Lead Extraction in Children and Young Adults: When is the Best Time for Lead/System Replacement?

The best strategy for lead management in children is a matter of debate, and our experiences are limited. This is a retrospective single-center study comparing difficulties and outcomes of transvenous lead extraction (TLE) implanted ich childhood and at age < 19 years (childhood-implanted-childhood-extracted, CICE) and at age < 19 (childhood-implanted-adulthood-extracted, CIAE). CICE patients-71 children (mean age 15.1 years) as compared to CIAE patients (114 adults (mean age 28.61 years) were more likely to have VVI than DDD pacemakers. Differences in implant duration (7.96 vs 14.08 years) appeared to be most important, but procedure complexity and outcomes also differed between the groups. Young adults with cardiac implantable electronic device implanted in childhood had more risk factors for major complications and underwent more complex procedures compared to children. Implant duration was significantly longer in CIAE patients than in children, being the most important factor that had an impact on patient safety and procedure complexity. CIAE patients were more likely to have prolonged operative duration and more complex procedures due to technical problems, and they were 2-3 times more likely to require second-line or advanced tools compared to children, but the rates of clinical and procedural success were comparable in both groups. The difference between the incidence of major complications between CICE and CIAE patients is very clear (MC 2.9 vs 7.0%, hemopericardium 1.4 vs 5.3% etc.), although statistically insignificant. Delay of lead extraction to adulthood seems to be a riskier option than planned TLE in children before growing up.

Transvenous Lead Extraction in Pediatric Patients　- Is It the Same Procedure in Children as in Adults?

BACKGROUND: Cardiac implantable electronic devices (CIED) are very rare in the pediatric population. In children with CIED, transvenous lead extraction (TLE) is often necessary. The course and effects of TLE in children are different than in adults. Thus, this study determined the differences and specific characteristics of TLE in children vs. adults.Methods and Results: A post hoc analysis of TLE data in 63 children (age ≤18 years) and 2,659 adults (age ≥40 years) was performed. The 2 groups were compared with respect to risk factors, procedure complexity, and effectiveness. In children, the predominant pacing mode was a single chamber ventricular system and lead dysfunction was the main indication for lead extraction. The mean implant duration before TLE was longer in children (P=0.03), but the dwell time of the oldest extracted lead did not differ significantly between adults and children. The duration (P=0.006) and mean extraction time per lead (P<0.001) were longer in children, with more technical difficulties during TLE in the pediatric group (P<0.001). Major complications were more common, albeit not significantly, in children. Complete radiographic and procedural success were significantly lower in children (P<0.001). CONCLUSIONS: TLE in children is frequently more complex, time consuming, and arduous, and procedural success is more often lower. This is related to the formation of strong fibrous tissue surrounding the leads in pediatric patients.

Transvenous Lead Extraction in Adult Patient with Leads Implanted in Childhood-Is That the Same Procedure as in Other Adult Patients?

BACKGROUND: Lead management in children and young adults is still a matter of debate. METHODS: To assess the course of transvenous lead extraction (TLE) in adults with pacemakers implanted in childhood (CIP) we compared 98 CIP patients with a control group consisting of adults with pacemakers implanted in adulthood (AIP). RESULTS: CIP patients differed from AIP patients with respect to indications for TLE and pacing history. CIP patients were four-eight times more likely to require second-line or advanced tools. Furthermore, CIP patients more often than AIP were prone to developing complications: major complications (MC) (any) 2.6 times; hemopericardium 3.2 times; severe tricuspid valve damage 4.4 times; need for rescue cardiac surgery 3.7 times. The rate of procedural success was 11% lower because of 4.8 times more common lead remnants and 3.1 times more frequent permanently disabling complications. CONCLUSIONS: Due to system-related risk factors TLE in CIP patients is more difficult and complex. TLE in CIP is associated with an increased risk of MC and incomplete lead removal. A conservative strategy of lead management, acceptable in very old patients seems to be less suitable in CIP because it creates a subpopulation of patients at high risk of major complications during TLE in the future.

Surgical Outcomes of Total Anomalous Pulmonary Venous Connection Repair.

