An international multicenter cohort study on implantable cardioverter-defibrillators for the treatment of symptomatic children with catecholaminergic polymorphic ventricular tachycardia.

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) may cause sudden cardiac death (SCD) despite medical therapy. Therefore, implantable cardioverter-defibrillators (ICDs) are commonly advised. However, there is limited data on the outcomes of ICD use in children. OBJECTIVE: The purpose of this study was to compare the risk of arrhythmic events in pediatric patients with CPVT with and without an ICD. METHODS: We compared the risk of SCD in patients with RYR2 (ryanodine receptor 2) variants and phenotype-positive symptomatic CPVT patients with and without an ICD who were younger than 19 years and had no history of sudden cardiac arrest at phenotype diagnosis. The primary outcome was SCD; secondary outcomes were composite end points of SCD, sudden cardiac arrest, or appropriate ICD shocks with or without arrhythmic syncope. RESULTS: The study included 235 patients, 73 with an ICD (31.1%) and 162 without an ICD (68.9%). Over a median follow-up of 8.0 years (interquartile range 4.3-13.4 years), SCD occurred in 7 patients (3.0%), of whom 4 (57.1%) were noncompliant with medications and none had an ICD. Patients with ICD had a higher risk of both secondary composite outcomes (without syncope: hazard ratio 5.85; 95% confidence interval 3.40-10.09; P < .0001; with syncope: hazard ratio 2.55; 95% confidence interval 1.50-4.34; P = .0005). Thirty-one patients with ICD (42.5%) experienced appropriate shocks, 18 (24.7%) inappropriate shocks, and 21 (28.8%) device-related complications. CONCLUSION: SCD events occurred only in patients without an ICD and mostly in those not on optimal medical therapy. Patients with an ICD had a high risk of appropriate and inappropriate shocks, which may be reduced with appropriate device programming. Severe ICD complications were common, and risks vs benefits of ICDs need to be considered.

Differences in Androgen Receptor Expression in Human Heart Tissue in Various Types of Cardiomyopathy and in Aortic Valve Stenosis.

Background: Sex-specific differences in heart disease outcomes are influenced by the levels of the steroid hormones, estrogen and testosterone. While the roles of estrogen receptors in cardiac disease are well-studied in animals and humans, respective research on androgen receptors (AR) is limited. Here we investigate AR protein and mRNA expression in human myocardium of various cardiac diseases. Methods: AR expression was analyzed by western blotting in myocardium from human non-failing hearts (NF, n = 6) and patients with aortic stenosis (AS, n = 6), hypertrophic cardiomyopathy (HCM, n = 7), dilated cardiomyopathy (DCM, n = 7), and ischemic cardiomyopathy (ICM, n = 7). Using an AR45-specific antibody, a subsequent western blot assessed samples from male and female patients with HCM (n = 10) and DCM (n = 10). The same sample set was probed for full-length AR and AR45 mRNA expression. Immunohistochemistry (IHC) localized AR in myocardium from HCM and AS hearts. Results: Full-length AR was notably enriched in AS and HCM hearts compared to ICM, DCM, and NF. Similarly, AR45 was more abundant in HCM than in DCM. In contrast to the pattern observed for AR protein, full-length AR mRNA levels were lower in HCM compared to DCM, with no discernible difference for the AR45 isoform. Although gender differences in AR expression were not detected in western blots or qRT-PCR, IHC showed stronger nuclear AR signals in males than in females. Conclusions: Our findings indicate disease-specific regulation of AR mRNA and/or AR protein in cardiac hypertrophy, underscoring a potential role in this cardiac pathology.

Leadless epicardial pacing at the left ventricular apex: an animal study.

