3D transvenous radiofrequency ablation of manifest epicardial posterior-septal accessory pathways in children: Can technology innovations improve the outcome?

AbstractIntroduction: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

A decade of insertable cardiac monitors with remote monitoring in pediatric patients

Background: Remote monitoring-enabled insertable cardiac monitors (ICMs) are useful tools for arrhythmias and symptom management. This study sought to evaluate the outcome of ICM implantation in a large, heterogeneous cohort of pediatric and young adult patients. Methods: Single centre, retrospective analysis of patients who underwent ICM implantation in 2010–2019. Patients were analysed according to age, symptoms, arrhythmias and underlying heart disease. Results: A total of 200 consecutive patients (58% male), aged 11.5 ± 5.8 years at ICM implantation, were included. Follow-up was 31 ± 18 months. Electrophysiologic study (EPS) was initially performed in 123 patients and was negative in 85%. Patients had no heart disease (57.5%), congenital heart defects (21%), channelopathies (14.5%), cardiomyopathies/heart tumors (8%). The commonest symptoms were syncope/presyncope (45.5%) and palpitations (12.5%). A definite diagnosis was made in 63% of patients (positive diagnosis in 25%, negative in 38%) after 8 (2–19) months of monitoring. EPS results and the presence/absence of an arrhythmia before ICM implantation had no impact on the diagnostic yield. Symptomatic patients as well as patients without structural heart disease showed higher diagnostic yield. Patients with a positive diagnosis underwent pacemaker/implantable cardioverter-defibrillator implantation (13%), pharmacological treatment (10.5%), or catheter ablation (1.5%). Conclusions: In a large cohort of 200 children and young adults, ICMs with remote monitoring showed a high diagnostic yield (63%), especially in symptomatic patients and in patients without structural heart disease

Clinical characteristics and risk of arrhythmic events in patients younger than 12 years diagnosed with Brugada syndrome

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

Anomalous venoatrial connections – CT and MRI assessment

Abnormal venous atrial (VA) connections present a congenital heart disease (CHD) challenge for pediatric cardiologists. Fully anatomical evaluation is very difficult in prenatal and perinatal follow-up, but it has a profound impact on surgical correction and outcome. The echocardiogram is first-line imaging and represents the gold standard tool for simple abnormal VA connection. CT and MRI are mandatory for more complex heart disease and “nightmare cases”. 3D post-processing of volumetric CT and MRI acquisition helps to clarify anatomical relationships and allows for the creation of 3D printing models that can become crucial in customizing surgical strategy. Our article describes a ten-year (2013–2022) tertiary referral CHD center of abnormal AV connections investigated with CT and MRI, illustrating most of these complex diseases with the help of volume rendering (VR) or multiplanar reconstructions (MPR). The nightmarish cases will also be addressed due to the complex cardiovascular arrangement that requires a challenging surgical solution for correction along with the post-surgical complications.</p

Correction to: Heart rate variability as possible marker of brain damage in neonates with hypoxic ischemic encephalopathy: a systematic review.

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

Heart rate variability as possible marker of brain damage in neonates with hypoxic ischemic encephalopathy: a systematic review.

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

Myocardial and Arrhythmic Spectrum of Neuromuscular Disorders in Children

Neuromuscular disorders (NMDs) are highly heterogenous from both an etiological and clinical point of view. Their signs and symptoms are often multisystemic, with frequent cardiac involvement. In fact, childhood onset forms can predispose a person to various progressive cardiac abnormalities including cardiomyopathies (CMPs), valvulopathies, atrioventricular conduction defects (AVCD), supraventricular tachycardia (SVT) and ventricular arrhythmias (VA). In this review, we selected and described five specific NMDs: Friedreich’s Ataxia (FRDA), congenital and childhood forms of Myotonic Dystrophy type 1 (DM1), Kearns Sayre Syndrome (KSS), Ryanodine receptor type 1-related myopathies (RYR1-RM) and Laminopathies. These changes are widely investigated in adults but less researched in children. We focused on these specific topics due their relative frequency and their potential unexpected cardiac manifestations in children. Moreover these conditions present different inheritance patterns and mechanisms of action. We decided not to discuss Duchenne and Becker muscular dystrophies due to extensive work regarding the cardiac aspects in children. For each described NMD, we focused on the possible cardiac manifestations such as different types of CMPs (dilated-DCM, hypertrophic-HCM, restrictive-RCM or left ventricular non compaction-LVNC), structural heart abnormalities (including valvulopathies), and progressive heart rhythm changes (AVCD, SVT, VA). We describe the current management strategies for these conditions. We underline the importance, especially for children, of a serial multidisciplinary personalized approach and the need for periodic surveillance by a dedicated heart team. This is largely due to the fact that in children, the diagnosis of certain NMDs might be overlooked and the cardiac aspect can provide signs of their presence even prior to overt neurological diagnosis

