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BACKGROUND: Infants with complete heart block (CHB) require epicardial pacemaker (PM) insertion. Prior studies described epicardial pacing outcomes in infants and children, although they were limited by small or heterogeneous populations. OBJECTIVE: This study aimed to explore patient- and procedure-level associations with device complications in infants with CHB who received a permanent PM. METHODS: This was a multicenter, retrospective cohort study including infants receiving an epicardial PM between 2000 and 2021 for CHB. The primary outcome was time to device-related adverse event: lead failure requiring revision; pocket infection; exit block requiring increased pacing output; or lead-related coronary artery compression. Time-to-event analysis was performed by the Kaplan-Meier method with a multivariable Cox proportional hazards model. RESULTS: There were 174 infants who received an epicardial PM (282 bipolar, 39 unipolar leads) for CHB. Median age and weight at PM were 93.5 days and 4.5 kg, respectively. Pacing indication was postoperative CHB in 63% and congenital CHB in 37%. The median follow-up was 2.1 years. The primary outcome occurred in 26 infants at a median time to event of 0.6 year. Age ≤90 days at PM implantation was the most significant risk factor for a device-related adverse event (hazard ratio, 7.02; P < .001), primarily driven by pocket infections. Lead failure occurred in 3% of leads with a 5- and 10-year freedom from failure of 93% and 83%, respectively. CONCLUSION: Device complications affect 15% of infants receiving a permanent PM for heart block. Age ≤90 days at PM implantation is especially associated with infectious complications. Epicardial lead durability appears similar to previously reported pediatric experiences.
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BACKGROUND: Catheter-based slow pathway modification (SPM) for atrioventricular nodal reentrant tachycardia (AVNRT) is traditionally performed at empiric sites using anatomical landmarks and test ablation feedback within the triangle of Koch (TK). While studies have described more tailored techniques such as bipolar low voltage bridge (LVB) and wavefront collision identification, few have systematically compared the diagnostic yields of each and none have investigated whether omnipolar mapping technology provides incremental benefit. The objective of this study was to compare the utility of omnipolar and bipolar-derived qualitative and quantitative measurements in identifying and localizing dual AVN substrate in patients with versus without AVNRT. METHODS: A retrospective case-control study of consecutive patients with paroxysmal supraventricular tachycardia undergoing electrophysiology study with both omnipolar and bipolar mapping from 2022-2023. RESULTS: Thirteen AVNRT cases (median age 16.1 years, 512 TK points) were compared to nine non-AVNRT controls (median age 15.7 years, 332 TK points). Among qualitative variables, an omnipolar activation vector pivot, defined as a ≥45 degree change in activation direction within the TK, had the highest positive (81%) and negative predictive values (100%) for identifying AVNRT cases and had a median distance of 1 mm from SPM sites. Among quantitative variables, the optimal discriminatory performance for successful SPM sites was observed using bipolar voltage restricted to a peak frequency >340 Hz (c statistic 0.75). CONCLUSIONS: Omnipolar vector pivot analysis represents an automated, annotation-independent qualitative technique that is sensitive and specific for AVNRT substrate and co-localizes with successful SPM sites. Bipolar voltage quantitatively describes SP anisotropy better than omnipolar voltage, and the addition of peak frequency signal analysis further optimizes the selection of SPM sites.
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Frecuencia Cardíaca , Dispositivos Electrónicos Vestibles , Humanos , Niño , Frecuencia Cardíaca/fisiología , Electrocardiografía Ambulatoria/instrumentación , Electrocardiografía Ambulatoria/métodos , Arritmias Cardíacas/fisiopatología , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/terapia , Diseño de Equipo , Monitoreo Fisiológico/métodos , Monitoreo Fisiológico/instrumentaciónRESUMEN
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.