Background and Objectives: Over the years, surgical repair of total anomalous pulmonary venous connection (TAPVC) outcomes have improved, however, morbidity and mortality still remain significant. This study aims to assess the early and long-term outcomes of surgical treatment of TAPVC children, operated on between 2006 and 2016, in one pediatric center in Poland. Materials and Methods: Diagnostics, surgical treatment, and follow-up data from 83 patients were collected. In addition, survival and risk factor analyses, control echocardiographic, and electrocardiographic examinations were performed. Results: In the analyzed group (n = 83), there were seven hospital deaths (within 30 days after the operation) (8.4%) and nine late deaths (10.8%). The mean follow-up time was 5.5 years, and, for patients who survived, it was 7.1 years. The mean survival time in patients with completed follow-up (n = 70) was 10.3 years; the overall five-year survival rate was 78.4%. Independent mortality risk factors were type I TAPVC, single ventricle physiology, time from admission to operation, intensive care unit stay, postoperative hospital stay, and temporary kidney insufficiency requiring dialysis. Conclusions: The presence of single ventricle physiology and the supracardiac subtype of TAPVC might be negative prognostic factors, while normal heart physiology presents with good post-repair results. This study indicates that cardiac arrhythmias may occur. Morbidity and mortality, related to surgical TAPVC correction, still remain significant.

The Indices of Cardiovascular Magnetic Resonance Derived Atrial Dynamics May Improve the Contemporary Risk Stratification Algorithms in Children with Hypertrophic Cardiomyopathy.

INTRODUCTION: The most efficient risk stratification algorithms are expected to deliver robust and indefectible identification of high-risk children with hypertrophic cardiomyopathy (HCM). Here we compare algorithms for risk stratification in primary prevention in HCM children and investigate whether novel indices of biatrial performance improve these algorithms. METHODS AND RESULTS: The endpoints were defined as sudden cardiac death, resuscitated cardiac arrest, or appropriate implantable cardioverter-defibrillator discharge. We examined the prognostic utility of classic American College of Cardiology/American Heart Association (ACC/AHA) risk factors, the novel HCM Risk-Kids score and the combination of these with indices of biatrial dynamics. The study consisted of 55 HCM children (mean age 12.5 ± 4.6 years, 69.1% males); seven had endpoints (four deaths, three appropriate ICD discharges). A strong trend (DeLong p = 0.08) was observed towards better endpoint identification performance of the HCM Risk-Kids Model compared to the ACC/AHA strategy. Adding the atrial conduit function component significantly improved the prediction capabilities of the AHA/ACC Model (DeLong p = 0.01) and HCM Risk-Kids algorithm (DeLong p = 0.04). CONCLUSIONS: The new HCM Risk-Kids individualised algorithm and score was capable of identifying high-risk children with very good accuracy. The inclusion of one of the atrial dynamic indices improved both risk stratification strategies.

Usefulness of long-term telemetric electrocardiogram monitoring in the diagnosis of tachycardia in children with a medical history of palpitations.

BACKGROUND: In children, palpitations, which may result from a life­threatening tachyarrhythmia, are one of the most common causes of cardiac visits and hospitalizations. Effective diagnosis is essential in this population of patients. AIMS: This study aimed to assess the usefulness of long­term telemetric electrocardiograms compared with Holter monitoring in the diagnostic workup in children with palpitations. METHODS: A total of 350 children with undocumented palpitations were examined in a multicenter study. In 167 patients (47.7%), the TELE group, month­long continuous telemetric electrocardiogram monitoring (using the PocketECG system) was performed. In 183 patients (52.3%), the HOLT group, 24­hour Holter electrocardiography was carried out and repeated after a month if tachyarrhythmia was not recorded. RESULTS: A total of 152 children (43.4%) reported palpitations, and 36.2% of them had sinus tachycardia during palpitations. Tachyarrhythmias were recorded in 68 patients (40.7%) in the TELE group and in 7 (3.8%) in the HOLT group after the second examination (P <0.001); the mean time to record tachycardia was 15.8 (8.7) days versus 25.4 (11.1) days (P = 0.004). In the TELE group, we noted a greater number of children with palpitations during recording (62.9% vs 18%), tachycardia with normal QRS complexes (21.6% vs 1.6%), ventricular tachycardia (11.4% vs 0.5%), and asymptomatic arrhythmias than in the HOLT group. CONCLUSIONS: In children, long­term telemetric electrocardiogram monitoring using the PocketECG system is well tolerated and has a high diagnostic efficacy. In young patients with palpitations, telemetric cardiac monitoring lasting up to a month increased the number of patients with recorded tachyarrhythmia by almost 10-fold compared with the analysis of 2 Holter electrocardiograms. We found that a large number of children have asymptomatic cardiac arrhythmias.

