One-Year Follow-Up Results of MIS-C Patients with Coronary Artery Involvement: A Multi-center Study.

Multisystem inflammatory syndrome (MIS-C) in children is a rare complication of SARS-CoV-2 infection. Knowing the course of the affected or unaffected coronary arteries in the patients under follow-up is important in terms of defining the long-term prognosis of the disease and determining the follow-up plan. This is a multicenter and retrospective study. The data were obtained from nine different centers. Between May 2020 and August 2022, 68 of 790 patients had coronary artery involvement. One-year echocardiographic data of 67 of 789 MIS-C patients with coronary artery involvement were analyzed. Existing pathologies of the coronary arteries were grouped as increased echogenicity, dilatation and aneurysm according to Z scores, and their changes over a 1-year period were determined. The data of all three groups are defined as frequency. SPSS Statistics version 22 was used to evaluate the data. In our study, aneurysm was observed in 16.4%, dilatation in 68.7% and increased echogenicity in 13.4% of the patients. All of the patients with involvement in the form of increased echogenicity recovered without sequelae by the end of the first month. No progression to aneurysm was observed in any of the patients with dilatation. No new-onset involvement was observed in patients with previously healthy coronary arteries during the convalescent period. In addition, from the sixth month follow-up period, there was no worsening in the amount of dilatation in any of the patients. At least 94% of the patients who completed the 12th month control period returned to normal.

Myocardial infarction and narrowed peripheral arterial vessels secondary to generalised arterial calcification syndrome in a two-month-old girl.

Generalised arterial calcification of infancy, an autosomal recessive disorder characterised by abnormal calcification of medium and large-sized arteries, represents a rare cause of dilated cardiomyopathy. We present the case of a two-month-old girl diagnosed posthumously with dilated cardiomyopathy. Studies suggest that early initiation of treatment can improve prognosis in generalised arterial calcification of infancy, so clinicians should be alert to the condition, especially in patients displaying generalised narrowing of medium and large-sized arteries.

Assessment of the frequency and risk factors of gastrointestinal bleeding after cardiopulmonary bypass in paediatric cases.

INTRODUCTION AND AIM: Gastrointestinal bleeding is a potential complication in paediatric patients undergoing cardiopulmonary bypass, as it develops secondary to low gastrointestinal perfusion. This study aimed to examine the incidence of gastrointestinal bleeding and identify its risk factors in these patients. METHOD: This retrospective study was undertaken to examine the demographic features, clinical findings, and operative data of paediatric patients under years old who had undergone congenital heart surgery with cardiopulmonary bypass between November 1, 2021, and November 1, 2023. The study aimed to investigate the incidence of gastrointestinal bleeding associated with cardiopulmonary bypass and to identify potential risk factors for gastrointestinal bleeding. The obtained results were statistically evaluated. RESULTS: The study period included 1100 patients who underwent congenital heart surgery with cardiopulmonary bypass. Fifty-two percent of the total participants were male. The median weight of the patients was 4.4 kg, with an interquartile range of 3.5-5.8 kg. The patients were categorised by age, revealing that 62% were newborns, 24% were infants, and 14% were children. Forty-four (4.2%) of the total number of patients experienced gastrointestinal bleeding. Newborns had a significantly higher incidence of bleeding (6% or 34 patients) compared to infants (3% or 8 patients) and children (1.5% or 2 patients) (p < 0.05). Patients who experienced gastrointestinal bleeding had a longer median hospital stay of 24 days compared to those who did not, with a median hospital stay of 14 days. Moreover, patients who suffered from bleeding had a significantly higher mortality rate (30%) in comparison to those who did not (9.9%) (p < 0.05). The incidence of gastrointestinal bleeding was found to be associated with several risk factors, such as low operative age and weight, high surgical score, presence of low cardiac output syndrome, extracorporeal membrane oxygenation (ECMO) usage, high lactate levels, and low platelet count. CONCLUSION: Gastrointestinal bleeding is a potential complication for patients who undergo cardiopulmonary bypass. It is particularly relevant for newborns who have undergone prolonged surgery, have a high surgical complexity score, exhibit high lactate levels, display low cardiac output, utilise ECMO, and possess low platelet counts. In such cases, there may be a heightened incidence of gastrointestinal bleeding. It is important to consider this possibility in order to ensure the best possible patient outcomes.

