Establishing a nationwide pediatric heart transplantation program with mid-term results comparable to worldwide data - The Czech experience.

BACKGROUND: Heart transplantation (HTx) is an established therapeutic option for children with end-stage heart failure. Comprehensive pediatric nationwide HTx program was introduced in 2014 in the Czech Republic. The aim of this study was to evaluate its mid-term characteristics and outcomes and to compare them with international data. METHODS: Retrospective observational study, including all patients who underwent HTx from June 2014 till December 2022. Data from the institutional database were used for descriptive statistics and survival analyses. RESULTS: A total of 30 HTx were performed in 29 patients with congenital heart disease (CHD, N = 15, single ventricular physiology in 10 patients) and cardiomyopathy (CMP, N = 14). Ten patients were bridged to HTx by durable left ventricular assist devices (LVADs) for a mean duration of 104 (SD 89) days. There was one early and one late death during median follow-up of 3.3 (IQR 1.3-6.1) years. Survival probability at 5 years after HTx was 93%. Two patients underwent re-transplantation (one of them in an adult center). Significant rejection-free survival at 1, 3, and 6 years after HTx was 76%, 63%, and 63%, respectively. CONCLUSIONS: The introduced pediatric HTx program reflects the complexity of the treated population, with half of the patients having complex CHD and one-third being bridged to HTx by LVADs. Mid-term results are comparable to worldwide data. The data confirm the possibility of establishing a successful nationwide pediatric HTx program in a relatively small population country with well-developed pediatric cardiovascular care and other transplantation programs.

First Real-Life Data on the Diving Response in Healthy Children.

Swimming and diving are popular recreational activities, representing an effective option in maintaining and improving cardiovascular fitness in healthy people. To date, only little is known about the cardiovascular adaption to submersion in children. This study was conducted to improve an understanding thereof. We used a stepwise apnea protocol with apnea at rest, apnea with facial immersion, and at last apnea during whole body submersion. Continuous measurement of heart rate, oxygen saturation, and peripheral resistance index was done. Physiologic data and analysis of influencing factors on heart rate, oxygen saturation, and peripheral vascular tone response are reported. The current study presents the first data of physiologic diving response in children. Data showed that facial or whole body submersion leads to a major drop in heart rate, and increase of peripheral resistance, while the oxygen saturation seems to be unaffected by static apnea in most children, with apnea times of up to 75 s without change in oxygen saturation.

Clinical experience of reoperative right ventricular outflow tract reconstruction with valved conduits: risk factors for conduit failure in long-term follow-up.

Reconstruction of right ventricular outflow tract in patients with congenital heart disease in various age groups remains a controversial issue. Currently, a little is known about the fate of secondary and subsequent conduit. The aim of the study was to determine risk factors of conduit failure, evaluate long-term conduit survival, find out which type of conduit should be preferred in case of reoperations. We performed a retrospective analysis of a total of 249 records of valved conduit secondary and subsequent replacement in right ventricular outflow tract in 197 patients. Median follow-up was 5.7 years. The study endpoints were defined as conduit explants; balloon dilatation of the graft (excluding balloon dilatation of left/right pulmonary artery), transcatheter pulmonary valve implantation; heart transplantation or death of the patient. There were total of 21 deaths (11% mortality) among 197 patients during the follow-up, 2 patients underwent heart transplant, in 23 implanted conduits pulmonary angioplasty or/including transcatheter pulmonary valve implantation was afterwards performed due to graft failure, conduit had to be explanted in 46 cases. After 28 years follow-up, freedom from graft failure after 5 years was 77%, 48% after 10 years and 21% after 15 years. Reoperative right ventricular outflow tract reconstruction demonstrates good mid-term and acceptable long-term outcomes regardless of the type of conduit implanted. Worse long-term graft survival of secondary and further conduits is associated with younger age of the recipient at implantation, small size of the conduit, younger age of donor and male donor in case of allograft implantation.

Coherent mapping of atrial tachycardias in patients with congenital heart disease.