AIMS: State-of-the-art pacemaker implantation technique in infants and small children consists of pace/sense electrodes attached to the epicardium and a pulse generator in the abdominal wall with a significant rate of dysfunction during growth, mostly attributable to lead failure. In order to overcome lead-related problems, feasibility of epicardial implantation of a leadless pacemaker at the left ventricular apex in a growing animal model was studied. METHODS AND RESULTS: Ten lambs (median body weight 26.8 kg) underwent epicardial implantation of a Micra transcatheter pacing system (TPS) pacemaker (Medtronic Inc., Minneapolis, USA). Using a subxyphoid access, the Micra was introduced through a short, thick-walled tube to increase tissue contact and to prevent tilting from the epicardial surface. The Micra's proprietary delivery system was firmly pressed against the heart, while the Micra was pushed forward out of the sheath allowing the tines to stick into the left ventricular apical epimyocardium. Pacemakers were programmed to VVI 30/min mode. Pacemaker function and integrity was followed for 4 months after implantation. After implantation, median intrinsic R-wave amplitude was 5 mV [interquartile range (IQR) 2.8-7.5], and median pacing impedance was 2235 Ω (IQR 1725-2500), while the median pacing threshold was 2.13 V (IQR 1.25-2.9) at 0.24 ms. During follow-up, 6/10 animals had a significant increase in pacing threshold with loss of capture at maximum output at 0.24 ms in 2/10 animals. After 4 months, median R-wave amplitude had dropped to 2.25 mV (IQR 1.2-3.6), median pacing impedance had decreased to 595 Ω (IQR 575-645), and median pacing threshold had increased to 3.3 V (IQR 1.8-4.5) at 0.24 ms. Explantation of one device revealed deep penetration of the Micra device into the myocardium. CONCLUSION: Short-term results after epicardial implantation of the Micra TPS at the left ventricular apex in lambs were satisfying. During mid-term follow-up, however, pacing thresholds increased, resulting in loss of capture in 2/10 animals. Penetration of one device into the myocardium was of concern. The concept of epicardial leadless pacing seems very attractive, and the current shape of the Micra TPS makes the device unsuitable for epicardial placement in growing organisms.

Contact force guided radiofrequency current application at developing myocardium.

INTRODUCTION: Catheter contact is a key determinant for lesion size in radiofrequency catheter ablation (RFA). Monitoring of contact force (CF) during RFA has been shown to improve efficacy of RFA in experimental settings as well as in adult patients. Coronary artery narrowing after RFA has been described in experimental settings as well as in children and adults and may be dependent from catheter contact. The value of CF monitoring concerning these issues has not been systematically studied yet. Value of high versus low CF during RFA in piglets was studied to assess lesion size and potential coronary artery involvement mimicking RFA in small children. METHODS: RFA with continuous CF monitoring was performed in 24 piglets (median weight 18.5 kg) using a 7 F TactiCath Quartz radiofrequency (RF) ablation catheter (Abbott). A total of 7 lesions were induced in each animal applying low (10-20 g) or high (40-60 g) CF. RF energy was delivered with a target temperature of 65°C at 30 W for 30 s. Coronary angiography was performed prior and immediately after RF application. Animals were assigned to repeat coronary angiography followed by heart removal after 48 h (n = 12) or 6 months (n = 12). Lesions with surrounding myocardium were excised, fixated, and stained. Lesion volumes were measured by microscopic planimetry. RESULTS: A total of 148 RF lesions were identified in the explanted hearts. Only in the subset of lesions at the AV annulus 6 month after ablation, lesion size and number of lesions exhibiting transmural extension were higher in the high CF group compared to low CF. In all other locations CF had no impact on lesion size and mural extension after 48 h as well as after 6 months. Additional parameters such as lesion size index and force time integral were also not related to lesion size. Coronary artery damage was present in two animals after 48 h and in one after 6 months and was not related to CF. CONCLUSION: In our experimental setting, lesion size in piglets was not related to catheter CF. Transmural extension of the RF lesions involving the layers of the coronary arteries was frequently noted irrespective of CF. Coronary artery narrowing was present in 3/24 animals and was not related to CF. In infants and toddlers, low CF (10-20 g) may be of adequate effect. Impact of CF monitoring during conventional RF ablation in children requires further investigation.

Repeat radiofrequency catheter ablation of atrial tachycardias in patients with congenital heart disease.

INTRODUCTION: Atrial tachycardias (AT) in patients with congenital heart disease (CHD) are significantly contributing to morbidity and mortality. Aim of this study was to evaluate the long-term course of CHD patients requiring repeat ablation procedures (RAP) of AT. PATIENTS AND METHODS: All 144 patients with CHD who had undergone ablation of AT at our center between January 2003 and October 2018 were enrolled. Patients were classified according to the complexity of CHD: complex CHD (cCHD), moderate CHD (mCHD), and simple CHD (sCHD). RESULTS: A total of 101 RAP were performed in 64 patients. One RAP was performed in n = 40, two in n = 13, three in n = 10, and five in n = 1. Acute success rate was 82% (83/101) and was not associated with the complexity of CHD (p = 1.0). Number of procedures was lower in patients with sCHD than in patients with mCHD and cCHD (sCHD 1.3 ± 0.6, mCHD 1.8 ± 1.0, and cCHD 1.8 ± 1.1, p = .04). RAP were most frequent in patients after Fontan palliation or Atrial switch procedure (2.0 ± 1.1 [n = 41] vs. 1.6 ± 0.9 all others, p = .016) and in patients with multiple unstable AT's (2.5 ± 1.1 [n = 11] vs. 1.7 ± 1.0, p = .008). Major complications occurred in 4/101 procedures. Complete follow-up was available in 125 patients. Since last RAP 73% of the patients were in sinus/atrial rhythm and 34/125 patients (27%) with AT recurrence did not require re-ablation with mean follow-up of 52 ± 40 months. CONCLUSIONS: Recurrences after ablation of AT in CHD patients were frequent. After RAP promising long-term results could be achieved. Data encourage repetitive ablation procedures in this patient population.

