomosis through a left-side thoracotomy in our centre between 2011 and 2021 was retrospectively analysed. The patients' preoperative characteristics, operative data, and post-operative early and long-term results were examined. RESULTS: All patients underwent resection and extended end-to-end anastomosis. A pulmonary artery band was applied simultaneously to three (3.3%) patients, and an aberrant right subclavian artery division was applied to one (1.1%) patient. The mean cross-clamp time was 29.13 ± 6.97 minutes. Two (2.2%) patients required reoperation in the early period. Mortality was observed in one (1.1%) patient in the early period. Eight (8.8%) patients developed recoarctation, of whom four (4.4%) underwent reoperation and four (4.4%) underwent balloon angioplasty. Twenty-two (26.8%) patients received follow-up antihypertensive treatment. The mean follow-up period was 41.3 ± 22.8 months. No mortality was observed in the late period. CONCLUSION: Isolated coarctation is successfully treated with left-side thoracotomy resection and an extended end-to-end anastomosis technique with low mortality, morbidity, and low long-term recoarctation rates. Long-term follow-up is required due to the risks of early and late post-operative recoarctation, which requires reintervention.

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.

Comparison of echocardiography and 320-row multidetector computed tomography for the diagnosis of congenital heart disease in children.

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.

Surgical treatment of Ebstein anomaly in pediatric patients: A 10-year single-center study.

OBJECTIVE: We report the early and long-term results of the strategies and surgical methods used in our center to treat pediatric patients who underwent surgical intervention to correct Ebstein anomaly (EA) in our center. MATERIALS AND METHODS: In our study, a consecutive sample of 29 patients who underwent surgery for EA between February 2011 and February 2020 in our center were evaluated retrospectively. RESULTS: The 29 patients underwent a total of 40 operations. Univentricular repair was performed in 5 (17.2%), 1.5 ventricular repair in 5 (17.2%), and biventricular repair in the remaining 19 (65.5%) patients. Cone reconstruction (CR) was performed in eight (27.5%), non-Cone tricuspid valve (TV) repair technique in five (17.2%), ring annuloplasty in two (6.9%), and TV replacement in two patients (6.9%) who had undergone biventricular repair. In two patients (6.9%), only close an atrial septal defect. Two (6.9%) patients underwent a second operation for advanced tricuspid regurgitation (TR) in the early period. None of the 15 patients who underwent CR and TV plasty had moderate or advanced TR before discharge. Early mortality was seen in 1 (3.4%) patient. The mean follow-up period of the patients was 48.4±27.6 months. Three (10.7%) of the patients who were discharged after their first operation later underwent a second operation for TV regurgitation in the long term. No mortality was observed in any patient during long-term follow-up. CONCLUSION: Surgical treatment of EA is difficult, but its overall results are good. The anatomical repair rate is lower in neonatal and infant patients requiring surgery, but most of these patients underwent biventricular repair. Our long-term results demonstrated an acceptable survival rate, low mortality in the early postoperative period, and low incidence of re-intervention and morbidity.

The Use of Chronic Total Occlusion (CTO) Wires for Perforation of Atretic Pulmonary Valve; Two Centers Experience.

Pulmonary valve atresia with intact ventricular septum (PA-IVS) can be treated either surgically or transcatheterly for eligible patients. Perforation of pulmonary valves using chronic total occlusion (CTO) guidewires has been reported as an alternative to radiofrequency (RF) perforation. We sought to report our experience with CTO guidewires for perforation of atretic pulmonary valves and subsequent balloon dilatation (with or without patent ductus arteriosus stenting) in patients with PA-IVS from two centers. A retrospective study was carried out on PA-IVS patients who underwent intervention between March 2014 and September 2019, in which CTO guidewire was employed for pulmonary valve perforation. A total of 26 patients were identified. The median age and weight of the patients were 5.2 days (range 1-21 days) and 3.1 kg (range 2.2-3.8 kg), respectively. All patients were situs solitus, except one patient with left atrial isomerism. The right ventricle (RV) morphology was bipartite in 22/26 patients and tripartite in 4/26 patients. Before the procedure, the mean saturation was 76% (range 70-86%) while the patients were under prostaglandin infusion. The pulmonary valve perforation attempt was performed with the Asahi Conquest Pro 9 CTO wire (n = 6) or Asahi Pro 12 CTO wire (n = 18) and/or Asahi Miracle CTO wire (n = 2). The procedure was successful in 20/26 (77%) patients using CTO wires. We analyzed the efficiency of CTO wire based on the subtypes: Conquest Pro 9 in 6/6 (100%) patients, Conquest Pro 12 in 12/18 (67%) patients, and Miracle in 2/2 (100%) patients. Before CTO wire usage in 3 patients, radiofrequency (RF) perforation was unsuccessful. Among these 3 patients, pulmonary valve perforation was successful in 2 patients with CTO wire; hence, in the remaining patient, perforation was also unsuccessful with CTO wire. After CTO wire perforation was unsuccessful in 6 patients, RF perforation was attempted in 3 patients (2 successful attempts and 1 unsuccessful attempts), and one patient as referred to surgery. Desaturation was persistent in 19 cases, which necessitated ductus arteriosus stenting. Early procedural complication was observed in 3/26 (11%) patients. Two of these patients had vascular complications due to the sheath, which was treated with heparin infusion and streptokinase, and the remaining patient had sudden bradycardia and cardiac arrest during the procedure and did not respond to cardiac resuscitation. CTO wires should be keep in mind for atretic pulmonary valve perforation as a first choice or when RF perforation is unsuccessful.