AIMS: Coherent mapping (CM) uses a new algorithm to identify critical conduction isthmuses of atrial tachycardias (ATs). We analysed our experience of ablation of AT in patients with congenital heart disease (CHD) with this new technology. METHODS AND RESULTS: All patients with CHD who had CM of AT using the high-density mapping PENTARAY™ catheter and three-dimensional electroanatomic mapping system Carto3 between June 2019 and June 2021 were included retrospectively (n = 27). As a control group, 27 patients with CHD and mapping of AT without CM between March 2016 and June 2019 were included. In total, 54 ablation procedures were performed in 42 patients [median age 35 (interquartile range, IQR 30-48) years] and 64 ATs were induced and mapped (thereof 50 AT intraatrial re-entrant tachycardia and 14 AT ectopic AT). The median procedure duration was 180 (120-214) min and median fluoroscopy time was 10 (5.2-14) min. Acute success was 100% (27/27) in the Coherence group and 74% (20/27) in the non-Coherence group (P = 0.01). During follow-up [median 26 (12-45) months], AT recurred in 28/54 patients, thereof 15 patients needed a re-ablation procedure. Log-rank test showed no difference in recurrence rate between the two groups (P = 0.29). Three minor complications occurred (5.5%). CONCLUSION: Mapping of AT in patients with CHD using the PENTARAY™ mapping catheter and the CM algorithm led to excellent acute success. All ATs were possible to map and no complications related to the PENTARAY™ mapping catheter were observed. Thus, the use of the CM algorithm represents a promising tool in patients with CHD and complex AT.

Electroanatomical mapping-guided left bundle branch area pacing in patients with structural heart disease and advanced conduction abnormalities.

AIMS: Left bundle branch area pacing (LBBAP) can be technically challenging and fluoroscopy-intense. Three-dimensional electroanatomical mapping (EAM) facilitates non-fluoroscopic lead navigation and electrogram mapping. We sought to prospectively evaluate the feasibility, safety, and outcomes of routine EAM-guided LBBAP in patients with structural heart disease (SHD) and advanced conduction abnormalities. METHODS AND RESULTS: Consecutive patients with SHD and conduction abnormalities who underwent an attempt at EAM-guided LBBAP were included. The feasibility, safety, procedural, and mid-term outcomes were evaluated. Electrical, echocardiographic, and clinical parameters were assessed at implantation and last follow-up. Thirty-two patients (68 ± 18 years; 19% female) were included, of which 75% had intrinsic QRS > 150 ms, 53% left bundle branch block, and 25% right bundle branch block. Primary EAM-guided LBBAP was successful in 29 patients (91%). The procedural duration was 95 (70-110) min, total fluoroscopy time 0.93 (0.40-1.73) min, and total fluoroscopy dose 35.4 (20.5-77.2) cGy cm2. Paced QRS duration (QRSd) was significantly shorter than intrinsic QRSd (121.9 ± 10.7 vs. 159.2 ± 34.4 ms; P < 0.001) and remained stable during the mean follow-up of 7.0 ± 5.9 months. The LBBAP capture threshold was 0.57 ± 0.23 V/0.4 ms at implantation and remained low during follow-up (0.58 ± 0.18 V/0.5 ± 0.2 ms; P = 0.877). Overall left ventricular ejection fraction improved significantly from 44.2 ± 14.3% at baseline to 49.4 ± 13.1% at follow-up (P = 0.009), New York Heart Association class from 2.4 ± 0.6 to 1.8 ± 0.6 (P = 0.002), respectively. No complications occurred that required intervention. CONCLUSION: Routine near-zero fluoroscopy EAM-guided LBBAP can safely be performed in patients with SHD and advanced conduction abnormalities with high success rates and favourable mid-term outcomes. Further studies are needed to investigate whether the use of EAM improves the overall outcome of conduction system pacing and to identify specific patient populations who benefit the most from EAM-guided lead implantation.

The SIDECAR project: S-IcD registry in European paediatriC and young Adult patients with congenital heaRt defects.