Bipolar ablation of epicardial posteroseptal accessory pathway.

We present a case of a 16-year-old male with WPW syndrome, referred for ablation after being resuscitated from cardiac arrest. Bipolar transseptal RF ablation successfully destroyed rapidly conducting epicardial posteroseptal accessory pathway after three failed attempts of endo- and epicardial ablation.

PocketECG: A New Noninvasive Method for Continuous and Real-Time ECG Monitoring-Initial Results in Children and Adolescents.

Long-term ECG is widely used in diagnosis and assessment of many cardiac symptoms which may be caused by dangerous arrhythmias that sometimes can be difficult to document. The PocketECG system is a new technological solution for a long-term, noninvasive, continuous and real-time ECG monitoring that provides automatic diagnosis of dysrhythmias. ECG data transmission occurs over a mobile network. The goal of this study was to assess the reliability of long-term ECG recordings acquired with the PocketECG system. One hundred and fifteen patients (43 girls and 72 boys) of an average age of 15.5 ± 2.5 years were examined at the Department of Cardiology at the Children's Memorial Health Institute. Two simultaneous 24-h ECG recordings were conducted: one with a Holter monitor and one with the PocketECG system. A linear correlation was demonstrated between the two methods with regard to the recorded QRS complexes [H = 1173.0 (-1946.40; 4838.50) + PocketECG*0.98 (0.94; 1.02)]. Mean diurnal heart rhythms were comparable (p > 0.05) despite the fact that the slowest and the fastest rates were different. The rate of detection for ventricular, supraventricular dysrhythmias and pauses in ventricular rhythm were comparable in both methods. The PocketECG system for continuous and real-time ECG recording is a reliable method for the assessment of heart rhythm and dysrhythmias in children and adolescents.

Radiofrequency catheter ablation of accessory pathways in patients with Ebstein's anomaly: At 8 years of follow-up.

BACKGROUND: Data regarding long-term follow-up of radiofrequency catheter ablation (RFCA) of accessory pathways (APs) in patients with Ebstein's anomaly (EA) are limited. The procedures are challenging due to multiple or wide APs. METHODS: Analysis was performed on clinical and periprocedural data of patients with EA referred to the centre in order to perform catheter ablation of AP. The group consisted of 22 patients (female 40.9%, mean age 33.6 ± 19.1 years). The follow-up utilized electrocardiogram and Holter monitoring. RESULTS: Twenty-two patients had 33 accessory pathways (8 patients had multiple APs, 11 patients broad AP). Twenty-nine different arrhythmias were ablated: 20 orthodromic atrioventricular reciprocating tachycardia (O-AVRT), 5 antidromic atrioventricular reciprocating tachycardia (A-AVRT), 3 slow/ fast atrioventricular nodal reentry tachycardia (s/f AVNRT) and 1 cavotricuspid-isthmus-dependent atrial flutter (CTI-AFL). In 3 (13.6%) patients multiple ablation targets for RFCA ablation were observed. The acute procedural success rate after the first RFCA performed was: 100% for AVNRT, 77.3% for APs and 50.0% for CTI-AFL ablation. Follow-up (mean 95.7 ± 49.8 months) was completed in 86.4% of patients. One patient had paroxysmal atrial fibrillation not targeted during ablation. One patient died due to heart failure 12 years after RFCA. Three patients who underwent RFCA of accessory pathways in the mid-1990s were lost in follow-up. CONCLUSIONS: Radiofrequency ablation in patients with EA is challenging but safe and have a high short-term as well as long-term success rate.

QTc prolongation in patients with hearing loss: Electrocardiographic and genetic study.