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Terapia de Resincronización Cardíaca , Cardiopatías Congénitas , Insuficiencia Cardíaca , Tetralogía de Fallot , Adulto , Humanos , Terapia de Resincronización Cardíaca/efectos adversos , Cardiopatías Congénitas/terapia , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/etiología , Estudios Retrospectivos , Volumen Sistólico , Tetralogía de Fallot/cirugía , Resultado del Tratamiento , Función Ventricular Izquierda , Persona de Mediana EdadRESUMEN
INTRODUCTION: Management of transvenous leads in patients with congenital heart disease (CHD) can be complicated by venous obstructions and residual shunts. We present our experience performing concurrent lead extraction and dilation/stenting of venous pathways, including patients with complete venous obstruction. METHODS: All cases of concurrent lead extraction and recanalization of vena cavae/baffles between 2017 and 2021 at Boston Children's Hospital were retrospectively included and reviewed for safety and efficacy. RESULTS: Eight patients, 4 female, median 38.5 years of age (range 16.7-49 years) and 81.6 kg weight (range 41.3-97.8 kg) at time of procedure were included. All patients had CHD, a majority (n = 7) having transposition of the great arteries palliated via atrial switch. All leads were removed in their entirety, with most patients having two leads extracted (n = 7). Median lead dwell time was 13.8 years (range 3.6-35.3 years). Three patients had complete obstructions, three required stenting of their innominate veins and three required recanalization of their femoral vessels. Median procedure time was 9.8 h (range 5.4-12.8 h). Complications included blood transfusion (n = 2), arrhythmia (n = 3), pleural effusion (n = 1), and pressure ulcer (n = 1). There were no cardiac perforations, venous tears, or deaths. CONCLUSION: Lead extraction along with dilation and stenting of venous anomalies, though long in duration, proved effective with minimal complications. This combined procedure can safely and effectively resolve complete obstructions secondary to transvenous leads.
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Cardiopatías Congénitas , Transposición de los Grandes Vasos , Enfermedades Vasculares , Malformaciones Vasculares , Niño , Humanos , Femenino , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Dilatación , Estudios Retrospectivos , Remoción de Dispositivos/efectos adversos , Remoción de Dispositivos/métodos , Resultado del TratamientoRESUMEN
BACKGROUND: Evidence for the efficacy of cardiac resynchronization therapy (CRT) in pediatric and congenital heart disease (CHD) has been limited to surrogate outcomes. OBJECTIVES: This study aimed to assess the impact of CRT upon the risk of transplantation or death in a retrospective, high-risk, controlled cohort at 5 quaternary referral centers. METHODS: Both CRT patients and control patients were <21 years of age or had CHD; had systemic ventricular ejection fraction <45%; symptomatic heart failure; and significant electrical dyssynchrony (QRS duration z score >3 or single-site ventricular pacing >40%) at enrollment. Patients with CRT were matched with control patients via 1:1 propensity score matching. CRT patients were enrolled at CRT implantation; control patients were enrolled at the outpatient clinical encounter where inclusion criteria were first met. The primary endpoint was transplantation or death. RESULTS: In total, 324 control patients and 167 CRT recipients were identified. Mean follow-up was 4.2 ± 3.7 years. Upon propensity score matching, 139 closely matched pairs were identified (20 baseline indices). Of the 139 matched pairs, 52 (37.0%) control patients and 31 (22.0%) CRT recipients reached the primary endpoint. On both unadjusted and multivariable Cox regression analysis, the risk reduction associated with CRT for the primary endpoint was significant (HR: 0.40; 95% CI: 0.25-0.64; P < 0.001; and HR: 0.44; 95% CI: 0.28-0.71; P = 0.001, respectively). On longitudinal assessment, the CRT group had significantly improved systemic ventricular ejection fraction (P < 0.001) and shorter QRS duration (P = 0.015), sustained to 5 years. CONCLUSIONS: In pediatric and CHD patients with symptomatic systolic heart failure and electrical dyssynchrony, CRT was associated with improved heart transplantation-free survival.