Evaluation of age at symptom onset, proband status, and sex as predictors of disease severity in pediatric catecholaminergic polymorphic ventricular tachycardia.

BACKGROUND: Children with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for sudden death, and a risk stratification tool does not exist. OBJECTIVE: The purpose of this study was to determine whether proband status, age at symptom onset, and/or sex are independent predictors of cardiac events. METHODS: A multicenter, ambispective, cohort of pediatric CPVT patients was categorized by sex, proband status, and age at symptom onset (D1: first decade of life [symptom onset <10 years] or D2: second decade of life [symptom onset 10-18 years, inclusive]). Demographics, therapy, genetics, and outcomes were compared between groups. RESULTS: A total of 133 patients were included and stratified into 58 D1 and 75 D2 patients (68 female and 65 male; 106 probands and 27 relatives). Localization of RYR2 variants to hotspots differed based on proband status and age at symptom onset. The cardiac event rate was 33% (n = 44/133), inclusive of a 3% (n = 4/133) mortality rate, over a median of 6 years (interquartile range 3-11) after time of symptom onset. Proband status, rather than age at of symptom onset or sex, was an independent predictor of time to first cardiac event (P = .008; hazard ratio = 4.4). The 5-, 10- and 15-year event-free survival rates for probands were 77%, 56%, and 46%, respectively, and for relatives were 96%, 91%, and 86%, respectively. Event risk after diagnosis was 48% (32/67) in patients on ß-blocker or flecainide alone vs 10% (5/48) in patients on ß-blocker plus flecainide and/or left cardiac sympathetic denervation (P <.001). CONCLUSION: Proband status, but not age at symptom onset or male sex, independently predicted an earlier onset of cardiac events. A larger sample size would enable a comprehensive investigation of other risk factors.

Safety of Transseptal Puncture for Access to the Left Atrium in Infants and Children.

Transseptal puncture (TSP) is a standard procedure to obtain access to the left heart. However, data on TSP in infants and children particularly with congenital heart defects (CHD) is sparse. Safety and efficacy of TSP in infants and children < 18 years with normal cardiac anatomy and with CHD were assessed. 327 TSP were performed in a total of 300 individuals < 18 years from 10/2002 to 09/2018 in our tertiary pediatric referral center. Median age at TSP was 11.9 years (IQR 7.8-15; range: first day of life to 17.9 years). 13 subjects were < 1 year. Median body weight was 43.8 kg (IQR 26.9-60; range: 1.8-121 kg). CHD was present in 28/327 (8.6%) procedures. TSP could be successfully performed in 323/327 (98.8%) procedures and was abandoned in 4 procedures due to imminent or incurred complications. Major complications occurred in 4 patients. 3 of these 4 subjects were ≤ 1 year of age and required TSP for enlargement of a restrictive atrial septal defect in complex CHD. Two of these babies deceased within 48 h after TSP attempt. The third baby needed urgent surgery in the cath lab. Pericardial effusion requiring drainage was noted in the forth patient (> 1 year) who was discharged well later. Minor complications emerged in 5 patients. The youngest of these individuals (0.3 years, 5.8 kg) developed small pericardial effusion after anterograde ballon valvuloplasty for critical aortic stenosis. The remaining 4/5 patients developed small pericardial effusion after ablation of a left-sided accessory atrioventricular pathway (6.1-12.2 years, 15.6-34.0 kg). TSP for access to the left heart was safe and effective in children and adolescents > 1 year of age. However, TSP was a high-risk procedure in small infants with a restrictive interatrial septum with need for enlargement of interatrial communication.

Lone Atrial Flutter in Children and Adolescents: Is It Really "Lone"?