AIMS: Subcutaneous-implantable cardiac defibrillators (S-ICDs) are used increasingly to prevent sudden cardiac death in young patients. This study was set up to gain insight in the indications for S-ICD, possible complications, and their predictors and follow-up results. METHODS AND RESULTS: A multicentre, observational, retrospective, non-randomized, standard-of-care registry on S-ICD outcome in young patients with congenital heart diseases (CHDs), inherited arrhythmias (IAs), idiopathic ventricular fibrillation (IVF), and cardiomyopathies (CMPs). Anthropometry was registered as well as implantation technique, mid-term device-related complications, and incidence of appropriate/inappropriate shocks (IASs). Data are reported as median (interquartile range) or mean ± standard deviation. Eighty-one patients (47% CMPs, 20% CHD, 21% IVF, and 12% IA), aged 15 (14-17) years, with body mass index (BMI) 21.8 ± 3.8 kg/m2, underwent S-ICD implantation (primary prevention in 59%). This was performed with two-incision technique in 81% and with a subcutaneous pocket in 59%. Shock and conditional zones were programmed at 250 (200-250) and 210 (180-240) b.p.m., respectively. No intraoperative complications occurred. Follow up was 19 (6-35) months: no defibrillation failure occurred, 17% of patients received appropriate shocks, 13% of patients received IAS (supraventricular tachycardias 40%, T-wave oversensing 40%, and non-cardiac oversensing 20%). Reprogramming, proper drug therapy, and surgical revision avoided further IAS. Complications requiring surgical revision occurred in 9% of patients, with higher risks in patients with three-incision procedures [hazard ratio (HR) 4.3, 95% confidence interval (95% CI) 0.5-34, P = 0.038] and BMI < 20 (HR 5.1, 95% CI 1-24, P = 0.031). CONCLUSION: This multicentre European paediatric registry showed good S-ICD efficacy and safety in young patients. Newer implantation techniques and BMI > 20 showed better outcome.

Evaluation of Clinical Course and Maintenance Drug Treatment of Supraventricular Tachycardia in Children During the First Years of Life. A Cohort Study from Eastern Germany.

Supraventricular tachycardia (SVT) is considered the most common cause of arrhythmia in children and infants. Regarding the likelihood of a spontaneous resolution of SVTs during the first years of life, drug treatment aims to bridge the time until children 'grow out' out of the arrhythmia. The choice of antiarrhythmic agents and the planning of maintenance therapy are mainly based on clinical experience and retrospective single- and multi-institutional analyses and databases from all over the world approaching differently to this topic. The current study aimed to evaluate the clinical course, pharmacological treatment strategies, and constellations of risk for recurrences in the management of SVTs in children aged 3 < years. The database of the Heart Center Leipzig, Department of Pediatric cardiology, was searched for pediatric patients aged < 3 years with a clinically documented SVT between 2000 and 2019 that received pharmacologic treatment. Patients with complex congenital heart disease or arrhythmias following cardiac surgery were excluded. 69 patients were included. Pharmacologic treatment, follow-up schedule, recurrences, outcomes, and risk factors for complicated courses are reported. Drug therapy of SVTs in young children remains a controversial topic with heterogeneous treatment and follow-up strategies applied. Risk factors for recurrences and/or stubborn clinical courses are difficult rhythm control with 3 or more antiarrhythmic drugs, ectopic atrial tachycardias, and a first occurrence of the SVT in the fetal period. Prospective studies are needed to sufficiently evaluate optimal treatment strategies.

Accuracy of the Apple Watch single-lead ECG recordings in pre-term neonates.

INTRODUCTION: Telemedicine gained an increasing use throughout the last years. Lifestyle tools like the Apple watch seem to have an increasing spread even in remote areas and underdeveloped regions. The increasing availability of these tools offers the chance to use the health care functions of these devices to improve provision of professional medical care. First data on the use of the Apple Watch as a remote monitoring device in children have been reported, showing good acceptability and usability of the Apple Watch for symptom monitoring in children. This study aimed to evaluate the accuracy of the Apple Watch iECG in comparison to a standard 12-lead ECG in pre-term babies. METHODS: In this prospective, single-arm study, consecutive preterm neonates hospitalised in Leipzig University Hospital neonatal ICU were eligible. A 12-lead ECG and an iECG using Apple Watch 4 were performed. iECG and 12-lead ECG measurements were performed by a paediatric cardiologist. Cardiac rhythm was classified and amplitudes and timing intervals were analysed for comparability. RESULTS: Fifty preterm neonates, gestational week (23-36 weeks), and body weight (0.65-3.09 kg) were enrolled. Overall good quality and excellent correlation of the Apple Watch generated iECG in comparison to the standard 12-lead ECG could be demonstrated (p < 0.001). When interpreted by a paediatric cardiologist, a correct rhythm classification could be done in 100% of cases. CONCLUSION: The Apple Watch iECG seems to be a valuable tool to record an ECG comparable to lead I of the standard 12-lead ECG even in pre-term neonates. With a widespread availability and excellent connectivity, the Apple Watch iECG function may provide practitioners with a tool to send an iECG for interpretation to a paediatric cardiac specialist.

Open field stress testing: finally an optimal method in young children? Reference values for mobile cardiopulmonary exercise testing in healthy children aged 4-8 years.

