Long-Term Outcomes of Cardiac Resynchronization Therapy in Patients With Repaired Tetralogy of Fallot: A Multicenter Study.

BACKGROUND: A growing number of patients with tetralogy of Fallot develop left ventricular systolic dysfunction and heart failure, in addition to right ventricular dysfunction. Although cardiac resynchronization therapy (CRT) is an established treatment option, the effect of CRT in this population is still not well defined. This study aimed to investigate the early and late efficacy, survival, and safety of CRT in patients with tetralogy of Fallot. METHODS: Data were analyzed from an observational, retrospective, multicenter cohort, initiated jointly by the Pediatric and Congenital Electrophysiology Society and the International Society of Adult Congenital Heart Disease. Twelve centers contributed baseline and longitudinal data, including vital status, left ventricular ejection fraction (LVEF), QRS duration, and NYHA functional class. Outcomes were analyzed at early (3 months), intermediate (1 year), and late follow-up (≥2 years) after CRT implantation. RESULTS: A total of 44 patients (40.3±19.2 years) with tetralogy of Fallot and CRT were enrolled. Twenty-nine (65.9%) patients had right ventricular pacing before CRT upgrade. The left ventricular ejection fraction improved from 32% [24%-44%] at baseline to 42% [32%-50%] at early follow-up (P<0.001) and remained improved from baseline thereafter (P≤0.002). The QRS duration decreased from 180 [160-205] ms at baseline to 152 [133-182] ms at early follow-up (P<0.001) and remained decreased at intermediate and late follow-up (P≤0.001). Patients with upgraded CRT had consistent improvement in left ventricular ejection fraction and QRS duration at each time point (P≤0.004). Patients had a significantly improved New York Heart Association functional class after CRT implantation at each time point compared with baseline (P≤0.002). The transplant-free survival rates at 3, 5, and 8 years after CRT implantation were 85%, 79%, and 73%. CONCLUSIONS: In patients with tetralogy of Fallot treated with CRT consistent improvement in QRS duration, left ventricular ejection fraction, New York Heart Association functional class, and reasonable long-term survival were observed. The findings from this multicenter study support the consideration of CRT in this unique population.

Multicenter retrospective evaluation of magnetic resonance imaging in pediatric and congenital heart disease patients with cardiac implantable electronic devices.

BACKGROUND: Guidelines addressing magnetic resonance imaging (MRI) in patients with cardiac implantable electronic devices (CIEDs) provide algorithms for imaging pediatric and congenital heart disease (CHD) patients. Guideline acceptance varies by institution. Guidelines also do not support routine MRI scans in patients with epicardial or abandoned leads, common in pediatric and CHD patients. OBJECTIVE: The purpose of this study was to determine the incidence of MRI-related complications in pediatric and CHD patients with CIEDs, including epicardial and/or abandoned leads. METHODS: A multicenter retrospective review included patients with CIEDs who underwent any MRI between 2007 and 2022 at congenital cardiac centers. The primary outcome was any patient adverse event or clinically significant CIED change after MRI, defined as pacing lead capture threshold increase >0.5 V with output change, P- or R- wave amplitude decrease >50% with sensitivity change, or impedance change >50%. RESULTS: Across 14 institutions, 314 patients (median age 18.8 [1.3; 31.4] years) underwent 389 MRIs. There were 288 pacemakers (74%) and 87 implantable cardioverter-defibrillators (22%); 52% contained epicardial leads, and 14 (4%) were abandoned leads only. Symptoms or CIED changes occurred in 4.9% of MRI scans (6.1% of patients). On 9 occasions (2%), warmth or pain occurred. Pacing capture threshold or lead impedance changes occurred in 1.4% and 2.0% of CIEDs post-MRI and at follow-up. CONCLUSION: Our data provide evidence that MRIs can be performed in pediatric and CHD patients with CIEDs, including non-MRI-conditional CIEDs and epicardial and/or abandoned leads, with rare minor symptoms or CIED changes but no other complications.

