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Introduction:The aim of the study was to revise our more recent experience about epicardial posterior-septal accessory pathways radiofrequency transcatheter ablation in children and young patients using a transvenous approach through the coronary sinus, to understand if new mapping and ablation technologies can increase success rate and safety.Methods and results:Twenty children (mean age 13 ± 3 years) with epicardial posterior-septal accessory pathways (14 in coronary sinus and 6 in the middle cardiac vein) underwent radiofrequency transcatheter ablation with CARTO-3® system with help of the CARTO-Univu® module. Acute success rate was 73%. No patient was lost to follow-up (mean time 11.4 ± 9 months). The recurrence rate was 19%. Two patients underwent a successful redo-procedure; the overall long-term success rate was 65%. Navistar® catheter presented the highest acute success rate in the coronary sinus. Navistar SmartTouch® was the only catheter that did not present recurrences after the acute success, and it was successfully used in two patients previously unsuccessfully treated with a Navistar ThermoCool®. Acute success rate was 79% without image integration with angio-CT, while it was 63% after the introduction of CARTO-Merge®.Conclusion:Epicardial posterior-septal accessory pathways can be definitively eliminated by transvenous radiofrequency transcatheter ablation in more than half of the cases in children. Acute success rate does not seem to depend on catheters used, but contact-force catheter seems to be useful in cases with recurrences. Image integration with cardiac-CT does not increase success rate, but it is useful to detect coronary sinus alterations to better guide ablation strategy.

Atrial flutter (AFL) in pediatric patients is a rare condition as the physical dimensions of the immature heart are inadequate to support the arrhythmia. This low incidence makes it difficult for patients in this particular setting to be studied. AFL accounts for 30% of fetal tachyarrhythmias, 11% to 18% of neonatal tachyarrhythmias, and 8% of supraventricular tachyarrhythmias in children older than 1 year of age. Transesophageal overdrive pacing can be used, instead, with lower success rate (60%-70%). The recommended drugs are digoxin which can decrease the ventricular rate until the spontaneous interruption of the AFL. Digoxin can be combined with flecainide or amiodarone in case of failure.

Aims The aims of this study are to evaluate the progressive involvement of the cardiac conduction system in the Kearn–Sayre syndrome (KSS) and to establish criteria for the prevention of episodes of syncope or sudden cardiac death. Methods and results This is a prospective monocentric study including KSS patients, with diagnosis based on clinical manifestations, muscle biopsy, and genetic tests, before the age of 18. All patients underwent cardiac screening examination with 12-lead electrocardiogram (ECG), 24-h Holter monitoring, and pacemaker (PM) interrogation twice a year. Fifteen patients (nine males, mean age 16.6 ± 3.9 years) with a sporadic KSS were recruited. All subjects manifested at least one of the intraventricular conduction defects (IVDs): 1 right bundle branch block (RBBB), 2 left anterior fascicular block (LAFB), 11 a bi-fascicular block (RBBB + LAFB), and 1 left posterior fascicular block. Most children with bi-fascicular block developed LAFB before the RBBB (P = 0.0049). In six patients, IVD degenerated into atrioventricular block (AVB). Endocavitary PM was implanted in 11 patients (6 with AVB and 5 with a bi-fascicular block), while an implantable cardioverter-defibrillator only in one patient with a non-sustained ventricular tachycardia. Four died at mean age of 14.7 ± 2.6 years, but none of them suddenly. Conclusion Even a ‘simple’ ECG can predict the arrhythmic risk and the occurrence of catastrophic events in young patients with KSS. Left anterior fascicular block precedes RBBB in determining the bi-fascicular block and this can predict an inexorable progression of the conduction defects even in a short time. Pacemaker implantation may be indicated in these patients since the first bi-fascicular block manifestation.

We report a unique case of a 6-year-old male child with aborted sudden cardiac death due to ventricular fibrillation. A rare anomalous aortic origin of the right coronary artery was detected and supposed to be the cause of the malignant arrhythmia. Clinical exome sequencing did not reveal any pathogenic variant related to channelopathies nor other known heart-related genes. The patient underwent cardiac surgery and a cardiac defibrillator was implanted for secondary prevention.

