RESUMO
Insertable cardiac monitors (ICMs) have been used more frequently and in a wider variety of circumstances in recent years. ICMs are used for symptom-rhythm correlation when patients have potentially arrhythmogenic syncope and for less traditional reasons such as rhythm surveillance in patients with genetic arrhythmia syndromes or other diseases with high arrhythmia risk. ICMs have good diagnostic yield in pediatric patients and in adults with congenital heart disease and have a low rate of complications. Implantation techniques should take patient-specific factors into account to optimize diagnostic yield and minimize risk.
Assuntos
Eletrocardiografia Ambulatorial , Cardiopatias Congênitas , Adulto , Humanos , Criança , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiologia , Síncope/diagnóstico , Síncope/etiologia , Cardiopatias Congênitas/diagnósticoRESUMO
AIMS: Calmodulinopathy due to mutations in any of the three CALM genes (CALM1-3) causes life-threatening arrhythmia syndromes, especially in young individuals. The International Calmodulinopathy Registry (ICalmR) aims to define and link the increasing complexity of the clinical presentation to the underlying molecular mechanisms. METHODS AND RESULTS: The ICalmR is an international, collaborative, observational study, assembling and analysing clinical and genetic data on CALM-positive patients. The ICalmR has enrolled 140 subjects (median age 10.8 years [interquartile range 5-19]), 97 index cases and 43 family members. CALM-LQTS and CALM-CPVT are the prevalent phenotypes. Primary neurological manifestations, unrelated to post-anoxic sequelae, manifested in 20 patients. Calmodulinopathy remains associated with a high arrhythmic event rate (symptomatic patients, n = 103, 74%). However, compared with the original 2019 cohort, there was a reduced frequency and severity of all cardiac events (61% vs. 85%; P = .001) and sudden death (9% vs. 27%; P = .008). Data on therapy do not allow definitive recommendations. Cardiac structural abnormalities, either cardiomyopathy or congenital heart defects, are present in 30% of patients, mainly CALM-LQTS, and lethal cases of heart failure have occurred. The number of familial cases and of families with strikingly different phenotypes is increasing. CONCLUSION: Calmodulinopathy has pleiotropic presentations, from channelopathy to syndromic forms. Clinical severity ranges from the early onset of life-threatening arrhythmias to the absence of symptoms, and the percentage of milder and familial forms is increasing. There are no hard data to guide therapy, and current management includes pharmacological and surgical antiadrenergic interventions with sodium channel blockers often accompanied by an implantable cardioverter-defibrillator.
Assuntos
Calmodulina , Síndrome do QT Longo , Taquicardia Ventricular , Criança , Humanos , Calmodulina/genética , Morte Súbita Cardíaca/etiologia , Síndrome do QT Longo/diagnóstico , Síndrome do QT Longo/genética , Mutação/genética , Sistema de Registros , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/genéticaRESUMO
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.
Assuntos
Desfibriladores Implantáveis , Cardiopatias Congênitas , Marca-Passo Artificial , Adolescente , Criança , Humanos , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/terapia , Imageamento por Ressonância Magnética/métodos , Estudos Retrospectivos , Lactente , Pré-Escolar , Adulto Jovem , AdultoRESUMO
We describe a case of a previously healthy adolescent who presented with junctional tachycardia and complete atrioventricular (AV) block due to Lyme carditis. The simultaneous presence of these findings suggested significant inflammation of the AV junction. Junctional tachycardia, particularly if seen in a patient with conduction abnormalities and potential tick exposure, should increase suspicion for Lyme carditis.
Assuntos
Bloqueio Atrioventricular , Doença de Lyme , Miocardite , Taquicardia Ectópica de Junção , Taquicardia Ventricular , Humanos , Adolescente , Bloqueio Atrioventricular/diagnóstico , Bloqueio Atrioventricular/etiologia , Miocardite/complicações , Miocardite/diagnóstico , Eletrocardiografia , Doença de Lyme/complicações , Doença de Lyme/diagnóstico , Taquicardia Ectópica de Junção/diagnósticoRESUMO
PURPOSE OF REVIEW: COVID-19-related guidance has changed dramatically since the onset of the pandemic. Awareness of data regarding prevention of disease, the cardiac manifestations and treatment of acute COVID-19 and multisystem inflammatory syndrome in children, and return to physical activity following an infection allows for appropriate adjustment of current care models and guides future study. RECENT FINDINGS: Severe acute respiratory syndrome coronavirus 2 transmission can be reduced using various mitigation strategies, though their effectiveness differs based on viral prevalence. The risk of severe disease during acute COVID-19 infection is low in children and adolescents, though specific risk factors have been identified. COVID-19 vaccination significantly decreases the risk of severe disease and poor outcomes. Regular physical activity positively affects well being and has been a focus of recent guidance regarding well tolerated return to activities following an infection. SUMMARY: The use of strategies to reduce viral transmission will depend on individuals' and communities' risk tolerance and on current viral prevalence. COVID-19 vaccination should be encouraged, particularly in patients with identified risk factors. Allowing children and adolescents to safely participate in physical and other activities should continue to be a focus of our clinical and research efforts given their myriad benefits in this population.