Ventricular arrhythmias following balloon-expandable transcatheter pulmonary valve replacement in the native right ventricular outflow tract.

BACKGROUND: Ventricular arrhythmia incidence in children and adolescents undergoing transcatheter pulmonary valve replacement (TPVR) within the native right ventricular outflow tract (nRVOT) is unknown. We sought to describe the incidence, severity, and duration of ventricular arrhythmias and identify associated risk factors in this population. METHODS: This was a retrospective cohort study of 78 patients <21 years of age who underwent TPVR within the nRVOT. Patients were excluded for pre-existing ventricular arrhythmia or antiarrhythmic use. Study variables included surgical history, valve replacement indication, valve type/size, and ventricular arrhythmia. Univariable logistic regression models were used to evaluate factors associated with ventricular arrhythmias, followed by subset analyses. RESULTS: Nonsustained ventricular arrhythmia occurred in 26/78 patients (33.3%). The median age at the procedure was 10.3 years (interquartle range [IQR]: 6.5, 12.8). Compared with other nRVOT types, surgical repair with transannular patch was protective against ventricular arrhythmia incidence: odds ratio (OR): 0.35 (95% confidence interval [CI], 0.13-0.95). Patient weight, valve type/size, number of prestents, and degree of stent extension into the RVOT were not associated with ventricular arrhythmia occurrence. Beta blocker was started in 16/26 (61.5%) patients with ventricular arrhythmia. One additional patient was lost to follow-up. The median beta blocker duration was 46 days (IQR 42, 102). Beta blocker was discontinued in 10 patients by 8-week follow-up and in the remaining four by 9 months. CONCLUSIONS: Though common after balloon-expandable TPVR within the nRVOT, ventricular arrhythmias were benign and transient. Antiarrhythmic medications were successfully discontinued in the majority at 6- to 8-week follow-up, and in all patients by 20 months.

The high-density grid catheter: A safe adjunctive tool in pediatric and complex congenital heart disease patients.

INTRODUCTION: The safety and utility of the Advisor™ High-Density Grid mapping catheter (HDGC) in children and congenital heart disease (CHD) patients are not well described. METHODS: A single-center retrospective cohort study of pediatric and CHD patients undergoing electrophysiology study and ablation to determine the effect of HDGC use on outcomes including complications, fluoroscopy use, procedure duration, acute ablation success, and arrhythmia recurrence. RESULTS: HDGC was used in 74/261 (28.3%) cases. HDGC subjects differed by median age (17 vs. 13 years; p < 0.001), weight (68 vs. 50 kg; p < 0.001), and prevalence of significant CHD (42% vs. 3%; p < 0.001). Arrhythmia substrates were dissimilar: HGDC cases had higher frequencies of intra-atrial re-entrant tachycardia (25.7% vs. 0.5%), atrial flutter (8.1% vs. 1.1%), ectopic atrial tachycardia (13.5% vs. 3.7%), and premature ventricular contractions (9.5% vs. 0.5%), and lower incidences of atrioventricular re-entrant tachycardia (16.2% vs. 46.1%). Complications were rare (n = 5, 1.9%) with no significant difference between groups (p = 1.00). Procedure duration-but not fluoroscopy exposure-was significantly longer in HDGC cases (median 256 vs. 216 min, p < 0.001). Acute success was lower (93.2% vs. 99.4%; p = 0.01) and recurrences higher (13.2% vs. 3.8%; p = 0.016) in HDGC compared to non-HDGC cases. CONCLUSION: HDGC use in children and CHD patients is safe and not associated with higher complication rates. The lower acute success and higher recurrence rates in HDGC cases likely reflect a bias toward HDGC use in more complex arrhythmia substrates rather than less favorable ablation outcomes.

Subcutaneous Implantable Cardioverter-Defibrillators in Pediatrics and Congenital Heart Disease: A Pediatric and Congenital Electrophysiology Society Multicenter Review.