INTRODUCTION: Cardiopulmonary exercise testing represents the diagnostic tool for determining cardiopulmonary function. Especially in small children, exercise testing is extremely challenging. To address this problem, field testing has been implemented using small mobile devices. This study aims at using this protocol for developing normal values for cardiopulmonary exercise testing in very young children. MATERIAL AND METHODS: Healthy children aged 4-8 years were recruited. All children were tested according to an outdoor protocol, in which they were instructed to walk, then run slowly, then a little harder and at last run at full speed. Each step lasted for 2 minutes, except the last step, in which the children were instructed to maintain as long as possible. RESULTS: A total of 104 children (64 female/35 male, mean age 6.6 years) performed outdoor cardiopulmonary exercise testing using a mobile device. Almost all tests were completed successfully (95%). Despite a predominance of female study subjects, anthropometric values did not differ between boys and girls. V̇O2peak/kg, respiratory exchange ratio, VT1, heart rate at VT1, and time of exercise were also comparable between sexes. Generally, a tendency of higher maximal oxygen uptake could be observed in older children. CONCLUSION: Open field mobile cardiopulmonary exercise testing represents a novel approach in very young children. In this study, we were able to determine normal values of maximal oxygen uptake and OUES/kg for 4-8-year-old children. The method is easy to achieve and safe.

Use of the Apple Watch iECG in adult congenital heart disease patients.

INTRODUCTION: This study evaluates the accuracy of iECGs in comparison to the gold standard ECG in adult patients with congenital heart disease and recommends the appropriate iECG derivation based on the patient's characteristics. METHODS: In 106 adults (51 female, 55 male) with congenital heart disease, a gold standard 12-lead ECG was recorded, followed by three iECGs with the Apple Watch series 4, which correspond to Einthoven leads I, II, and III. Two experienced and independent cardiologists analyzed the time intervals, amplitudes, and polarities of the ECG parameters as well as the rhythm type and correlated the patient characteristics with the iECG parameters. RESULTS: The iECG parameters of all three iECG leads correlate strongly with those of the gold standard ECG, with exception of the P and T wave durations. We demonstrated that the informative value of the individual iECGs was independent of the patient's characteristics, in particular the heart axis, anatomy, and situs, even if the quality of the Einthoven III-like derivation was partially inadequate. The automatic rhythm analysis of the Apple Watch and the heart rhythm classification of a standard ECG analyzed manually by a cardiologist corresponded in 77%. CONCLUSION: iECG recordings of adults with congenital heart disease provide comparable results with Einthoven recordings I, II, and III of the 12-lead ECG and current data encourage the use of the Apple Watch not only in patients with structurally normal hearts but also in patients with congenital heart disease.

Impact of electroanatomical mapping-guided lead implantation on procedural outcome of His bundle pacing.

AIMS: Conventional His bundle pacing (HBP) can be technically challenging and fluoroscopy-intense, particularly in patients with His-Purkinje conduction disease (HPCD). Three-dimensional electroanatomical mapping (EAM) facilitates non-fluoroscopic lead navigation and HB electrogram mapping. We sought to assess the procedural outcome of routine EAM-guided HBP compared with conventional HBP in a real-world population and evaluate the feasibility and safety of EAM-guided HBP in patients with HPCD. METHODS AND RESULTS: We included 58 consecutive patients (72 ± 13 years; 71% male) who underwent an attempt to conventional (EAM- group; n = 29) or EAM-guided (EAM+ group; n = 29) HBP between June 2019 and April 2020. The centre's learning curve was initially determined (n = 40 cases) to define the conventional control group and minimize outcome bias favouring EAM-guided HBP. His bundle pacing was successful in 26 patients (90%) in the EAM+ and 27 patients (93%) in the EAM- group (P = 0.64). The procedure time was 90 (73-135) and 110 (70-130) min, respectively (P = 0.89). The total fluoroscopy time [0.7 (0.5-1.4) vs. 3.3 (1.4-6.5) min; P < 0.001] and fluoroscopy dose [21.9 (9.1-47.7) vs. 78.6 (27.2-144.9) cGycm2; P = 0.001] were significantly lower in the EAM+ than EAM- group. There were no significant differences between groups in His capture threshold (1.2 ± 0.6 vs. 1.4 ± 1.0 V/1.0 ms; P = 0.33) and paced QRS duration (113 ± 15 vs. 113 ± 17 ms; P = 0.89). In patients with HPCD, paced QRS duration was similar in both groups (121 ± 15 vs. 123 ± 12 ms; P = 0.77). The bundle branch-block recruitment threshold tended to be lower in the EAM+ than EAM- group (1.3 ± 0.7 vs. 1.8 ± 1.2 V/1.0 ms; P = 0.31). No immediate procedure-related complications occurred. One patient (2%) experienced lead dislodgement during 4-week follow-up. CONCLUSION: Implementation of routine EAM-guided HBP lead implantation is feasible and safe in a real-world cohort of patients with and without HPCD and results in a tremendous reduction in radiation exposure without prolonging procedure time or increasing procedure-related complications.