Atrial flutter (AFL) in children and adolescents beyond the neonatal period in the absence of any underlying myocardial disease ("lone AFL") is rare and data is limited. Our study aims to present clinical and electrophysiological data of presumed "lone AFL" in pediatric patients and discuss the role of endomyocardial biopsy (EMB) and further follow-up. Since July 2005, eight consecutive patients at a median age of 12.7 (range 10.4-16.7) years presenting with presumed "lone AFL" after negative non-invasive diagnostic work-up had electrophysiological study (EPS) and induction of cavotricuspid isthmus (CTI) conduction block by radiofrequency (RF) current application. In 6/8 patients EMB could be taken. Induction of CTI conduction block was achieved in all patients. Histopathological examination of EMB from the right ventricular septum exhibited myocarditis or cardiomyopathy in 4/6 patients, respectively. During follow-up, 4/8 patients had recurrent arrhythmia (AFL n = 2, wide QRS complex tachycardia n = 1, monomorphic premature ventricular contractions n = 1) after the ablation procedure. 3/4 patients with recurrent arrhythmia had pathological EMB results. The remaining patient with recurrent arrhythmia had a negative EMB but was diagnosed with Brugada syndrome during further follow-up. Taking together results of EMB and further clinical course, only 3/8 patients finally turned out to have true "lone AFL". Our study demonstrates that true "lone AFL" in children and adolescents is rare. EMB and clinical course revealed an underlying cardiac pathology in the majority of the individuals studied. EMB was very helpful in order to timely establish the diagnosis of myocarditis or cardiomyopathy.

Epicardial implantation of a leadless pacemaker in a lamb model.

BACKGROUND: Pacemaker used in small children typically consist of an abdominally placed generator and epicardially affixed leads, making such a system prone to lead dysfunction during growth. Aim of this study was to investigate the feasibility of epicardial pacing with a leadless pacemaker in a lamb model. ANIMALS AND METHODS: Seventeen lambs underwent epicardial implantation of a Micra transcatheter pacing system (TPS) (Medtronic, Minneapolis, MN, USA) via left-lateral thoracotomy to the left ventricle (LV) surface (n = 11/17) and to the left atrial appendage (n = 6). Ventricular devices were fixated with the tines within the pericardium, whereas the tines of the atrial devices penetrated the myocardium of the left atrial appendage. After 31 weeks, animals were sacrificed and hearts were explanted for histological analysis. RESULTS: Following implantation, median P/R amplitude was 4.25/5.5 mV while median pacing threshold was 1.1/1.9 V at 0.24 ms. After 31 weeks, median P/R amplitude was 3.3/4.2 mV. Median atrial pacing threshold was 0.5/0.24 ms. Eight of 10 ventricular pacemakers had lost capture at standard impulse width even at maximum impulse amplitude. On explantation, firm adhesion of the device to the thoracic wall and dislodgement of the electrode tip was found in those ventricular devices. CONCLUSIONS: Firm fixation of the Micra electrode to the epicardial surface as applied to the atrial devices resulted in excellent electrical properties during midterm follow up. Pericardial fixation as in the ventricular devices was associated with loss of capture. Therefore, it is important to embed the tines in the myocardium and to choose an alternative implantation site allowing for safe fixation of the Micra TPS in a position perpendicular to ventricular epimyocardium.

Value of defibrillation threshold testing in children with nontransvenous implantable cardioverter defibrillators: Are routine DFT tests indicated?

BACKGROUND: Nontransvenous implanted cardioverter defibrillators (NT-ICD) are used in infants and small children with life-threatening ventricular tachyarrhythmias. With growth, shock vector shift may result in increase of defibrillation threshold (DFT) and fatal ICD failure. OBJECTIVES: To date, the only way to verify ICD function in children with NT-ICD is repetitive DFT testing, which is potentially harmful and may even be life threatening. The aim of the study was to analyze data from NT-ICD DFT testing to prospectively predict individual DFT. PATIENTS AND METHODS: Data from all pediatric patients with NT-ICD implanted in our center from July 2004 to August 2019 were collected. Postoperative DFT testing was scheduled according to individual DFT but at least annually. Surgical revision of NT-ICD was performed if DFT was > 25 J. Selected noninvasive parameters from DFT testing were analyzed as predictors for DFT using a logistic regression model. RESULTS: A total of 46 children with NT-ICD underwent a total of 402 DFT tests. Mean age at implantation had been 5.4 ± 3.3 years, mean follow-up was 5.6 ± 3.7 years in 5 (1%) DFT testing, maximum device output failed, and external defibrillation was necessary. A retrospective multiple mixed logistic regression model was able to predict a DFT ≥25 J (area under the curve [AUC] = 0.836). However, when prospectively validated the model showed moderate performance only (AUC = 0.70). CONCLUSION: A significant number of NT-ICD failures were detected by serial DFT testing. Serial DFT testing was safe in pediatric patients with an NT-ICD as all induced arrhythmia could be terminated. Prediction of DFT with noninvasive markers remains difficult and might help to schedule intervals for routine DFT tests to avoid unnecessary tests.