BACKGROUND: The aim of the study was to determine, whether electrocardiogram (ECG) screening could reduce the risk of sudden cardiac death in patients with hearing loss through the early diagnosis of Jervell and Lange-Nielsen syndrome and the introduction of the therapy. METHODS: One thousand and eighty patients with hearing loss (aged 21.8 ± 19.9 years) underwent ECG. Additionally, all subjects were asked to complete a 3-question survey. Those who met, at least, one of the high-risk criteria underwent further cardiac assessment and genetic testing. RESULTS: QTc assessment was possible in 1,027 patients. Mean QTc measured 422.8 ± 23.7 ms in 313 women, 414.9 ± 27.7 ms in 273 men and 421.1 ± 21.5 ms in 441 children (individuals younger than 14 years). Abnormal QTc was found in 13 (4.1%) women, 20 (7.3%) men, and 72 (16.3%) children. In the studied group, no recessive mutation of KNCQ1 or KCNE1 was found. In 6 patients, other mutations were found: in KCNQ1 (n = 1), in KCNH2 (n = 3) and in SCN5A (n = 1), which were pathogenic for long-QT-syndromes (LQTS), and 2 mutations of unknown clinical significance in SCN5A. Overall, out of these 6 patients LQTS was diagnosed in 3 asymptomatic patients, but with abnormal QTc and in 2 patients with normal QTc, but who were previously treated for epilepsy. CONCLUSIONS: Jervell and Lange-Nielsen syndrome is a very rare condition even in a population with hearing loss. In this population, the prevalence of prolonged QT interval is increased over the general population. Further investigations are necessary.

Andersen-Tawil syndrome: report of 3 novel mutations and high risk of symptomatic cardiac involvement.

INTRODUCTION: Andersen-Tawil syndrome (ATS) is a potassium channelopathy affecting cardiac and skeletal muscle. Periodic paralysis is a presenting symptom in some patients, whereas, in others, symptomatic arrhythmias or prolongation of QT in echocardiographic recordings will lead to diagnosis of ATS. Striking intrafamilial variability of expression of KCNJ2 mutations and rarity of the syndrome may lead to misdiagnosis. METHODS: We report 15 patients from 8 Polish families with ATS, including 3 with novel KCNJ2 mutations. RESULTS: All patients had dysmorphic features; periodic paralysis affected males more frequently than females (80% vs. 20%), and most attacks were normokalemic. Two patients (with T75M and T309I mutations) had aborted sudden cardiac death. An implantable cardioverter-defibrillator was utilized in 40% of cases. CONCLUSIONS: KCNJ2 mutations cause a variable phenotype, with dysmorphic features seen in all patients studied, a high penetrance of periodic paralysis in males and ventricular arrhythmia with a risk of sudden cardiac death.

Idiopathic ventricular arrhythmia in children and adolescents: early effectiveness of radiofrequency current ablation.

BACKGROUND AND AIM: The aim of the study was to evaluate early effectiveness of radiofrequency (RF) current ablation for idiopathic ventricular arrhythmia (iVA) in children and to identify factors affecting treatment results. METHODS: Among over 600 children, 30 ablation procedures were performed for iVA. Patient age ranged from 2.0 to 17.9 years (mean 14.7 ± 3.1). We analysed arrhythmia recurrences at 24 h and the effect of patient age, arrhythmia location, type of anaesthesia, and the presence of spontaneous arrhythmia during the procedure on the treatment results. RESULTS: Sixteen (53%) children had right ventricular (RV) arrhythmia, including RV outflow tract arrhythmia in 9 of them (56%). In 14 (47%) children, arrhythmia was located in the left ventricle (LV), including LV outflow tract in 2 children, and the posterior fascicle in 4 children. The procedure was effective in 13 (81%) children in the RV arrhythmia group and in 11 (78%) children in the LV arrhythmia group, including all children with fascicular tachycardia. The only variable that affected the treatment results was the presence of spontaneous arrhythmia during the procedure (p = 0.012). No procedural complications were noted. CONCLUSIONS: RF current ablation of iVA is a safe procedure with high early effectiveness. The only variable that affected the treatment results was the presence of spontaneous arrhythmia during the procedure.

Proceedings of the fourth international conference on central hypoventilation.