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Terapia de Resincronización Cardíaca , Cardiopatías Congénitas , Insuficiencia Cardíaca Sistólica , Trasplante de Corazón , Humanos , Niño , Estudios Retrospectivos , Cardiopatías Congénitas/terapia , Insuficiencia Cardíaca Sistólica/terapiaRESUMEN
Cardiac implantable electronic device (CIED) remote transmissions are an integral part of longitudinal follow-up in pediatric and adult congenital heart disease (ACHD) patients. To evaluate baseline CIED remote monitoring (RM) data among pediatric and ACHD centers prior to implementation of a Pediatric and Congenital Electrophysiology Society (PACES)-sponsored quality improvement (QI) project. This is a cross-sectional study of baseline CIED RM. Centers self-reported baseline data: individual center RM compliance was defined as high if there was > 80% achievement and low if < 50%. A total of 22 pediatric centers in the USA and Australia submitted baseline data. Non-physicians were responsible for management of the RM program in most centers: registered nurse (36%), advanced practice provider (27%), combination (23%), and third party (9%). Fifteen centers (68%) reported that > 80% of their CIED patients are enrolled in RM and only two centers reported < 50% participation. 36% reported high compliance of device transmission within 14 days of implant and 77% of centers reported high compliance of CIED patients enrolled in RM. The number of centers achieving high compliance differed by device type: 36% for pacemakers, 50% for ICDs, and 55% for Implantable Cardiac Monitors (ICM). All centers reported at least 50% adherence to recommended follow-up for PM and ICD, with 23% low compliance rate for ICMs. Based on this cross-sectional survey of pediatric and ACHD centers, compliance with CIED RM is sub-optimal. The PACES-sponsored QI initiative will provide resources and support to participating centers and repeat data will be evaluated after PDSA cycles.
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Desfibriladores Implantables , Cardiopatías Congénitas , Marcapaso Artificial , Niño , Humanos , Adulto , Cardiopatías Congénitas/terapia , Estudios Transversales , Mejoramiento de la Calidad , Tecnología de Sensores RemotosRESUMEN
Importance: Surgical correction of congenital heart defects (CHDs) has improved the lifespan and quality of life of pediatric patients. The number of congenital heart surgeries (CHSs) in children has grown continuously since the 1960s. This growth has been accompanied by a rise in the incidence of postoperative heart block requiring permanent pacemaker (PPM) implantation. Objective: To assess the trends in permanent pacing after CHS and estimate the economic burden to patients and their families after PPM implantation. Design, Setting, and Participants: In this economic evaluation study, procedure- and diagnosis-specific codes within a single-institution database were used to identify patients with postoperative heart block after CHS between January 1, 1960, and December 31, 2018. Patients younger than 4 years with postoperative PPM implantation were selected, and up to 20-year follow-up data were used for cost analysis based on mean hospital event charges and length of stay (LOS) data. Data were analyzed from January 1, 2020, to November 30, 2022. Exposure: Implantation of PPM after CHS in pediatric patients. Main Outcomes and Measures: Annual trends in CHS and postoperative PPM implantations were assessed. Direct and indirect costs associated with managing conduction health for the 20 years after PPM implantation were estimated using Markov model simulation and patient follow-up data. Results: Of the 28â¯225 patients who underwent CHS, 968 (437 female [45.1%] and 531 male [54.9%]; 468 patients aged <4 years) received a PPM due to postoperative heart block. The rate of CHS and postoperative PPM implantations increased by 2.2% and 7.2% per year between 1960 and 2018, respectively. In pediatric patients younger than 4 years with PPM implantation, the mean (SD) 20-year estimated direct and indirect costs from Markov model simulations were $180â¯664 ($32â¯662) and $15â¯939 ($1916), respectively. Using follow-up data of selected patients with clinical courses involving 1 or more complication events, the mean (SD) direct and indirect costs were $472â¯774 ($212â¯095) and $36â¯429 ($16â¯706), respectively. Conclusions and Relevance: In this economic evaluation study, the cost of PPM implantation in pediatric patients was found to accumulate over the lifespan. This cost may represent not only a substantial financial burden but also a health care burden to patient families. Reducing the incidence of PPM implantation should be a focused goal of CHS.