Impact of high-flow nasal oxygen therapy on postoperative atelectasis and reintubation rate after paediatric cardiac surgery.

INTRODUCTION: Airway problems emerging after congenital cardiac surgery operations may have an impact on mortality and morbidity. Recently, to improve alveolar gas exchange and reduce respiratory effort, high-flow nasal cannula (HFNC) has started to be used in paediatric cases. This study aimed to evaluate the potential effects of high-flow nasal oxygen therapy on postoperative atelectasis development and reintubation rate in paediatric cardiac surgery patients. METHODS: This study was conducted retrospectively in term newborns and infants younger than six months of age who underwent congenital cardiac surgery operation from 1 November 2022 to 1 November 2023 and were followed in the paediatric cardiac ICU. Patients who were receiving mechanical ventilator support at least 12 hours postoperatively were evaluated for the development of postoperative atelectasis and reintubation in the first 3 days of extubation. The patients were grouped as HFNC and non-HFNC users. Demographic characteristics, surgery type, and ICU clinical follow-up data were obtained from medical records. The results were statistically evaluated. RESULTS: A total of 40 patients who did not use HFNC in the early postoperative period and 40 patients with HFNC in the late period during the study period were included in the study. The median age was 1 month (IQR 15 days-2 months) with equal gender distribution. Among patients, 70% of them were in the neonatal age group. Reintubation rates in the first 72 hours in HFNC users and non-HFNC users were 2.5% and 12.5%, respectively (p < 0.05). The median postoperative atelectasis scores at 24, 48, and 72 hours of extubation were 2 versus 2.5 (p > 0.05), 1.5 versus 3.5 (p < 0.05), and 1 versus 3 (p < 0.05) in HFNC users and non-HFNC users, respectively. CONCLUSION: HFNC therapy may have a positive effect on preventing atelectasis and reducing the reintubation rate in the early postoperative period.

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.

A unique bizarre subtype of mixed total anomalous pulmonary venous connection.

Mixed total anomalous pulmonary venous connection is a rare CHD with a wide variation in pulmonary venous anatomy and drainage. We present a ten-day-old newborn with a rare bizarre subtype of mixed total anomalous pulmonary venous connection.

Comparison of the effects of conventional method and primary sutureless techniques on early postoperative rhythm problems in patients with total abnormal pulmonary venous return anomaly.

BACKGROUND: Total abnormal pulmonary venous return anomaly is a CHD characterised by abnormal pulmonary venous flow directed to the right atrium. In this study, we aimed to compare the effects of these techniques on early rhythm problems in total abnormal pulmonary venous return anomaly cases operated with conventional or primary sutureless techniques. METHOD: Seventy consecutive cases (median age 1 month, median weight 4 kg) who underwent total abnormal pulmonary venous return anomaly repair with conventional or primary sutureless technique between May 1 2020 and May 1 2022 were evaluated. The rate, diagnosis, and possible risk factors of postoperative arrhythmias were investigated. The results were evaluated statistically. RESULTS: When the total abnormal pulmonary venous return anomaly subgroup of 70 cases was evaluated, 40 cases were supracardiac, 18 cases were infracardiac, 7 cases were cardiac, and 5 cases were mixed type. Twenty-eight (40%) cases had a pulmonary venous obstruction. Primary sutureless technique (57%, supracardiac n = 24, mixed = 3, infracardiac = 13) was used in 40 patients. Median cardiopulmonary bypass time (110 versus 95 minutes) and median aortic clamp time (70 versus 60 minutes), median peak lactate (4.7 versus 4.8 mmol/l) in the first 72 hours, and median peak vasoactive inotropic score in the first 72 hours of the primary sutureless and conventional technique used cases value (8 versus 10) were similar. The total incidence of arrhythmias in the conventional group was significantly higher than in the primary sutureless group (46.7% versus 22.5%, p = 0.04). Supraventricular early beat was observed in 3 (7.5%), sinus tachycardia was seen in 6 (15%), junctional ectopic tachycardia was seen in 1 (2.5%), intra-atrial reentry tachycardia was seen in 1 (2.5%), usual supraventricular tachyarrhythmia was seen in 2 cases (5%) in the primary sutureless group. In the conventional group, supraventricular early beat was observed in six of the cases (20%), sinus tachycardia in five (16.7%), junctional ectopic tachycardia in four (13.3%), intra-atrial reentry tachycardia (10%) in three, and supraventricular tachyarrhythmia in seven cases (23.3%). In the first 30 days, there was a similar mortality rate (10% versus 10%), with four patients in the primary sutureless group and three in the conventional group. The median follow-up period of the cases was 8 months (interquartile range (IQR) 6-10 months). In the follow-up, arrhythmias were detected in two cases (one supraventricular tachyarrhythmia and one intra-atrial reentry tachycardia) in the primary sutureless group and three cases (two supraventricular tachyarrhythmia, one intra-atrial reentry tachycardia) in the conventional technique. All cases were converted to normal sinus rhythm with cardioversion and combined antiarrhythmic therapy. CONCLUSION: Different arrhythmias can be observed in the early period in patients with operated total abnormal pulmonary venous return anomaly. Although a higher rate of rhythm problems was observed in the early period in the conventional method compared to the primary sutureless technique, no significant effect was found on mortality and morbidity between the groups.