High density mapping and catheter ablation of atrial tachycardias in adults with congenital heart disease.

AIMS: We used a new grid-style multi-electrode mapping catheter (Advisor™ HD Grid, Abbott) and investigated its use for high density mapping of atrial tachycardias in adult patients with congenital heart disease. PATIENTS AND METHODS: All patients with congenital heart disease who had mapping of atrial tachycardias using the new grid-style catheter between March 2018 and April 2019 were included. RESULTS: A total of 24 adult patients had high density mapping of atrial tachycardias using the grid-style multi-electrode catheter. Mean procedure duration was 207 ± 72 min., mean fluoroscopy time was 7.1 ± 7.9 min. In patients with right atrial substrates, fluoroscopy time was shorter compared to biatrial or left atrial substrates (0.9 ± 2.2 min for right atrial substrates, n = 19 vs. 6.3 ± 8.3 min for left atrial substrates, n = 2 and 7.5 ± 4.3 min for biatrial substrates, n = 3, p = 0.01). A mean number of 14.814 ± 10.140 endocardial points were collected and 2.319 ± 1244 points were finally used to characterize the tachycardia. Procedural success was achieved in 21/24 (88%) subjects and partial success in 2/24 (8%) patients. Recurrence rate was low (12.5%). In one patient, radiofrequency ablation within the cavotricuspid isthmus resulted in occlusion of a branch of the right coronary artery. No complications related to the use of the mapping catheter itself occurred. CONCLUSION: High density mapping of AT using the grid-style catheter showed promising results with respect to procedural and midterm outcome and fluoroscopy time. Using the grid-style catheter might offer advantages compared to other multi-electrode catheters used for high density mapping of AT in patients with CHD.

Double cryoenergy application (freeze-thaw-freeze) at growing myocardium: Lesion volume and effects on coronary arteries late after energy application. Implications for efficacy and safety in pediatric patients.

INTRODUCTION: Cryoenergy is accepted as an alternative to radiofrequency ablation (RFA) in childen for ablation of supraventricular tachycardia substrates. Single cryoenergy application has been shown to be inferior to RFA. Double cryoenergy application has therefore been introduced into clinical practice, but experience concerning efficacy is limited. Coronary artery stenosis has been reported as serious complication after RFA for arrhythmia substrates but not after single cryoablation. The purpose of the study was to assess lesion volume (efficacy) and risk of coronary artery damage (safety), late, that is, 6 months, after double cryoenergy application in a piglet model. METHODS: Two sequential cycles of cryoenergy were delivered at -75°C for 4 minutes at the atrioventricular groove in five piglets. Animals were restudied after 6 months by coronary angiography and intracoronary ultrasound (ICUS). Ablation lesions were examined histologically and lesion volume was determined by three-dimensional morphometric analysis. RESULTS: Cryolesion volume was 174.04 ± 67.18 mm3 for atrial and 238.69 ± 112.1 mm3 for ventricular lesions (P > .05). Ventricular lesions, 4.06 ± 1.05 mm, were significantly deeper than atrial lesions, 3.58 ± 0.78 mm, (P < .05). In two of the 29 lesions, cryoenergy induced minor coronary artery injury with mild medial and adventitial thickening as well as minimal intimal proliferation, which had neither been detected by coronary angiography nor by ICUS. CONCLUSION: Late after double cryoenergy application at growing myocardium, subclinical minor affection of the coronary artery wall could be detected with minimal intimal proliferation. As lifetime sequelae of this finding remains unknown, further studies are warranted to address safety of repeated cycles of cryoenergy application for tachycardia substrates in children.

Transvenous and non-transvenous implantable cardioverter-defibrillators in children, adolescents, and adults with congenital heart disease: who is at risk for appropriate and inappropriate shocks?