OBJECTIVES: The primary goal of this study was to evaluate the implant experience and midterm results of subcutaneous implantable cardioverter-defibrillators (S-ICDs) in pediatric patients and those with congenital heart disease. BACKGROUND: The S-ICD was developed to avoid the lead-related complications associated with transvenous systems. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients and those with congenital heart disease. METHODS: This international, multicenter, retrospective, standard-of-care study was conducted through the Pediatric & Congenital Electrophysiology Society. Complications at 30 and 360 days, inappropriate shocks, and delivery of appropriate therapy were assessed. RESULTS: The study included 115 patients with a median follow-up of 32 (19 to 52) months. Median age was 16.7 years (14.8 to 19.3 years), 29% were female, and 55% had a primary prevention indication. Underlying disease substrate was cardiomyopathy (40%), structural heart disease (32%), idiopathic ventricular fibrillation (16%), and channelopathy (13%). The complication rate was 7.8% at 30 days and 14.7% at 360 days. Overall, inappropriate shocks occurred in 15.6% of patients, with no single clinical characteristic reaching statistical significance. At implant, 97.9% of patients had successful first shock conversion with 96% requiring ≤65 J. Appropriate therapy was delivered to 11.2% of patients with an annual incidence of 3.9% and an acute first shock conversion success rate of 92.5%. CONCLUSIONS: This study found that in a heterogeneous population of pediatric patients and those with congenital heart disease, the S-ICD had comparable rates of complications, inappropriate shocks, and conversion efficacy compared with previously published studies on transvenous systems in similar populations.

Validating defibrillation simulation in a human-shaped phantom.

BACKGROUND: We previously developed a computational model to aid clinicians in positioning implantable cardioverter-defibrillators (ICDs), especially in the case of abnormal anatomies that commonly arise in pediatric cases. We have validated the model clinically on the body surface; however, validation within the volume of the heart is required to establish complete confidence in the model and improve its use in clinical settings. OBJECTIVE: The goal of this study was to use an animal model and thoracic phantom to record the ICD potential field within the heart and on the torso to validate our defibrillation simulation system. METHODS: We recorded defibrillator shock potentials from an ICD suspended together with an animal heart in a human-shaped torso tank and compared them with simulated values. We also compared the scaled distribution threshold, an analog to the defibrillation threshold, calculated from the measured and simulated electric fields within the myocardium. RESULTS: ICD potentials recorded on the tank and cardiac surface and within the myocardium agreed well with those predicted by the simulation. A quantitative comparison of the recorded and simulated potentials yielded a mean correlation of 0.94 and a relative error of 19.1%. The simulation can also predict scaled distribution thresholds similar to those calculated from the measured potential fields. CONCLUSION: We found that our simulation could predict potential fields with high correlation with the measured values within the heart and on the torso surface. These results support the use of this model for the optimization of ICD placements.

Frequency of Ventricular Arrhythmias and Other Rhythm Abnormalities in Children and Young Adults With the Marfan Syndrome.

Patients with the Marfan syndrome (MFS) are at risk for sudden death. The contribution of arrhythmias is unclear. This study examines the prevalence of arrhythmias in children with the MFS and their relation to clinical and/or echocardiographic factors. Data from the Pediatric Heart Network randomized trial of atenolol versus losartan in MFS were analyzed (6 months to 25 years old, aortic root diameter z-score > 3.0, no previous aortic surgery and/or dissection). Baseline 24-hour ambulatory electrocardiographic monitoring was performed. Significant ventricular ectopy (VE) and supraventricular ectopy (SVE) were defined as ≥10 VE or SVE/hour, or the presence of high-grade ectopy. Three-year composite clinical outcome of death, aortic dissection, or aortic root replacement was analyzed. There were 274 analyzable monitors on unique patients from 11 centers. Twenty subjects (7%) had significant VE, 13 (5%) significant SVE; of these, 2 (1%) had both. None had sustained ventricular or supraventricular tachycardia. VE was independently associated with increasing number of major Ghent criteria (odds ratio [OR] = 2.13/each additional criterion, p = 0.03) and greater left ventricular end-diastolic dimension z-score (OR = 1.47/each 1 unit increase in z-score, p = 0.01). SVE was independently associated with greater aortic sinotubular junction diameter z-score (OR = 1.56/each 1 unit increase in z-score, p = 0.03). The composite clinical outcome (14 events) was not related to VE or SVE (p ≥ 0.3), but was independently related to heart rate variability (higher triangular index). In conclusion, in this cohort, VE and SVE were rare. VE was related to larger BSA-adjusted left ventricular size. Routine ambulatory electrocardiographic monitoring may be useful for risk stratification in select MFS patients.