Evaluation of perfusion index and left ventricular output changes in low cardiac output syndrome after arterial switch operation.

INTRODUCTION: Transposition of great arteries is one of newborns' most common cyanotic CHDs, and its treatment is arterial switch operation in the first days of life. Low cardiac output syndrome may develop in the early postoperative period. In this study, we evaluated perfusion index and left ventricular output blood flow changes in patients who underwent arterial switch operation and developed low cardiac output syndrome. METHODS: This study was conducted prospectively in newborns with transposition of great arteries who underwent arterial switch operation between 1st August 2020 and 1st August 2022. Low cardiac output syndrome score and left ventricular output were investigated. Initially, 6th, 12th, 18th, and 24th hour perfusion index and left ventricular output values of patients with and without low cardiac output syndrome were recorded. The results were evaluated statistically. RESULTS: A total of 60 patients were included in the study. Sex distribution was equal. The median age at the time of surgery was 5 days (interquartile range 3-7 days), and the median weight was 3.1 kg (interquartile range 2.9-3. 4). Low cardiac output syndrome was detected in 30% (n = 18) of cases. The median perfusion index of patients who developed low cardiac output syndrome was significantly lower at the 12th, 18th, and 24th hours (p < 0.05) (0.99 versus 1.25, 0.86 versus 1.21, and 0.96 versus 1.33, respectively). Similarly, the median left ventricular output of patients who developed low cardiac output syndrome was significantly lower at 12th, 18th, and 24th hours (p < 0.05) (95 versus 110 ml/kg/min, 89 versus 109 ml/kg/min, and 92 versus 112 ml/kg/min, respectively). There was a significant correlation between perfusion index values and left ventricular output at all measurements (r > 0.500, p < 0.05). CONCLUSION: Perfusion index and left ventricular output measurements decreased in newborns who developed low cardiac output syndrome after arterial switch operation, especially at 12th and 18th hours. Serial perfusion index and left ventricular output measurements can be instructive in predicting low cardiac output syndrome development.

The comparison of three different acute kidney injury classification systems after congenital heart surgery.