Central hypoventilation syndromes (CHS) are rare diseases of central autonomic respiratory control associated with autonomous nervous dysfunction. Severe central hypoventilation is the hallmark and the most life-threatening feature. CHS is a group of not-fully defined disorders. Congenital CHS (CCHS) (ORPHA661) is clinically and genetically well-characterized, with the disease-causing gene identified in 2003. CCHS presents at birth in most cases, and associated with Hirschsprung's disease (ORPHA99803) and neural crest tumours in 20% and 5% of cases, respectively. The incidence of CCHS is estimated to be 1 of 200,000 live births in France, yet remains unknown for the rest of the world. In contrast, late-onset CHS includes a group of not yet fully delineated diseases. Overlap with CCHS is likely, as a subset of patients harbours PHOX2B mutations. Another subset of patients present with associated hypothalamic dysfunction. The number of these patients is unknown (less than 60 cases reported worldwide). Treatment of CHS is palliative using advanced techniques of ventilation support during lifetime. Research is ongoing to better understand physiopathological mechanisms and identify potential treatment pathways.The Fourth International Conference on Central Hypoventilation was organised in Warsaw, Poland, April 13-15, 2012, under the patronage of the European Agency for Health and Consumers and Public Health European Agency of European Community. The conference provided a state-of-the-art update of knowledge on all the genetic, molecular, cellular, and clinical aspects of these rare diseases.

Sick sinus syndrome: a family study.

A case of related individuals affected by sick sinus syndrome is presented in this study. The clinical and electrocardiographic signs of sinus node dysfunction and the most common causes of this disease are presented. Subsequently, the article includes descriptions of sinus node disease in three related children as well as details of the disease in their relatives. A literature review of the genetics of familial sinus node dysfunction concludes the study.

Persistent multifocal atrial tachycardia in infant with encephalocraniocutaneous lipomatosis: a case report.

UNLABELLED: Encephalocraniocutaneous lipomatosis (ECCL, Haberland syndrome, Fishman syndrome) is a very rare congenital disorder, involving skin, eye, bone and central nervous system malformations. In this paper we present a case of a 2-month-old boy with encephalocraniocutaneous lipomatosis diagnosed on the basis of characteristic clinical manifestations and neuroimaging findings. Neurologically, the child presented only with mild physical and mental retardation. 24-h Holter monitoring revealed asymptomatic multifocal atrial tachycardia. Initial therapy with digoxin and metoprolol was not effective. Introduction of propafenone resulted in supression of supraventricular arrhythmia. During the 3- years follow-up, sinus rhythm persisted, but neurological status deteriorated. CONCLUSION: Supraventricular arrhythmia may be associated with Haberland syndrome. It seems that propafenone is most effective in this condition.

Tpeak -tend interval in 12-lead electrocardiogram of healthy children and adolescents tpeak -tend interval in childhood.

BACKGROUND: Tpeak (Tp) to the Tend (Te) interval is an index of transmural dispersion of repolarization. Prolongation of this interval predisposes to life-threatening ventricular arrhythmias in long QT syndrome, polymorphic catecholaminergic ventricular tachycardia, Brugada syndrome and short QT syndrome and may be an indicator of increased risk of sudden cardiac death. Very little is known about TpTe interval in children and adolescents. METHODS: In 131 healthy children (64 girls) aged from 2.3 to 18.5 years (mean 9.1 years) the RR, QT, JT and TpTe intervals were measured manually in all leads of resting electrocardiogram (ECG). The statistical analysis were performed. RESULTS: TpTe intervals vary significantly (P < 0.0001) between individual leads-the longest were in lead V3 , the shortest ones in leads III and V1 . Boys had longer TpTe intervals, with statistically significant differences in leads I, aVR and precordial V2 -V6 . Greater values were also observed in older children. TpTe dispersion varied from 6 to 80 ms (mean 38.6 ms ± 14.6 ms, median 40 ms) with no gender differences and greater values in older subjects (P = 0.003). In most leads, higher TpTe/QT and TpTe/JT ratios were seen in boys regardless of age. The TpTe intervals lengthens with lowering heart rate. CONCLUSIONS: In healthy children and adolescents, TpTe intervals vary between individual leads of ECG, with the longest in lead V3 . The TpTe interval is longer in boys and in older children and prolongs as heart rate decelerates. TpTe/QT and TpTe/JT ratios are higher in boys. TpTe interval should be measured in precordial leads.