A Functional Assay for Sick Sinus Syndrome Genetic Variants.

BACKGROUND/AIMS: Congenital Sick Sinus Syndrome (SSS) is a disorder associated with sudden cardiac death due to severe bradycardia and prolonged pauses. Mutations in HCN4, the gene encoding inward Na+/K+ current (If), have been described as a cause of congenital SSS. The objective of this study is to develop an SSS model in embryonic zebrafish, and use zebrafish as a moderate-throughput assay to functionally characterize HCN4 variants. METHODS: To determine the function of hcn4 in zebrafish, embryos were either bathed in the If -specific blocker (ZD-7288), or endogenous hcn4 expression was knocked down using splice-blocking morpholinos. To assess whether the zebrafish model discriminates benign from pathogenic variants, we tested four HCN4 mutations known to cause human SSS and four variants of unknown significance (VUS). RESULTS: Pharmacological blockade and knockdown of hcn4 in zebrafish phenocopied human SSS, displaying bradycardia and cardiac pauses in intact embryos and explanted hearts. The zebrafish assay correctly identified all disease-causing variants. Of the VUS, the assay predicted 2 as benign and 2 as hypomorphic variants. CONCLUSIONS: We conclude that our embryonic zebrafish assay is a novel and effective tool to functionally characterize human HCN4 variants, which can be translated into important clinical prognostic information.

Catheter ablation of supraventricular tachyarrhythmia after extracardiac Fontan surgery.

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.

Inappropriate ICD shocks in pediatrics and congenital heart disease patients: Risk factors and programming strategies.

BACKGROUND: Inappropriate implantable cardioverter-defibrillator (ICD) shocks are a common and significant problem in pediatric patients and patients with congenital heart disease (CHD). OBJECTIVE: The purpose of this study was to evaluate the effect of programming high detection rates and long detection duration on inappropriate shocks in this population. METHODS: We performed a retrospective review of all patients with ICDs at a single pediatric center. Inappropriate shocks were defined as a shock for any rhythm except ventricular tachycardia or fibrillation. RESULTS: A total of 144 patients were included, 63 (44%) with CHD. At implant, mean age and weight were 17 ± 10 years and 57 ± 23 kg. ICDs were single chamber in 35 (24%), dual chamber in 97 (67%), and biventricular in 12 (8%). The mean follow-up duration was 42 ± 39 months. Appropriate shocks occurred in 29 (20.1%) and inappropriate shocks in 14 (9.7%). Causes of inappropriate shocks were supraventricular tachycardia (n = 6), lead malfunction (n = 4), sinus tachycardia (n = 3), and T-wave oversensing (n = 1). The mean ventricular fibrillation detection rate was 222 ± 15 beats/min, and the detection duration was 18 ± 12 beats. Patients with shocks programmed in the ventricular tachycardia zone were more likely to receive an inappropriate shock (P = .03). There were no associations between inappropriate shocks and age or weight at implant, presence of CHD, dual-chamber vs single-chamber device, history of supraventricular tachycardia, or antiarrhythmic use. There were no adverse events as a result of programming. CONCLUSION: Programming high detection rates and long detection duration resulted in a low rate of inappropriate shocks without associated adverse events in this large cohort of pediatric and CHD patients with ICDs.