BACKGROUND: We aimed to compare the frequency of acute kidney injury (AKI) and its effects on mortality and morbidity with different classification systems in pediatric patients who had surgery under cardiopulmonary bypass for congenital heart disease. METHODS: This study included children younger than 18 years old who were followed up in the pediatric cardiac intensive care unit between September 1 and December 1, 2020, after congenital heart surgery with cardiopulmonary bypass. Each case was categorized postoperatively in terms of AKI using Pediatric-Modified Risk, Injury, Failure, Loss, and End-Stage (pRIFLE), Acute Kidney Injury Network (AKIN), and Kidney Disease: Improving Global Outcomes (KDIGO). Hospital mortality (developed within the first 30 days postoperatively) and morbidity (longer than 7 days intensive care unit stay) were compared by three model classes. Results were evaluated statistically. RESULTS: One hundred patients were included in the study. The median age was 3 months (1 day-180 months). Acute kidney injury was diagnosed in 49% of the cases according to the pRIFLE classification. It was diagnosed in 31% of the patients by AKIN classification. It was diagnosed in 41% of the patients with the KDIGO criteria. Morbidity was observed in 25% (n = 25) of all cases. The morbidity predictor was 0.800 for pRIFLE, 0.747 for AKIN and 0.853 for KDIGO by receiver operating characteristics analysis. All three categories predicted morbidity significantly (P < 0.001). Mortality was 10% (n = 10) for all groups. The mortality predictor was 0.783 for pRIFLE, 0.717 for AKIN and 0.794 for KDIGO by receiver operating characteristics analysis, and all three categories predicted mortality significantly (P < 0.001). CONCLUSIONS: Regardless of the three methods used, AKI was commonly detected in pediatric patients undergoing congenital heart surgery. pRIFLE classification diagnosed more patients with AKI than AKIN and KDIGO. The KDIGO and pRIFLE classifications were better in predicting hospital mortality.

Comparison of clinical and laboratory features in coronavirus disease 2019 and pediatric multisystem inflammatory syndrome patients.

BACKGROUND: The coronavirus disease 2019 (COVID-19) and pediatric multisystem inflammatory syndrome (PIMS) are a major public health issue affecting many people worldwide. Although there are new studies in children, little is known about these two new conditions. The aim of this study was to evaluate and compare the clinical and laboratory features of children with COVID-19 and PIMS. METHODS: We conducted a prospective, single-center study of pediatric COVID-19 and PIMS at a tertiary care hospital in Turkey between November 2020 and March 2021. RESULTS: A total of 115 patients with COVID-19 and PIMS were examined during the study period. The median age was 60 (range, 1-215) months and 64% of the patients were male. The most common clinical symptoms were fever (70%) and cough (43%). Conjunctivitis and skin rash were not seen in PIMS patients. Of all patients, 64% had a history of close contact in household. Lymphopenia was present in 34/115 (30%) patients. Acute phase reactants were significantly higher in PIMS patients. Abnormal chest computed tomography scan findings were detected in 68% of the patients, while 36% had abnormal echocardiographic findings. In multivariate analysis, longer duration of fever, diarrhea, lower thrombocyte and higher neutrophil count were significantly associated with diagnosis of PIMS. The treatment included antibiotics, favipiravir, intravenous immunoglobulin, corticosteroids, interleukin-1 blockade. and supportive therapy. Seven patients (6%) required intensive care support. All patients were discharged without any complications, except one who died. CONCLUSIONS: Longer duration of fever, diarrhea, lower thrombocyte, and higher neutrophil count can warn clinicians for diagnosis of PIMS.

Can we predict risk for cardiac involvement in paediatric inflammatory multi-system syndrome?

INTRODUCTION: Increasing recognition of paediatric inflammatory multi-system syndrome is a cause of concern. This study aimed to evaluate children with paediatric inflammatory multi-system syndrome and compare the clinical and laboratory features of children with and without cardiac involvement. MATERIAL AND METHODS: We conducted a prospective single-centre study including 57 (male 37, 65%) patients with paediatric inflammatory multi-system syndrome at a tertiary care hospital between November, 2020 and March, 2021. The mean age was 8.8 ± 4.5 years (range, 10 months-16.7 years). RESULTS: The most frequent symptoms were fever (100%), abdominal pain (65%) and diarrhoea (42%). SARS-CoV-2 PCR and serology tests were positive in 3 (5%) and 52 (91%) patients, respectively. Eight patients required intensive care support. Nineteen patients (33%) had cardiac involvement (valvular regurgitation in 15, left ventricular systolic dysfunction in 11 and coronary artery dilation in 1). The presence and duration of cough and intensive care admissions were significantly higher in children with cardiac involvement than those without it. The cut-off values of troponin T, pro-brain natriuretic peptide and interleukin 6 for predicting cardiac involvement were 11.65 ng/L (95% confidence interval, 0.63-0.90; sensitivity, 0.63; specificity, 0.84; area under the curve: 0.775, p = 0.009), 849.5 pg/mL (95% CI, 0.54-0.86; sensitivity, 0.63; specificity, 0.63; area under the curve: 0.706, p = 0.009) and 39.8 pg/mL (95% CI, 0.54-0.85; sensitivity, 0.63; specificity, 0.60; area under the curve: 0.698, p = 0.023), respectively. CONCLUSIONS: Cardiac involvement in children with paediatric inflammatory multi-system syndrome is common. The risk of cardiac involvement can be predicted by troponin T, pro-brain natriuretic peptide and interleukin 6 levels.