AIMS: Non-transvenous implantable cardioverter-defibrillators (ICDs) as used in small patients and in patients with congenital heart disease (CHD) have not been compared with transvenous systems with respect to safety and efficacy yet. Aim of the present study was to describe the prevalence of and to identify contributing factors for appropriate and inappropriate ICD discharges in patients with non-transvenous and transvenous ICD. METHODS AND RESULTS: Single centre analysis of all paediatric and CHD patients who had received an ICD since 1995. One hundred and ninety-five patients were included. A transvenous system had been implanted in 153 (78%) subjects, the remaining 42 (22%) individuals received an extracardiac (EC)-ICD system. During mean follow-up of 4.5 years appropriate ICD shocks were noted in 32 (16%) individuals, whereas inappropriate shocks occurred in 22 (11%) patients. Appropriate shocks were more frequent in patients with an EC-ICD than in individuals with transvenous systems (29% vs. 13%, P = 0.02). Rapidly conducted atrial tachycardia (AT) was the most common reason for inappropriate shocks (76%). Rate of inappropriate shocks was not different between EC and transvenous systems (12% vs. 11%, P = 0.26). Lead failure was more prevalent in subjects with an EC-ICD (29% vs. 7%, P = 0.001). CONCLUSION: Individuals with EC-ICD systems were particularly prone to experience appropriate shocks. As rapidly conducted AT was the most common reason for inappropriate ICD shocks, rigorous treatment of these arrhythmias and proper ICD programming are mandatory. Though lead failure was of concern in EC-ICD patients, EC-ICD systems were not inferior with respect to inappropriate shocks.

Efficacy and safety of non-transvenous cardioverter defibrillators in infants and young children.

BACKGROUND: Implantable cardioverter defibrillators (ICD) protect from sudden cardiac death (SCD). In infants and young children, ICD implantation and programming is challenging due to small body size, elevated heart rates, and high physical activity. PURPOSE: We report our experience applying a non-transvenous ICD (NT-ICD) system to infants and children < 12 years of age and < 45-kg body weight. METHODS: Between 07/2004 and 07/2016, NT-ICD had been implanted in 36 patients. Nine out of 36 patients (25%) had NT-ICD implantation for primary and 27/36 (75%) for secondary prevention. Underlying diseases included inherited primary electrical arrhythmogenic diseases (n = 26; 72%), cardiomyopathies (n = 8; 22%), and congenital heart defects (n = 2; 6%). The median (interquartile range) age at implantation was 6 (1.9-8.4) years, and the median body weight was 21.7 (11.2-26.8) kg. Three different NT-ICD implantation techniques had been applied over time: (1) abdominal device/subcutaneous shock coil, (2) abdominal device/pleural shock coil, and (3) subcardiac device/pleural shock coil. RESULTS: During median follow-up of 5.2 (2.7-7.2) years, appropriate ICD discharges were documented in 12 (33.3%) and inappropriate shocks in 4 patients (11.1%). In 12/36 individuals (33.3%), a total of 25 surgical revisions were required due to NT-ICD malfunction. Eighteen out of 25 (72%) surgical revisions were necessary in patients with subcutaneous shock coil/abdominal device position. Surgical revisions (3/25, 12%) were significantly reduced (p < 0.001) after modifying the implantation technique to subcardiac device/pleural shock coil. CONCLUSIONS: NT-ICD was safe and effective in infants and young children. Appropriate ICD discharges occurred in a considerable number of patients. After modifying the implantation technique, the need for surgical revision could significantly be decreased.

Long-Term Follow-Up After Radiofrequency Catheter Ablation of Accessory Atrioventricular Pathways in Children.

OBJECTIVES: The purpose of this study was to evaluate long-term safety and efficacy of catheter ablation of accessory atrioventricular pathways (AP) in a pediatric cohort. BACKGROUND: Radiofrequency catheter ablation of accessory AP is the recommended treatment for patients with atrioventricular re-entrant tachycardia. Data on long-term results ≥1 year after AP ablation in pediatric patients is sparse. METHODS: A total of 296 patients <18 years of age who had undergone radiofrequency-AP ablation between October 2002 and June 2015 were included into the study. Follow-up was >1 year in all patients. Median age at ablation had been 11.6 years, and median follow-up was 5.6 years. Recurrence of AP conduction after ablation was defined as documentation of pre-excitation, supraventricular tachycardia attributable to AP, or proof of AP conduction during repeat electrophysiological study. RESULTS: AP ablation succeeded in 268 of 296 individuals (91%). After successful ablation, recurrence of AP conduction was observed in 29 of 268 individuals (10.8%). Of those 29, 23 (79%) had AP recurrence within the first year after ablation, whereas 13 (45%) had recurrence of AP conduction already within the first month. Six patients had late recurrence of AP conduction >1 year post-ablation. Procedural success and freedom from AP conduction after a single ablation procedure was 86% at 1 month, 83% at 1 year, and 81% at 5 years after ablation. CONCLUSIONS: After radiofrequency ablation of AP in children, recurrence of AP conduction occurred in 23 subjects (8% of the study cohort) within the first year after ablation. Late recurrences >1 year after ablation were noticed in 6 children (2% of the study group), highlighting the need for longer follow-up >1 year. Results of the present study on late AP recurrence should be taken into account whenever families are counselled for pediatric AP ablation.