A teenage fainter (dizziness, syncope, postural orthostatic tachycardia syndrome).

This article informs the general pediatrician about the diagnosis, evaluation, and treatment of teenage patients with presyncope and loss of consciousness. The focus is on distinguishing noncardiac fainting from life-threatening syncope. Current treatment strategies of vasovagal syncope and postural orthostatic tachycardia syndrome are also outlined.

Safety and results of cryoablation in patients <5 years old and/or <15 kilograms.

Current recommendations discourage elective radiofrequency ablation in patients <5 years old and/or weighing <15 kg, primarily because of the greater complication rate. To describe the current use, complications, and immediate outcomes of cryoablation in this patient population, a multicenter retrospective review of all patients <5 years old and/or weighing <15 kg who were treated with cryoablation for arrhythmia was performed. Eleven centers contributed data for 68 procedures on 61 patients. Of those, 34% were elective and 24% (n = 16) were both cryoablation and radiofrequency ablation. The median age and weight at ablation was 3.5 years (range 8 days to 9.9 years) and 15.2 kg (range 2.3 to 23), respectively. Congenital heart disease was present in 23% of the patients. The immediate success rate of cryoablation alone was 74%. No major complications occurred with cryoablation only; however, 2 of the 16 patients who underwent cryoablation and radiofrequency ablation had major complications. Of the 50 patients receiving cryoablation, 8 (16%) had variable degrees of transient atrioventricular block. The recurrence rate was 20% after cryoablation and 30% after cryoablation plus radiofrequency ablation. In conclusion, cryoablation appears to have a high safety profile in these patients. Compared to older and larger patients, the efficacy of cryoablation in this small, young population was lower and the recurrence rates were higher. Cryoablation's effect on the coronary arteries has not been fully elucidated and requires additional research.

Measurement of Defibrillator Surface Potentials for Simulation Verification.

Despite the growing use of implantable cardioverter defibrillators (ICDs) in adults and children, there has been little progress in optimizing device and electrode placement. To facilitate effective placement of ICDs, especially in pediatric cases, we have developed a predictive model that evaluates the efficacy of a delivered shock. Most recently, we have also developed an experimental validation approach based on measurements from clinical cases. The approach involves obtaining body surface potential maps of ICD discharges during implantation surgery and comparing these measured potentials with simulated surface potentials to determine simulation accuracy. Comparison of the simulated and measured potentials yielded very similar patterns and a typical correlation greater than 0.9, suggesting that the predictive simulation generates realistic potential values. Ongoing sensitivity studies will determine the robustness of the results and pave the way for use of this approach for assisting optimization of ICD use.

Contrasting effects of convective flow on catheter ablation lesion size: cryo versus radiofrequency energy.

BACKGROUND: Cryoablation has now become an alternative to treat many cardiac arrhythmias, and may be the treatment of choice in some patient populations. We compared the effects of convective flow on large-tip cryo and radiofrequency (RF) lesions dimensions. METHODS: Cryoablation and RF ablation were performed on porcine heart sections in a saline bath with varying directed flow rates. Cryoablation was performed for 4 minutes on 50 tissue pieces with tip temperature controlled at -80 degrees C. RF ablation was performed on 50 tissue pieces for 60 seconds at 60 degrees C tip temperature. The pieces were placed in culture media for 24 hours, and then sectioned, stained, and measured. RESULTS: Cryoablation and RF lesion sizes varied significantly with flow such that higher flow rates produced smaller cryoablation lesions and larger RF lesions (mean cryoablation volumes: 854 +/- 402, 808 +/- 217, 781 +/- 217, 359 +/- 114, and 292 +/- 117 mm(3), and mean RF volumes: 211 +/- 35, 304 +/- 79, 439 +/- 125, 525 +/- 187, and 597 +/- 126 mm(3) for 0, 1, 2, 3, and 5 L/min flow rates, respectively, P < 0.0005). Trabeculated pieces had larger cryoablation lesions and smaller RF lesions than nontrabeculated ones at higher flow rate (P < 0.005). Cryoablation lesion volume increased as the time to reach -80 degrees C decreased (r(2)= 0.72). CONCLUSION: In contrast to RF ablation, cryoablation lesion size is smaller at high flow rates, and larger at low flow rates due to the warming effects of local convective flow. The effects of high flow are reduced in areas of trabeculation, and the time to reach -80 degrees C predicts cryoablation lesion size.

