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Background: Tricuspid valve (TV) surgery is often required for adult congenital heart disease (ACHD), but may hinder catheter ablation when an artificial material or imbricated tissue covers the tricuspid annulus., Objective: The purpose of this study was to determine the outcomes of catheter ablation after TV surgery in a large ACHD cohort., Methods: An international retrospective study involving 7 centers was conducted. Patients who did and did not undergo TV surgery were matched for age, lesion classification, and postsurgical duration. TV operations were classified as valve ring/replacement vs repair., Results: One hundred thirty-six patients (42 ring/replacement, 39 repair, and 55 no TV surgery; median 32 years [IQR 20 - 46]) underwent 180 procedures targeting 239 tachycardias (cavotricuspid-isthmus dependent intraatrial reentrant tachycardia 36%, other intraatrial reentrant tachycardia 29%, focal atrial tachycardia 18%, and other supraventricular tachyarrhythmia 17%). Post-TV surgery, procedures were longer (4.3 hours vs 3.3 hours; P = .003) and required longer fluoroscopy time (31 minutes vs 18 minutes; P = .001). At least partial acute success was achieved in 81% of procedures in the TV ring/replacement group vs 94% in both TV repair and no TV surgery groups (P = .03). The difference was driven mainly by ablation of annular substrates, with acute success in 73% of TV ring/replacement, 92% of TV repair, and 94% of no TV surgery groups (P = .01). Over a median of 3.0 years, tachycardia recurred after 26% of procedures. TV ring/replacement predicted recurrence in the multivariable analysis (hazard ratio 2.4; 95% confidence interval 1.2-5.2; P = .009)., Conclusion: After surgery for ACHD, catheter ablation success was lower and tachycardia recurrence was higher after TV valve ring/replacement surgery. The findings of this retrospective report support future larger multicenter series and prospective evaluation to determine the role of empirical annular substrate ablation., (Published by Elsevier Inc.)

Background: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM)., Objective: The purpose of this study was to determine predictors of LAE in children with HCM., Methods: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions., Results: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P = .0025), LV posterior wall thickness z score (HR 1.02; P < .005), and LV outflow gradient < 30 mm Hg (HR 2.0; P < .006). LV posterior wall thickness z score ≥ 5 was associated with LAE., Conclusion: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM., (Copyright © 2019 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Extracardiac total cavopulmonary connection (E-TCPC) is widely performed for single ventricle palliation, yet there is little experience with catheter ablation in this population., Objectives: We hypothesized that atrial tachycardia substrates after primary E-TCPC would be similar to those in other forms of congenital heart disease and that catheter ablation could be performed effectively using a primarily transconduit approach., Methods: Catheter ablation characteristics of patients with E-TCPC from 9 centers were collected. Acute procedural success was defined as elimination of all sustained supraventricular tachyarrhythmias. Procedural complications, acute success, and recurrences were assessed., Results: Forty-six catheter ablation procedures were performed in 36 patients. Access to the atrium was by transconduit puncture in 29 procedures (63%). The most common supraventricular tachyarrhythmia mechanism was intra-atrial reentrant tachycardia (IART) in 21 patients (58%); and for all patients with primary E-TCPC and IART, an isthmus between the atrioventricular valve annulus and the oversewn inferior vena cava was critical for maintenance of tachycardia. Overall, acute success was achieved in 38 procedures (83%). There were 8 complications, with only 1 requiring intervention (epicardial pacemaker) and none related to conduit puncture. Recurrence after the final procedure occurred in 6 patients (17%) over a median follow-up duration of 0.4 years (interquartile range 0.1-1.5 years)., Conclusion: Catheter ablation could be performed effectively in this group of patients with E-TCPC, and the underlying IART substrate after primary E-TCPC appears to be reproducible. Catheter ablation may be a reasonable alternative to long-term antiarrhythmic therapy in this patient group., (Published by Elsevier Inc.)