Radiofrequency Catheter Ablation of Atrial Tachycardias in Congenital Heart Disease: Results With Special Reference to Complexity of Underlying Anatomy.

BACKGROUND: Radiofrequency catheter ablation has become the treatment strategy of choice for atrial tachyarrhythmias in patients with congenital heart disease (CHD). We analyzed results of radiofrequency catheter ablation in a large cohort of patients with CHD with special reference to complexity of underlying anatomy. METHODS AND RESULTS: One hundred and forty-four patients with CHD and atrial tachyarrhythmias undergoing radiofrequency catheter ablation were classified according to complexity of underlying CHD: simple CHD, n=18 (12%); moderate CHD, n=53 (37%); and complex CHD, n=73 (51%). Overall acute success was achieved in 81% of the patients. Acute success was lower for tachycardias involving the left atrium compared with right atrial tachycardias. Complexity of CHD was associated with longer procedure duration. Tachycardia recurrence was observed in 54% of the patients after a total follow-up of 7.4 years. 75% of all recurrences occurred within the first year. Recurrence of tachycardia was more likely in patients with complex surgical atrial anatomy (ie, Fontan palliation or atrial switch procedure). Major complications occurred in 4 patients and were related to vascular access. CONCLUSIONS: Acute procedural success of atrial tachycardia ablation in congenital heart patients was not influenced by complexity of CHD. Long-term outcome with regard to tachycardia recurrence was worse in patients with complex surgical atrial anatomy.

Effects of Triple Cryoenergy Application on Lesion Formation and Coronary Arteries in the Developing Myocardium.

To improve long-term outcome after cryoablation of substrates of supraventricular tachycardia, application of two and three consecutive freeze-thaw cycles has been performed. The effect of triple freeze-thaw cycles on lesion formation within developing myocardium and coronary arteries, however, has not been studied yet. In eight piglets (mean age 15 weeks, weight 15-20 kg), 30 cryolesions (three consecutive freeze-thaw cycles) were applied to the atrial aspect of both AV valve annuli (n = 18) as well as to ventricular myocardium below the valves (n = 12). Coronary angiography was performed before and after cryoenergy application. The animals were reevaluated by coronary angiography and intracoronary ultrasound (ICUS) after 48 h. All hearts were removed for histological examination of the lesions subsequently. After staining (hematoxylin-eosin, desmin immunohistochemistry), lesions was measured by planimetry with a digital virtual miscroscope analysis system and volumes of the cryolesions were calculated. Mean atrial lesion volume was 190.68 ± 167.53 mm3 (n = 18), and mean ventricular lesion volume was 184.34 ± 107.42 mm3 (n = 12). Compared with previously reported data on lesion volumes after single and double freeze-thaw cycles, lesions were significantly larger. Coronary arteries were unaffected on coronary angiography as well as on ICUS. No affection of coronary arteries was found on histological examination. Application of three consecutive freeze-thaw cycles resulted in increased lesion volume compared with single and double freeze-thaw cycles. No affection of the coronary arteries was evident. To evaluate the clinical benefit and safety of triple cryoenergy application for catheter ablation, prospective randomized trials are required.

Long-Term Follow-Up After Catheter Ablation of Atrioventricular Nodal Reentrant Tachycardia in Children.