Convective cooling affects cardiac catheter cryoablation and radiofrequency ablation in opposite directions.

Recently, cryoablation has received increased attention as a safer alternative to radiofrequency (RF) ablation. The purpose of this study was to compare the effect of convective cooling at physiologic flow rates on RF lesion size and on cryo lesion size. Porcine hearts were sectioned into 40 pieces and placed in a temperature-controlled saline bath (37 degrees C) with varying directed flow rates (0, 1, 2 and 3 L/min). Large-tip cryoablation (8 mm tip) was performed for 4 minutes on 20 tissue sections at -80 degrees C tip temperature. Large-tip RF catheter ablation (10 mm tip) was performed at 60 degrees C target temperature for 1 minute on 20 tissue sections. For each catheter, flow rates were randomized between applications. The tissue pieces were placed in culture medium for 24 hrs, sectioned, stained and measured to determine lesion depth, width and volume. Average lesion geometry was estimated from the data. Lesion dimensions were dependent on the flow rate for RF ablation with larger lesions at higher flow rates (mean volumes: 211+/-35, 304+/-79, 439+/-125 and 525.7+/-187 mm3 for 0, 1, 2 and 3 L/min flow rate, respectively, p<0.01). Also for cryoablation lesion size varied significantly with flow, such that lower flow rates produced larger lesions (mean volumes: 855+/-402, 809+/-218, 658+/-91 and 360+/-14 mm3 for 0, 1, 2 and 3 L/min flow rate respectively, p<0.01). While RF ablation creates larger lesions at high flow rates (3 L/min), cryoablation creates larger lesions at low flow rates (0-1 L/min, p<0.05).

Convective cooling effect on cooled-tip catheter compared to large-tip catheter radiofrequency ablation.

BACKGROUND: Both actively cooled-tip and large-tip catheters are currently available clinically to create large endomyocardial lesions during application of radiofrequency (RF) energy. The purpose of this study was to compare the effect of convective cooling at physiologic flow rates on RF lesion size using both actively cooled and large-tip catheters. METHODS: Porcine hearts were sectioned into 72 pieces and placed in a temperature-controlled saline bath (37 degrees C) with varying directed flow rates (0, 1, 2, and 3 L/min). Cooled-tip RF ablation (4 mm tip) was performed for 1 minute on 36 tissue sections with power manually titrated to keep tip temperature below 40 degrees C. Large-tip catheter ablation (10 mm tip) was performed at 65 degrees C target temperature for 1 minute on 36 tissue sections. For each catheter, flow rates were randomized between applications. The tissue pieces were sectioned and measured to determine lesion depth, width, and volume. RESULTS: Lesion dimensions were independent of the flow rate for the cooled-tip catheter (mean volumes: 382.0 +/- 121.6, 419.9 +/- 133.4, 375.9 +/- 169.1, and 346.7 +/- 173.4 mm(3) for 0, 1, 2, and 3 L/min flow rate, respectively, P = 0.78). For the large-tip catheter, lesion size varied significantly with flow, such that higher flow rates produced larger lesions (mean volumes: 120.7 +/- 50.7, 256.5 +/- 97.9, 393.4 +/- 149.9, and 548.9 +/- 157.0 mm(3) for 0, 1, 2, and 3 L/min flow rate respectively, P < 0.001) CONCLUSION: During RF ablation, blood flow rate significantly affects lesion size for large-tip but not cooled-tip catheters. At low flow rates (0-1 L/min) cooled-tip catheters create larger lesions, while at high flow rates (3 L/min) large-tip catheters create larger lesions.