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Cardiopatías Congénitas , Marcapaso Artificial , Humanos , Masculino , Niño , Femenino , Calidad de Vida , Marcapaso Artificial/efectos adversos , Bloqueo Cardíaco/epidemiología , Bloqueo Cardíaco/etiología , Bloqueo Cardíaco/terapia , Arritmias Cardíacas , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/complicacionesRESUMEN
INTRODUCTION: The use of flecainide and propafenone for medical cardioversion of atrial fibrillation (AF) and atrial flutter/intra-atrial reentrant tachycardia (IART) is well-described in adults without congenital heart disease (CHD). Data are sparse regarding their use for the same purpose in adults with CHD and in adolescent patients with anatomically normal hearts and we sought to describe the use of class IC drugs in this population and identify factors associated with decreased likelihood of success. METHODS: Single center retrospective cohort study of patients who received oral flecainide or propafenone for medical cardioversion of AF or IART from 2000 to 2022. The unit of analysis was each episode of AF/IART. We performed a time-to-sinus rhythm analysis using a Cox proportional hazards model clustering on the patient to identify factors associated with increased likelihood of success. RESULTS: We identified 45 episodes involving 41 patients. As only episodes of AF were successfully cardioverted with medical therapy, episodes of IART were excluded from our analyses. Use of flecainide was the only factor associated with increased likelihood of success. There was a statistically insignificant trend toward decreased likelihood of success in patients with CHD. CONCLUSIONS: Flecainide was more effective than propafenone. We did not detect a difference in rate of conversion to sinus rhythm between patients with and without CHD and were likely underpowered to do so, however, there was a trend toward decreased likelihood of success in patients with CHD. That said, medical therapy was effective in >50% of patients with CHD with AF.
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Fibrilación Atrial , Aleteo Atrial , Cardiopatías Congénitas , Taquicardia Supraventricular , Adulto , Adolescente , Humanos , Antiarrítmicos/efectos adversos , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/tratamiento farmacológico , Fibrilación Atrial/inducido químicamente , Flecainida/efectos adversos , Propafenona/efectos adversos , Cardioversión Eléctrica/efectos adversos , Estudios Retrospectivos , Taquicardia Supraventricular/inducido químicamente , Aleteo Atrial/diagnóstico , Aleteo Atrial/tratamiento farmacológico , Taquicardia , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/terapiaRESUMEN
In minimally invasive endovascular procedures, surgeons rely on catheters with low dexterity and high aspect ratios to reach an anatomical target. However, the environment inside the beating heart presents a combination of challenges unique to few anatomic locations, making it difficult for interventional tools to maneuver dexterously and apply substantial forces on an intracardiac target. We demonstrate a millimeter-scale soft robotic platform that can deploy and self-stabilize at the entrance to the heart, and guide existing interventional tools toward a target site. In two exemplar intracardiac procedures within the right atrium, the robotic platform provides enough dexterity to reach multiple anatomical targets, enough stability to maintain constant contact on motile targets, and enough mechanical leverage to generate newton-level forces. Because the device addresses ongoing challenges in minimally invasive intracardiac intervention, it may enable the further development of catheter-based interventions.
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Robótica , Catéteres , Atrios Cardíacos , Diseño de EquipoRESUMEN
Pediatric patients with congenital heart disease present unique challenges when it comes to cardiac implantable electronic devices. Pacing strategy is often determined by patient size/weight and operator experience. Anatomic considerations, including residual shunts, anatomic obstructions and barriers, and abnormalities in the native conduction system, will affect the type of CIED implanted. Given the young age of patients, it is important to have an "eye on the future" when making pacemaker/defibrillator decisions, as one can expect several generator changes, lead revisions, and potential lead extractions during their lifetime.
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Desfibriladores Implantables , Cardiopatías Congénitas , Marcapaso Artificial , Humanos , Niño , Cardiopatías Congénitas/terapia , Electrodos Implantados , Remoción de DispositivosRESUMEN
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.
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Desfibriladores Implantables , Cardiopatías Congénitas , Marcapaso Artificial , Adolescente , Niño , Humanos , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/terapia , Imagen por Resonancia Magnética/métodos , Estudios Retrospectivos , Lactante , Preescolar , Adulto Joven , AdultoRESUMEN
INTRODUCTION: Accessory atrioventricular pathways (APs) may mediate atrioventricular reciprocating tachycardia and, in some cases, have the potential to conduct atrial tachycardia rapidly, which can be life threatening. While catheter ablation can be curative, ablation of right free wall APs is associated with a high rate of recurrence, likely secondary to reduced catheter stability along the right free wall atrioventricular groove. We sought to identify characteristics associated with a lower rate of recurrence and hypothesized ablation lesions placed on the ventricular side of the atrioventricular groove using a retroflexed catheter approach would decrease rates of recurrence. METHODS AND RESULTS: Retrospective chart review of patients who underwent catheter ablation of a right free wall AP from January 1, 2008 through June 1, 2021 with >2 months follow up. Cox proportional hazards regression was used to identify relationships between predictor variables and AP recurrence. We identified 95 patients who underwent ablation of 98 right free wall APs. Median age was 13.1 years and median weight at ablation was 52.3 kg. Overall, 23/98 (23%) APs recurred. Use of a retroflexed catheter course approaching the atrioventricular groove from the ventricular aspect was associated with reduced risk of AP recurrence with (univariable hazard ratio of 0.10 [95% confidence interval: 0.01-0.78]), which remained significant in multiple two variable Cox proportional hazards models. CONCLUSION: Use of a retroflexed catheter course is associated with a reduced likelihood of AP recurrence. This approach results in improved catheter stability and should be considered for ablation of right free wall APs.