BACKGROUND: Catheter ablation of the slow conducting pathway (SP) is treatment of choice for atrioventricular nodal reentrant tachycardia (AVNRT). Although there are abundant data on AVNRT ablation in adult patients, little is known about the long-term results ≥3 years after AVNRT ablation in pediatric patients. METHODS AND RESULTS: Follow-up data from 241 patients aged ≤18 years who had undergone successful AVNRT ablation were analyzed. Median age at ablation had been 12.5 years, and median follow-up was 5.9 years. Radiofrequency current had been used in 168 patients (70%), whereas cryoenergy had been used in 73 patients (30%). Procedural end point of AVNRT ablation had been either SP ablation (no residual dual atrioventricular nodal physiology) or SP modulation (residual SP conduction allowing for a maximum of one atrial echo beat). After the initial AVNRT ablation, calculated freedom from AVNRT was 96% at 1 year, 94% at 3 years, 93% at 5 years, and 89% at 8 years. Age, sex, body weight, the choice of ablation energy, and the procedural end point of AVNRT ablation did not impact freedom from AVNRT. Six of 22 AVNRT recurrences (27%) occurred ≥5 years after ablation. No late complications including atrioventricular block were noted. CONCLUSIONS: Cumulatively, catheter ablation of AVNRT continued to be effective in >90% of our pediatric patients during the long-term course. A significant part of recurrences occurred >5 years post ablation. Body weight, energy source, and the end point of ablation had no impact on long-term results. No adverse sequelae were noted.

Internal Cardioverter Defibrillator Indications and Therapies after Atrial Baffle Procedure for d-Transposition of the Great Arteries: A Multicenter Analysis.

BACKGROUND: Sudden cardiac death (SCD) is the most important cause of late mortality after atrial baffle procedure for d-transposition of the great arteries (d-TGA). Experience with internal cardioverter defibrillator (ICD) therapy in this population is limited. We conducted a multicenter cohort study to determine the current state of ICD therapy in individuals after atrial baffle procedure. METHODS: Demographic and clinical data as well as data on device implantation, programming, ICD discharges, and complications after atrial baffle procedure for d-TGA from four German centers were analyzed retrospectively. RESULTS: ICD implantation was undertaken in 33 subjects. ICD implantation was undertaken as primary prevention in 29 (88%) and secondary prevention in four (12%) individuals. There were 21 (64%) subjects with atrial reentrant tachycardia (IART). During a median follow-up of 4.8 years, seven appropriate ICD therapies were delivered in three (10%) individuals with primary prevention indication. No appropriate shocks were documented in subjects with secondary prevention indication. A total of 12 inappropriate ICD discharges occurred in eight (24%) individuals due to IART (n = 6) or lead failure (n = 2). ICD-related complications were noted in seven individuals (21%): lead dislodgement/failure in five (15%) and ICD infection in two subjects (6%). CONCLUSIONS: The majority of individuals received an ICD for primary prevention of SCD, thus representing a liberal attitude of physicians for ICD implantation. During a median follow-up of 4.8 years, the rate of appropriate ICD therapies was low and clearly exceeded by inappropriate ICD discharges. Lead failure and IART were present in >20% of the individuals and were frequent reasons for inappropriate ICD discharges. Facing these results, rigorous treatment of IART and careful ICD programming seems mandatory.

Radiofrequency Catheter Ablation of Accessory Atrioventricular Pathways in Infants and Toddlers ≤ 15 kg.

Accessory atrioventricular pathways (AP) are the most common substrate for paroxysmal supraventricular tachycardia in infants and small children. Up-to-date data on AP ablation in infants and small children are limited. The aim of the present study was to gain additional insight into radiofrequency (RF) catheter ablation of AP in infants and toddlers focusing on efficacy and safety in patients with a body weight of ≤ 15 kg. Since 10/2002, RF ablation of AP was performed in 281 children in our institution. Indications, procedural data as well as success and complication rates in children with a body weight ≤ 15 kg (n = 22) were compared with children > 15 kg (n = 259). Prevalence of structural heart anomalies was significantly higher among children ≤ 15 kg (27 vs. 5.7 %; p = 0.001). Procedure duration (median 262 vs. 177 min; p = 0.001) and fluoroscopy time (median 20.6 vs. 14.0 min; p = 0.007) were significantly longer among patients ≤ 15 kg. Procedural success rate did not differ significantly between the two groups (82 vs. 90 %). More RF lesions were required for AP ablation in the smaller patients (median 12 vs. 7; p = 0.019). Major complication rate was significantly higher in children ≤ 15 kg (9 vs. 1.1 %; p = 0.05) with femoral vessel occlusion being the only major adverse event in patients ≤ 15 kg. Catheter ablation of AP in children was effective irrespective of body weight. In children ≤ 15 kg, however, procedures were more challenging and time-consuming. Complication rate and number of RF lesions in smaller children were higher when compared to older children.