Aims Different authors have described three-dimensional (3D) voltage mapping of the Koch’s triangle (KT) in order to find low-voltage bridges (LVBs) as targets for a successful transcatheter ablation (TCA) of the slow pathway (SP) in children. Recently, the advisor high density (HD) Grid™ mapping catheter was introduced as new multipolar catheter for HD mapping. The aim of the study was to describe our preliminary experience with the use of HD Grid™ catheter in LVB and electrophysiologically guided cryoablation of SP in children. Methods and results Twenty-one children (mean age 13 ± 3 years) with atrioventricular nodal re-entrant tachycardia (AVNRT) underwent cryoablation of SP guided by voltage HD mapping of the KT using HD Grid™ catheter. In order to better highlight the differences with conventional mapping, point collection was performed in each patient with this new multipolar catheter and with a quadripolar catheter. The conventional mapping collected 871 ± 262 points and used 211 ± 80 points in 887 ± 275 s, whereas HD mapping collected 7468 ± 2947 points, using 604 ± 165 points in 513 ± 181 s (P Conclusions Our preliminary experience shows that HD mapping is faster and offers higher spatial resolution and definition. Procedural time can be reduced maintaining the TCA safe, with reduced fluoroscopy use, and successful.

Aims The aim of the study was to analyse our recent single-centre experience about epicardial posterior-septal accessory pathways transcatheter ablation in children and young patients using radiofrequency through the coronary sinus, in order to understand which mapping and ablation strategy is associated with higher success rate and safety. Methods and results We reviewed all the cases of ablation of overt accessory pathways (in Wolff–Parkinson–White syndrome) with epicardial posterior-septal localization performed in children or young patients at our institution in the last 5 years. Twenty-two paediatric patients (mean age: 13 ± 3 years) with epicardial posterior-septal accessory pathways (15 in coronary sinus and 7 in the Middle Cardiac Vein) underwent radiofrequency transcatheter ablation with CARTO 3TM. Acute success rate was 77%. No patient was lost to follow-up (mean time 14.4 ± 9 months). The recurrence rate was 18%. Two patients underwent a successful redo-procedure; the overall long-term success rate was 68%. NAVISTAR® catheter presented the highest acute success rate in the coronary sinus. NAVISTAR SMARTTOUCH® was the only catheter that did not present recurrences after the acute success and it was successfully used in two patients previously unsuccessfully treated with a NAVISTAR THERMOCOOL®. Integration with angio-CT of coronary sinus branches obtained with CARTOMERGE was associated with higher success rate in patients with a previous failed ablation attempt. Conclusions Epicardial posterior-septal accessory pathways can be successfully treated with transvenous radiofrequency ablation in more than half of the cases in children/young patients. Acute success rate does not seem to depend on catheters used but contact-force catheter seems to be useful in cases with recurrences. Image integration with cardiac-CT reconstruction of coronary sinus branches anatomy can be useful to better guide ablation in case of previously failed attempts.

PURPOSE Different authors have described three-dimensional (3D) voltage mapping of the Koch's triangle (KT) in order to find low-voltage bridges (LVB) as targets for a successful transcatheter ablation (TCA) of the slow pathway (SP) in children. Recently, the Advisor High Density (HD) Grid™ mapping catheter was introduced as new multipolar catheter for HD mapping. The aim of the study was to describe our preliminary experience with the use of HD Grid™ catheter in LVB and electrophysiologically guided cryoablation of SP in children. METHODS Twenty-one children (mean age 13±3 years) with atrioventricular nodal re-entrant tachycardia (AVNRT) underwent cryoablation of SP guided by voltage HD mapping of the KT using HD Grid™ catheter. In order to better highlight the differences with conventional mapping, point collection was performed in each patient with this new multipolar catheter and with a quadripolar catheter. RESULTS The conventional mapping collected 871±262 points and used 211±80 points in 887±275 s, whereas HD mapping collected 7468±2947 points, using 604±165 points in 513±181 s (p

We report a unique case of a 6-year-old male child with aborted sudden cardiac death due to ventricular fibrillation. A rare anomalous aortic origin of the right coronary artery was detected and supposed to be the cause of the malignant arrhythmia. Clinical exome sequencing did not reveal any pathogenic variant related to channelopathies nor other known heart-related genes. The patient underwent cardiac surgery and a cardiac defibrillator was implanted for secondary prevention.