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Fascículo Atrioventricular Accesorio , Ablación por Catéter , Tabique Interventricular , Síndrome de Wolff-Parkinson-White , Humanos , Adolescente , Estudios Retrospectivos , Fascículo Atrioventricular , Fascículo Atrioventricular Accesorio/cirugía , Catéteres , Ablación por Catéter/efectos adversosRESUMEN
BACKGROUND: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied. OBJECTIVE: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology. METHODS: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics. RESULTS: For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads. CONCLUSION: Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding.
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Miocardio , Marcapaso Artificial , Humanos , Femenino , Adulto Joven , Adulto , Masculino , Estudios Retrospectivos , Miocardio/patología , Corazón , Marcapaso Artificial/efectos adversos , FibrosisRESUMEN
Conduction system pacing (CSP) has evolved rapidly to become the pacing method of choice for many adults with structurally normal hearts. Studies in this population have repeatedly demonstrated superior hemodynamics and outcomes compared to conventional pacing with the recruitment of the native conduction system. Children and patients with congenital heart disease (CHD) are also likely to benefit from CSP but were excluded from original trials. However, very recent studies have begun to demonstrate the feasibility and efficacy of CSP in these patients, with growing evidence that some outcomes may be superior in comparison to conventional pacing techniques. Concerns regarding the technical challenges and long-term lead parameters of His Bundle Pacing (HBP) have been overcome to many extents with the development of Left Bundle Branch Area Pacing (LBBAP), and both techniques are likely to play an important role in pediatric and CHD pacing in the future. This review aims to assimilate the latest developments in CSP and its application in children and CHD patients.
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BACKGROUND: Ventricular fibromas frequently present with life-threatening ventricular tachycardia (VT) or ventricular fibrillation (VF) in children. The long-term risk of sustained ventricular arrhythmias after surgical resection is unknown. OBJECTIVES: The aims of this study were to quantify the VT/VF risk after surgical resection and to examine the results of early (during index surgical hospitalization) and late (≥3 months after surgery) postoperative ventricular stimulation (V-stim) studies. METHODS: We performed a retrospective cohort study of all patients with ventricular fibromas who underwent surgical resection at our institution (2000-2020). The primary outcome was defined as recurrent VT/VF ≥3 months after index surgical resection. RESULTS: Forty-six patients with a median age at surgery of 2 years (range 0.3-18.9 years) formed our study cohort. Indications for surgery included cardiac arrest in 11 (24%), sustained VT in 16 (35%), premature ventricular contractions/nonsustained VT in 10 (22%), and hemodynamic abnormalities in 9 (20%). Of the 11 patients who presented with cardiac arrest, 4 underwent pre-resection implantable cardioverter-defibrillator (ICD) implantation, all of which were explanted at the time of surgery. An early postoperative V-stim study was performed in 26 (57%) patients, and all were negative for inducible VT/VF. Of the 13 late postoperative V-stim studies, 3 were positive: 2 underwent ICD implantation and 1 was initiated on amiodarone and underwent loop recorder implantation. At the time of last follow-up (median 1.5 years; range 0.3-16.5 years), 45 (98%) were free of clinical VT/VF and no patient with an ICD has received an appropriate shock. CONCLUSION: Surgical resection of ventricular fibromas significantly reduces the risk of life-threatening arrhythmias in children; however, a small number of patients remain vulnerable.