Pediatric catheter ablation at the beginning of the 21st century: results from the European Multicenter Pediatric Catheter Ablation Registry 'EUROPA'.

AIMS: Contemporary data from prospective multicentre registries on catheter ablation in pediatric patients are sparse. Aim of the European Pediatric Catheter Ablation Registry EUROPA was to contribute data to fill this gap of knowledge. METHODS AND RESULTS: From July 2012 to June 2017, data on catheter ablation in pediatric patients (≤18 years of age) including a 1-year follow-up from five European pediatric EP centres were collected prospectively. A total of 683 patients (mean age 12.4 ± 3.9 years, mean body weight 50.2 ± 19 kg) were enrolled. Target tachycardia was WPW/atrioventricular-nodal re-entrant tachycardia (AVRT) in 380 (55.7%) patients, AVNRT in 230 (33.8%) patients, ventricular tachycardia (VT) in 24 (3.5) patients, focal atrial tachycardia (FAT) in 20 (2.9%) patients, IART in 14 (2%) patients, and junctional ectopic tachycardia in 3 (0.45) patients. Overall procedural success was 95.6%. Compared with all other substrates, success was significantly lower in FAT patients (80%, n = 16, P = 0.001). Mean procedure duration was 136 ± 67 min and mean fluoroscopy time was 4.9 ± 6.8 min. Major complications occurred in 0.7% of the patients. No persisting AV block requiring permanent pacing was reported. At 1-year follow-up (605/683 patients, 95%), tachycardia recurrence was reported in 7.8% of patients. Recurrence after VT ablation (33%) was significantly higher (P = 0.001) than after ablation of all other substrates. CONCLUSION: The present study proves overall high efficacy and safety of catheter ablation of various tachycardia substrates in pediatric patients. Of note, complication rate was exceptionally low. Long-term success was high except for patients after VT ablation.

Recommendations from the Association for European Paediatric and Congenital Cardiology for training in diagnostic and interventional electrophysiology.

The field of electrophysiology (EP) in paediatric cardiology patients and adults with congenital heart disease is complex and rapidly growing. The current recommendations for diagnostic and invasive electrophysiology of the working group for Cardiac Dysrhythmias and Electrophysiology of the Association for European Paediatric and Congenital Cardiology acknowledges the diveristy of European countries and centers. These training recommendations can be fulfilled in a manageable period of time, without compromising the quality of training required to become an expert in the field of paediatric and congenital EP and are for trainees undergoing or having completed accredited paediatric cardiologist fellowship. Three levels of expertise, the training for General paediatric cardiology EP, for non-invasive EP and invasive EP have been defined. This Association for European EP curriculum describes the theoretical and practicsal knowledge in clinical EP; catheter ablation, cardiac implantable electronic devices, inherited arrhythmias and arrhythmias in adults with congenital heart defects for the 3 levels of expertise.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary.

Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984 [1]. CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay [2], which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document [3], with further data easily accessible in electronic searches or textbooks.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

Performance of pacemaker leads in alternative lead positions after tricuspid valve replacement.