Background Brugada syndrome (BrS) is an inheritable disease with an increased risk of sudden cardiac death. Although several score systems have been proposed, the management of children with BrS has been inconsistently described. Objective The purpose of this study was to identify the characteristics, outcome, and risk factors associated with cardiovascular and arrhythmic events (AEs) in children younger than 12 years with BrS. Methods In this single-center case series, all children with spontaneous or drug/fever-induced type 1 Brugada electrocardiographic (ECG) pattern and younger than 12 years at the time of diagnosis were enrolled. Results Forty-three patients younger than 12 years at the time of diagnosis were included. The median follow-up was 3.97 years (interquartile range 2–12 years). In terms of first-degree atrioventricular block, premature beats, nonmalignant AEs, malignant AEs, and episodes of syncope, no significant differences were observed either between patients with spontaneous and drug/fever-induced type 1 Brugada ECG pattern or between female and male patients (except a significant difference between female and male patients for first-degree atrioventricular block). A higher incidence of malignant AEs was observed in patients with syncope (3 of 8 [37.5%] vs 0 of 35 [0%]; P = .005) than in patients without syncope. SCN5A mutations were associated with a higher occurrence of malignant AEs (3 of 14 [21.4%] vs 0 of 25 [0%]; P = .04) compared with no SCN5A mutations. Conclusion A spontaneous type 1 Brugada ECG pattern is not associated with a higher incidence of syncope, first-degree atrioventricular block, premature beats, nonmalignant AEs, and malignant AEs than the drug/fever-induced type 1 Brugada ECG pattern. Syncope events are correlated with an increased incidence of malignant AEs. Moreover, SCN5A mutations are associated with a higher occurrence of malignant AEs.

Transcatheter ablation (TA) for treatment of pediatric tachyarrhythmias was first introduced in the early 1990s. Since then, its use as an alternative to antiarrhythmic drugs to treat supraventricular tachycardias caused by accessory pathway has been increasing. Nowadays, TA can be performed with high success rates and low complication rates even in very young children. Recently, the use of different types of energy (radiofrequency and cryoenergy), special ablation catheters, and 3D nonfluoroscopic mapping systems equipped with high technological tools has further improved safety and efficacy of TA, especially in difficult substrates and in patients with congenital heart disease.

Heart rate variability (HRV) is currently considered the most valuable non-invasive test to investigate the autonomic nervous system function, based on the fact that fast fluctuations might specifically reflect changes of sympathetic and vagal activity. An association between abnormal values of HRV and brain impairment has been reported in the perinatal period, although data are still fragmentary. Considering such association, HRV has been suggested as a possible marker of brain damage also in case of hypoxic-ischemic encephalopathy following perinatal asphyxia. The aim of the present manuscript was to review systematically the current knowledge about the use of HRV as marker of cerebral injury in neonates suffering from hypoxic-ischemic encephalopathy. Findings reported in this paper were based on qualitative analysis of the reviewed data. Conclusion: A growing body of research supports the use of HRV as non-invasive, bedside tool for the monitoring of hypoxic-ischemic encephalopathy. The currently available data about the role of HRV as prognostic tool in case of hypoxic ischemic encephalopathy are promising but require further validation by future studies. What is Known: • Heart rate variability (HRV) is a non-invasive monitoring technique to assess the autonomic nervous system activity. • A correlation between abnormal HRV and cerebral injury has been reported in the perinatal period, and HRV has been suggested as possible marker of brain damage in case of hypoxic-ischemic encephalopathy. What is New: • HRV might provide precocious information about the entity of brain injury in asphyxiated neonates and be of help to design early, specific, and personalized treatments according to severity. • Further investigations are required to confirm these preliminary data.

Correction to: European Journal of Pediatrics https://doi.org/10.1007/s00431-020-03882-3