Topsy-turvy heart: A first recorded case report with intracardiac anomaly.

Topsy-turvy heart is a rare congenital anomaly; it has a specific characteristic of cardiac malrotation and superior-inferior arrangement of right/left ventricles. A five-day-old patient was diagnosed antenatally with hypoplastic left heart and right hydronephrosis and had been admitted to the hospital with symptoms of respiratory distress. The postnatal imaging studies revealed an abnormal ventricular arrangement with a large aortopulmonary window, ventricular septal defect (VSD), and the upside-down orientation resulting in the posterior-inferior displacement of the common arterial confluence and, in turn, the left main bronchial stenosis. For the first time, the diagnosis of a topsy-turvy heart with the intracardiac anomaly (VSD) was confirmed.

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.

Univentricular Pulmonary Artery Banding: How Tight is Tight Enough for Successful Progress?

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.

Transcatheter pulmonary valve implantation in tetralogy of Fallot and Ebstein's anomaly with one and a half ventricular repair.

The coexistence of tetralogy of Fallot and Ebstein's anomaly is extremely rare. There are only a few case reports in the literature, and surgical options for the treatment are controversial. There is insufficient data on long-term follow-up of patients and management of complications. In this case report, we present a 20-year-old adult with operated tetralogy of Fallot, Ebstein's anomaly, and Glenn anastomosis who underwent transcatheter pulmonary valve implantation for severe pulmonary insufficiency.

Transcatheter Closure of Aortopulmonary Window in Infants with Amplatzer Duct Occluder-I.

Aortopulmonary window (APW) is a septation defect between the ascending aorta and main pulmonary artery, and it accounts for 0.2-0.6% of all congenital heart diseases. The diagnosis is made by detecting the defect between the ascending aorta and pulmonary artery while both semilunar valves are fully developed. Based on the anatomical characteristics, APW is classified into four types: proximal (Type 1) defect, distal (Type 2) defect, total (Type 3) defect and intermediate (Type 4) defect. APW is traditionally treated by surgery, and there are a few reports about transcatheter APW closure in infancy. Only defects with adequate superior and inferior rims can be considered for device closure. We describe two cases who underwent transcatheter APW closure with the Amplatzer duct occluder-I (ADO-I). Our experience shows that the ADO-I can achieve good results in closure of APW for selected patients.

Cryoablation with an 8-mm-Tip Catheter for Right-Sided Accessory Pathways in Children.

BACKGROUND: Cryoablation is increasingly utilized in children because of its safety profile. Recently, larger catheter tips have been more widely used to improve long-term success rates. The aim of this study was to assess the safety and efficacy of 8-mm-tip catheters for cryoablation of right-sided accessory pathways (APs) in children. METHODS: Electrophysiological procedures were performed using the EnSite™ system (St. Jude Medical Inc., St. Paul, MN, USA). RESULTS: Between July 2010 and July 2014, 54 patients (mean age: 13.1 ± 3.7 years) underwent cryoablation using an 8-mm-tip catheter. In 18 of 54 (33%) patients where an 8-mm-tip catheter was the first-choice catheter, the success rate was 18 of 18 (100%). There was a history of previous failed attempts or recurrence with radiofrequency ablation and/or 6-mm-tip cryoablation in 36 of 54 (67%) patients. The success rate in these patients was 24 of 36 (67%). No fluoroscopy was used in 34 of 54 procedures. The recurrence rate was six of 42 (14%) during a mean follow-up period of 32 ± 15 months. In one patient, transient atrioventricular block occurred. CONCLUSIONS: Cryoablation with an 8-mm-tip catheter for right-sided APs in children who weigh over 40 kg appears to be safe and acutely effective in cases where conventional ablation methods fail and also as a first choice for ablation procedure. However, the recurrence rate still seems to be high.