Cardiopulmonary response to exercise and cardiac assessment in patients with turner syndrome.

Turner syndrome (TS) is a chromosomal disorder; however, little is known about the exercise tolerance of patients with this syndrome. The aim of the present study was to measure the maximal aerobic capacity and cardiac function using cardiopulmonary exercise testing and lung function tests and to evaluate the cardiac parameters using echocardiography in patients with TS and control subjects. A total of 50 women with TS (mean age 21.3 +/- 8.5 years) and 56 age-matched controls (mean age 21.1 +/- 3.7 years) were enrolled from the Pediatric Department of "Sapienza" University of Rome and underwent cardiopulmonary exercise testing, lung function testing, and echocardiography. The maximal oxygen uptake was lower in the patients with TS than in the controls (28.4 +/- 4.0 vs 35.6 +/- 6.2 ml/min/kg; p < 0.0001). Also, the forced expiratory volume in 1 second, expressed as a percentage of the predicted value, was greater in the patients with TS than in the controls (116.2 +/- 15.2% vs 102.8 +/- 4.8%, p < 0.0001). The patients with TS had a smaller left ventricle than did the controls. Tissue Doppler imaging revealed subclinical systolic and diastolic dysfunction in the left ventricle in those with TS but not in the controls. The left ventricular mass index was greater in the patients with TS than in the controls (38.6 +/- 9.3 vs 27.2 +/- 4.5 g/m(2.7), p < 0.0001). In conclusion, the patients with TS had a lower maximal aerobic capacity and exercise tolerance than did the controls. The anatomic and functional cardiac aspects were peculiar to those with TS and might represent a specific cardiac phenotype. (C) 2011 Published by Elsevier Inc. (Am J Cardiol 2011;107:1076-1082

Physiological pacing in young patients with complex congenital heart defects

Aim: Young patients with operated complex congenital heart defects (CHD) often develop sinus node dysfunction (SND) requiring permanent pacing with rate-responsive function. Activity-driven sensors cannot account for nonmovement stress and cannot modulate heart rate physiologically. Closed Loop Stimulation (CLS, Biotronik, Berlin, Germany) is a physiological rate-responsive pacemaker based on the indirect measure of ventricular contractility. No data are available on the effects of such pacing strategy in young patients. Methods: We report a series of nine patients with CHD and SND who underwent single-chamber CLS-atrial pacing with endocardial or epicardial lead. During the first 30 days, the pacemaker was programmed in AAI pacing mode and then was switched to CLS-atrial pacing mode. An in-hospital control was scheduled 1–2 months later to evaluate the CLS response to neurovegetative stresses (i.e., nonmovement stress [Stroop color test, handgrip] and exercise stress test) and Holter monitor. CLS pacing was compared with rate-responsive accelerometer-driven pacing (AAIR). Results: At telemetric interrogation, CLS pacing showed a more physiological pattern of 24-h heart rate trends than accelerometer sensors. The data obtained during nonmovement/exercise stress demonstrated a physiological increase in the pacing rate with CLS, in synergy with spontaneous events. The accelerometer sensor histogram, during nonmovement stress, showed a “nonresponse” behavior (only lower rate events), and during exercise test showed most events in lower rate range. Holter monitoring showed increase of average and maximum heart rate compared with AAIR. Conclusion: In young CHD patients, endocardial/epicardial CLS-atrial pacing demonstrated a physiological response of heart rate to neurovegetative and physical stresses