Right ventricular outflow tract stenting during neonatal and infancy periods: A multi-center, retrospective study.

BACKGROUND: The aim of this study was to evaluate the outcomes of right ventricular outflow tract stenting for palliation during the newborn and infancy periods. METHODS: Between January 2013 and January 2018, a total of 38 patients (20 males, 18 females; median age 51 days; range, 3 days to 9 months) who underwent transcatheter right ventricular outflow tract stenting in three centers were retrospectively analyzed. Demographic characteristics, cardiac pathologies, angiographic procedural, and clinical follow-up data of the patients were recorded. RESULTS: The diagnoses of the cases were tetralogy of Fallot (n=27), double outlet right ventricle (n=8), complex congenital heart disease (n=2), and Ebstein"s anomaly (n=1). The median weight at the time of stent implantation was 3.5 (range, 2 to 10) kg. Five cases had genetic abnormalities. The median pre-procedural oxygen saturation was 63% (range, 44 to 80%), and the median procedural time was 60 (range, 25 to 120) min. Acute procedural success ratio was 87%. Reintervention was needed in seven of patients due to stent narrowing during follow-up. During follow-up period, seven cases died. Total correction surgery was performed in 26 patients without any mortality. While a transannular patch was used in 22 patients, valve protective surgery was implemented in two patients, and the bidirectional Glenn procedure was performed in two patients. CONCLUSION: Based on our study results, right ventricular outflow tract stenting is a form of palliation which should be considered particularly in cases in whom total correction surgery is unable to be performed due to morbidity.

Comparison of 6-mm Versus 8-mm-Tip Cryoablation Catheter for the Treatment of Atrioventricular Nodal Reentrant Tachycardia in Children: A Prospective Study.

Due to its safety profile, cryoablation (Cryo) for atrioventricular nodal reentrant tachycardia (AVNRT) is more commonly preferred over radiofrequency (RF) ablation in children in recent years. Recent studies demonstrated high long-term success rates comparable to radiofrequency ablation. The aim of this prospective study was to compare the efficacy and safety of an 8-mm-tip versus 6-mm-tip Cryo catheter in the treatment of AVNRT in children. A total of 125 consecutive patients over 10 years of age with AVNRT were included. EnSite system (St. JudeMedical, St Paul, MN, USA) was used to reduce or eliminate fluoroscopy. The acute procedural success was 100% in both groups. The prodecure duration for the 8-mm-tip group was shorter (151.6 ± 63.2 vs. 126.6 ± 36.7 min, p < 0.01, respectively). Fluoroscopy was used in only 7 patients. The mean follow-up duration was 14.6 ± 8.4 months (median 13.5 months, min. 3 months and max. 27 months). The recurrence rate for AVNRT was also comparable between the two groups (6-mm tip: 9.6 vs. 8-mm tip: 8%). Cryo of AVNRT is a safe and effective procedure with comparable acute and mid-term follow-up success rates using 6-mm and 8-mm-tip catheters in children. In addition, procedure duration is shorter with an 8-mm-tip Cryo catheter.

A rare coronary anomaly with masked diagnosis: Anomalous left circumflex artery from right pulmonary artery.

Anomalous origin of the circumflex coronary artery from the pulmonary artery is a rare congenital coronary anomaly. While it generally follows an asymptomatic course, if undiagnosed it may lead to severe clinical outcomes, including sudden death. The condition can be masked by associated defects, so when it is clinically suspected, diagnosis must be confirmed by conventional and/or magnetic resonance angiography, even if echocardiography clearly shows coronary roots. This report describes a patient who underwent neonatal surgery for aortic coarctation and was diagnosed with coronary artery anomaly at 15 months old.