Background: The angiotensin-converting enzyme insertion/deletion (ACE I/D) polymorphism is described in association with numerous phenotypes, including arrhythmias, and may provide predictive value among pediatric patients undergoing congenital heart surgery., Objective: The purpose of this study was to examine the role of a common polymorphism on postoperative tachyarrhythmias in a large cohort of pediatric patients undergoing congenital heart surgery with cardiopulmonary bypass (CPB)., Methods: Subjects undergoing congenital heart surgery with CPB at our institution were consecutively enrolled from September 2007 to December 2012. In addition to DNA, perioperative clinical data were obtained from subjects., Results: Postoperative tachyarrhythmias were documented in 45% of 886 enrollees and were associated with prolonged mechanical ventilation (P <.001) and intensive care unit length of stay (P <.001). ACE I/D was in Hardy-Weinberg equilibrium (19% I/I, 49% I/D, 32% D/D). I/D or D/D genotypes were independently associated with a 60% increase in odds of new tachyarrhythmia (odds ratio [OR] 1.6, 95% confidence interval [CI] 1.1-2.3, P = .02). Preoperative ACE inhibitor administration was independently associated with a 47% reduction in odds of postoperative tachyarrhythmia in the entire cohort (OR 0.53, 95% CI 0.32-0.88, P = .01), driven by a 5-fold reduction in tachyarrhythmias among I/I genotype patients (OR 0.19, 95% CI 0.04-0.88, P = .02)., Conclusion: The risk of tachyarrhythmias after congenital heart surgery is independently affected by the ACE I/D polymorphism. Preoperative ACE inhibition is associated with a lower risk of postoperative tachyarrhythmias, an antiarrhythmic effect that appears genotype dependent. An understanding of genotype variation may play an important role in the perioperative management of congenital heart surgery., (Copyright © 2014 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Antitachycardia pacing (ATP) can reduce implantable cardioverter-defibrillator shocks, but its use in children and patients with congenital heart disease (CHD) is not well described., Objective: To review the efficacy of ATP in children and patients with CHD., Methods: We reviewed implantable cardioverter-defibrillator therapies in children and patients with CHD (aged 2-52 years) at our institution. Appropriate therapies were defined as those delivered for true ventricular tachycardia (VT) or ventricular fibrillation; other therapies were defined as inappropriate., Results: During a median follow-up of 4 years (range 0.5-15 years), 17 of 79 patients (23%) received appropriate therapy and 14 received ATP for 100 episodes of VT. ATP was highly successful (88%) in terminating VT, and only 10 of 100 episodes required a shock. Shocks were effective in terminating VT/ventricular fibrillation in 21 of 24 episodes (87%). The outcomes of appropriate therapy were similar for ATP and shocks (success 88% vs 87%, failure 9% vs 8%, acceleration 3% vs 4% for ATP and shocks, respectively). Thirty-one patients (39%) received inappropriate therapy. Inappropriate ATP (without subsequent shocks) was delivered to 11 patients for the following: sinus tachycardia (19 episodes in 7 patients) with slowing of the rate after ATP, T-wave oversensing (2 episodes in 2 patients) with loss of oversensing after ATP, and reentrant supraventricular tachycardia (14 episodes in 2 patients) terminated with ventricular ATP., Conclusions: ATP is highly efficacious for VT in children and patients with CHD. In addition to reducing appropriate shocks, inappropriate shocks due to sinus or supraventricular tachycardia can be significantly reduced with ATP., (Copyright © 2012 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Junctional ectopic tachycardia (JET) is a common arrhythmia complicating pediatric cardiac surgery, with many identifiable clinical risk factors but no genetic risk factors to date., Objective: To test the hypothesis that the angiotensin-converting enzyme insertion/deletion (ACE I/D) polymorphism associates with postoperative JET., Methods: DNA samples were collected from children undergoing the Norwood procedure; arterial switch operation; and repairs of Tetralogy of Fallot, balanced atrioventricular septal defect, and ventricular septal defect at a single center. The incidence of postoperative JET was associated with previously identified clinical risk factors and ACE I/D genotype., Results: Of the 174 children who underwent the above-mentioned surgeries, 21% developed JET. Postoperative JET developed in 31% of children with the D/D genotype but only in 16% of those with the I/I genotype or the I/D genotype (P = .02). Clinical predictors of JET were selected a priori and included age, inotrope score, cardiopulmonary bypass time, and cross-clamp time. Multivariable logistic regression identified a significant correlation between the D/D genotype and postoperative JET independent of these predictors (odds ratio = 2.4; 95% confidence interval, 1.04-5.34; P = .04). A gene-dose effect was apparent in the homogeneous subset of subjects with atrioventricular septal defect (58% JET in D/D subjects, 12% JET in I/D subjects, and 0% JET in I/I subjects; P <.01)., Conclusion: The common ACE deletion polymorphism is associated with a greater than 2-fold increase in the odds of developing JET in children undergoing surgical repair of atrioventricular septal defect, Tetralogy of Fallot, ventricular septal defect or the Norwood and arterial switch procedures. These findings may support the potential role of the renin-angiotensin-aldosterone system in the etiology of JET., (Copyright © 2011 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)