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Desfibriladores Implantables , Paro Cardíaco , Taquicardia Ventricular , Niño , Humanos , Lactante , Preescolar , Adolescente , Estudios Retrospectivos , Estudios de Seguimiento , Fibrilación Ventricular/diagnóstico , Fibrilación Ventricular/etiología , Fibrilación Ventricular/terapia , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiología , Taquicardia Ventricular/terapiaRESUMEN
Background The objective of this international multicenter study was to investigate both early and late outcomes of cardiac resynchronization therapy (CRT) in patients with a systemic right ventricle (SRV) and to identify predictors for congestive heart failure readmissions and mortality. Methods and Results This retrospective international multicenter study included 13 centers. The study population comprised 80 adult patients with SRV (48.9% women) with a mean age of 45±14 (range, 18-77) years at initiation of CRT. Median follow-up time was 4.1 (25th-75th percentile, 1.3-8.3) years. Underlying congenital heart disease consisted of congenitally corrected transposition of the great arteries and dextro-transposition of the great arteries in 63 (78.8%) and 17 (21.3%) patients, respectively. CRT resulted in significant improvement in functional class (before CRT: III, 25th-75th percentile, II-III; after CRT: II, 25th-75th percentile, II-III; P=0.005) and QRS duration (before CRT: 176±27; after CRT: 150±24 milliseconds; P=0.003) in patients with pre-CRT ventricular pacing who underwent an upgrade to a CRT device (n=49). These improvements persisted during long-term follow-up with a marginal but significant increase in SRV function (before CRT; 30%, 25th-75th percentile, 25-35; after CRT: 31%, 25th-75th percentile, 21-38; P=0.049). In contrast, no beneficial change in the above-mentioned variables was observed in patients who underwent de novo CRT (n=31). A quarter of all patients were readmitted for heart failure during follow-up, and mortality at latest follow-up was 21.3%. Conclusions This international experience with CRT in patients with an SRV demonstrated that CRT in selected patients with SRV dysfunction and pacing-induced dyssynchrony yielded consistent improvement in QRS duration and New York Heart Association functional status, with a marginal increase in SRV function.
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Terapia de Resincronización Cardíaca , Insuficiencia Cardíaca , Transposición de los Grandes Vasos , Humanos , Adulto , Femenino , Persona de Mediana Edad , Masculino , Terapia de Resincronización Cardíaca/efectos adversos , Terapia de Resincronización Cardíaca/métodos , Ventrículos Cardíacos , Estudios Retrospectivos , Resultado del Tratamiento , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/terapiaRESUMEN
BACKGROUND: Palliation of the single ventricle (SV) circulation is associated with a burden of lifelong complications. Previous studies have identified that the need for a permanent ventricular pacing system (PPMv) may be associated with additional adverse long-term outcomes. OBJECTIVES: The goal of this study was to quantify the attributable risk of PPMv in patients with SV, and to identify modifiable risk factors. METHODS: This international study was sponsored by the Pediatric and Congenital Electrophysiology Society. Centers contributed baseline and longitudinal data for functionally SV patients with PPMv. Enrollment was at implantation. Controls were matched 1:1 to PPMv subjects by ventricular morphology and sex, identified within center, and enrolled at matched age. Primary outcome was transplantation or death. RESULTS: In total, 236 PPMv subjects and 213 matched controls were identified (22 centers, 9 countries). Median age at enrollment was 5.3 years (quartiles: 1.5-13.2 years), follow-up 6.9 years (3.4-11.6 years). Median percent ventricular pacing (Vp) was 90.8% (25th-75th percentile: 4.3%-100%) in the PPMv cohort. Across 213 matched pairs, multivariable HR for death/transplant associated with PPMv was 3.8 (95% CI 1.9-7.6; P < 0.001). Within the PPMv population, higher Vp (HR: 1.009 per %; P = 0.009), higher QRS z-score (HR: 1.19; P = 0.009) and nonapical lead position (HR: 2.17; P = 0.042) were all associated with death/transplantation. CONCLUSIONS: PPMv in patients with SV is associated with increased risk of heart transplantation and death, despite controlling for increased associated morbidity of the PPMv cohort. Increased Vp, higher QRS z-score, and nonapical ventricular lead position are all associated with higher risk of adverse outcome and may be modifiable risk factors.