Evaluation of Permanent or Transient Complete Heart Block after Open Heart Surgery for Congenital Heart Disease.

BACKGROUND: The features of pediatric patients with postoperative transient and permanent complete atrioventricular (AV) block (CAVB) were compared. METHODS: Patients who developed CAVB in postoperative period after congenital cardiac surgery between 2010-2015 were included in the study. They were classified as patients with transient CAVB and with permanent CAVB. The demographics and perioperative and postoperative variables of the groups were evaluated. RESULTS: A total of 1,550 patients underwent surgery during the study period. CAVB was determined in 96 patients (6.2%) in the early postoperative period: 66 had transient CAVB, 30 had permanent CAVB that necessitated pacemaker implantation. The median body weight and age at surgery were similar in both groups. The most frequent diagnosis was tetralogy of Fallot (TOF, n = 22), complete AV septal defect (AVSD, n = 15), and ventricular septal defect (n = 13). Junctional ectopic tachycardia (JET) developed in 27 patients with transient CAVB and in four with permanent CAVB (P < 0.05). There were no significant differences in the congenital cardiac pathology, the cardiopulmonary bypass time, cross-clamp time, and the presence of preoperative arrhythmia between the groups (P > 0.05). The duration of intensive care unit stay was 6 days (range 2-25) for patients with transient CAVB and 13 days (range 4-90) for patients with permanent CAVB. The duration of hospital stay was 10 days (range 2-33) for patients with transient CAVB and 20 days (range 10-90) for patients with permanent CAVB. Both were significantly longer in the patients with permanent CAVB. CONCLUSIONS: Complete AVSD and TOF are the most risky operations for the development of postoperative AV block. Ninety-seven percent of the patients with transient CAVB regained AV conduction within the first 10 postoperative days. The high incidence of JET in patients with transient CAVB was striking.

Evaluation rhythm problems in unexplained syncope etiology with implantable loop recorder.

BACKGROUND: Syncope is a frequent complaint in children and adolescents and may be a significant sign of serious pathology. Although patient history, family history, and physical examination are sufficient to reach a diagnosis in most cases of syncope, the cause of syncope still cannot be determined after initial investigation in one-third to half of all patients. The aim of this study was to evaluate the diagnostic yield of implantable loop recorder (ILR) in children with unexplained syncope. METHODS: A retrospective review was carried out of clinical data, indications, findings, and a final management strategy in patients who underwent ILR implantation. RESULTS: A total of 12 patients with a mean age of 9.4 ± 4.5 years underwent ILR (Reveal Plus; Medtronic) implantation. ILR implantation indication was syncope in all of the patients. Family history, routine cardiac assessment, including resting 12-lead electrocardiogram, transthoracic echocardiography, 24 h Holter recording, and event recorder findings, were normal with the exception of one patient with (previously corrected) tetralogy of Fallot. After an average of 20 months (range, 1-36 months), six patients developed symptoms. ILR memory showed torsades de pointes-ventricular fibrillation (n = 3), catecholaminergic polymorphic ventricular tachycardia (n = 1), asystole and ventricular tachycardia (n = 1), and normal sinus rhythm (n = 1). At the time of writing six patients were still in follow up with no symptoms after an average of 25.2 months. CONCLUSION: Implantable loop recorder plays an important role in the diagnosis of life-threatening arrhythmias in which syncope is otherwise unexplained. ILR implantation should be remembered in children whose symptoms are strongly correlated with rhythm disturbances.