BACKGROUND: Bradycardic arrhythmias requiring pacemaker (PM) implantation are still common in patients in need of tricuspid valve replacement (TVR). Leaving an existing PM lead in an extravalvular position may represent a helpful alternative in special situations like the implantation of a mechanical TV. This study aimed to examine the short- to mid-term outcome of paravalvular leads concerning lead survival and prosthesis dysfunction in patients after TVR. METHODS: A retrospective case-control study of patients with TVR and ventricular pacing was conducted. Patients from the database of the Leipzig Heart Center were included. Data of the paravalvular lead group (PVG) and coronary sinus lead group (CSG) were compared to a control group with conventional transvalvular leads (TVG). RESULTS: Eighty patients with TVR and cardiac PM (TVG [n = 13], PVG [n = 40], and CSG [n = 27]) were included. The mean follow-up was 2.8 years. The rate of lead revisions (TVG 15.4%, PVG 2.5%, and CSG 7.5%) was lower in PVG but without significance (P = .286). The CSG demonstrated significantly higher pacing thresholds (1.4 V/0.8 ms) than TVG (0.5 V/0.4 ms), P = .004. However, the deterioration of threshold amplitudes during follow-up was similar in CSG (7.4%) and PVG (7.5%) compared with controls (7.7%). Function of TV prosthesis regarding development of stenosis or regurgitation showed a similarity between the groups (regurgitation PVG P = .692, CSG P = 1; stenosis PVG P = .586, CSG P = 0.69). CONCLUSION: Paravalvular positioning of PM leads seems to represent a reasonable alternative to the conventional transvalvular lead positioning concerning the lead and Tricuspid Valve prosthesis's function and durability in selected patients.

Quality of life and psychological co-morbidities in children and adolescents with cardiac pacemakers and implanted defibrillators: a cohort study in Eastern Germany.

INTRODUCTION: The implantation of a pacemaker or an implantable cardioverter-defibrillator during childhood may reduce quality of life and lead to mental health problems. This study aimed to evaluate potential mental health problems (i.e., depressive and anxiety symptoms) and quality of life in children with cardiac active devices in comparison to healthy peers. METHODS: We analysed data of children with pacemakers or implantable cardioverter-defibrillators aged 6-18 years. Quality of life, depressive and anxiety symptoms were assessed by standardised questionnaires. The results were compared to age-matched reference groups. RESULTS: Children with implantable cardioverter-defibrillator showed significant lower quality of life in comparison to reference group (p = 0.03), but there was no difference in quality of life between children with pacemaker and reference group. There was no significant difference in depressive symptoms between children with a cardiac rhythm device compared to reference group (self-report: p = 0.67; proxy report: p = 0.49). There was no significant difference in anxiety (p = 0.53) and depressive symptoms (p = 0.86) between children with pacemaker and children with implantable cardioverter-defibrillator. CONCLUSIONS: Living with an implantable cardioverter-defibrillator in childhood seems to decrease the patients' quality of life. Although children with pacemaker and implantable cardioverter-defibrillator don't seem to show more depressive and anxiety symptoms in comparison to their healthy peers, there still can be an increased risk for those children to develop mental health problems. Therefore, treating physicians should be aware of potential mental health problems and provide the patients and their families with appropriate therapeutic offers.

A practical approach to the examination of the congenitally malformed heart at autopsy.

Congenital heart defects (CHD) represent the most frequent type of the heart disease in childhood, with incidence up to 1 % of all live-born children. Despite the improving echocardiographic diagnostics, part of CHD remains undiagnosed and can manifest in the later age or may be the cause of the early abortion. On the other hand, some foetuses with prenatally diagnosed severe CHD may be recommended to interruption. Therefore, each pathologist can encounter a malformed heart at the autopsy. Despite the current quality of the echocardiography, the macroscopic assessment of the heart by the pathologist is still considered the best method for evaluation of the structural heart disease. Knowledge of the basic pathologic anatomy thus remains an important prerequisite for adequately performed paediatric autopsy.

Tree differences in primary and secondary growth drive convergent scaling in leaf area to sapwood area across Europe.

Trees scale leaf (AL ) and xylem (AX ) areas to couple leaf transpiration and carbon gain with xylem water transport. Some species are known to acclimate in AL  : AX balance in response to climate conditions, but whether trees of different species acclimate in AL  : AX in similar ways over their entire (continental) distributions is unknown. We analyzed the species and climate effects on the scaling of AL vs AX in branches of conifers (Pinus sylvestris, Picea abies) and broadleaved (Betula pendula, Populus tremula) sampled across a continental wide transect in Europe. Along the branch axis, AL and AX change in equal proportion (isometric scaling: b Ëœ 1) as for trees. Branches of similar length converged in the scaling of AL vs AX with an exponent of b = 0.58 across European climates irrespective of species. Branches of slow-growing trees from Northern and Southern regions preferentially allocated into new leaf rather than xylem area, with older xylem rings contributing to maintaining total xylem conductivity. In conclusion, trees in contrasting climates adjust their functional balance between water transport and leaf transpiration by maintaining biomass allocation to leaves, and adjusting their growth rate and xylem production to maintain xylem conductance.