Assuntos
COVID-19 , Adolescente , COVID-19/complicações , COVID-19/epidemiologia , COVID-19/prevenção & controle , Vacinas contra COVID-19 , Criança , Humanos , Pandemias/prevenção & controle , Volta ao Esporte , Síndrome de Resposta Inflamatória SistêmicaRESUMO
BACKGROUND: TANGO2 deficiency disorder (TDD) is an autosomal recessive disease associated with metabolic crisis, lethal cardiac arrhythmias, and cardiomyopathy. Data regarding treatment, management, and outcomes of cardiac manifestations of TDD are lacking. OBJECTIVE: The purpose of this study was to describe TDD-related cardiac crises. METHODS: Retrospective multicenter chart review was made of TDD patients admitted with cardiac crises, defined as development of ventricular tachycardia (VT), cardiomyopathy, or cardiac arrest during metabolic crises. RESULTS: Twenty-seven children were admitted for 43 cardiac crises (median age 6.4 years; interquartile range [IQR] 2.4-9.8 years) at 14 centers. During crisis, QTc prolongation occurred in all (median 547 ms; IQR 504-600 ms) and a type I Brugada pattern in 8 (26%). Arrhythmias included VT in 21 (78%), supraventricular tachycardia in 3 (11%), and heart block in 1 (4%). Nineteen patients (70%) developed cardiomyopathy, and 20 (74%) experienced a cardiac arrest. There were 10 deaths (37%), 6 related to arrhythmias. In 5 patients, recalcitrant VT occurred despite use of antiarrhythmic drugs. In 6 patients, arrhythmias were controlled after extracorporeal membrane oxygenation (ECMO) support; 5 of these patients survived. Among 10 patients who survived VT without ECMO, successful treatment included intravenous magnesium, isoproterenol, and atrial pacing in multiple cases and verapamil in 1 patient. Initiation of feeds seemed to decrease VT events. CONCLUSION: TDD-related cardiac crises are associated with a high risk of arrhythmias, cardiomyopathy, cardiac arrest, and death. Although further studies are needed, early recognition and appropriate treatment are critical. Acutely, intravenous magnesium, isoproterenol, atrial pacing, and ECMO as a last resort seem to be the best current treatment options, and early initiation of feeds may prevent VT events.
Assuntos
Cardiomiopatias , Parada Cardíaca , Taquicardia Ventricular , Antiarrítmicos/uso terapêutico , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/terapia , Cardiomiopatias/complicações , Cardiomiopatias/diagnóstico , Criança , Parada Cardíaca/etiologia , Parada Cardíaca/terapia , Humanos , Isoproterenol , Magnésio , VerapamilRESUMO
We present a case of a young boy who developed persistent tachycardia despite fluid resuscitation, antipyretics, and analgesia after a Fontan procedure. Review of telemetry and ECGs revealed repolarization abnormalities, including the appearance of T-wave alternans, for which an uncommon cause was ultimately identified.
Assuntos
Eletrocardiografia , Cardiopatias Congênitas , Arritmias Cardíacas , Eletrocardiografia/métodos , Cardiopatias Congênitas/complicações , Cardiopatias Congênitas/cirurgia , Humanos , MasculinoRESUMO
In children, the Impella® is most commonly used in the setting of cardiogenic shock. There are few reported cases of Impella® use in pediatric patients undergoing ablation; description of troubleshooting techniques may improve success rates. We describe a pediatric patient with tachycardia-induced cardiomyopathy due to incessant ectopic atrial tachycardia whose ablation was notable for significant electromagnetic interference (EMI) from the Impella® leading to incomplete mapping. This case highlights the need for multidisciplinary planning and consideration of possible EMI with the use of magnet-based electroanatomic mapping systems as well as troubleshooting techniques to reduce the impact of EMI.
RESUMO
BACKGROUND: Electronic gaming has recently been reported as a precipitant of life-threatening cardiac arrhythmia in susceptible individuals. OBJECTIVE: The purpose of this study was to describe the population at risk, the nature of cardiac events, and the type of game linked to cardiac arrhythmia associated with electronic gaming. METHODS: A multisite international case series of suspected or proven cardiac arrhythmia during electronic gaming in children and a systematic review of the literature were performed. RESULTS: Twenty-two patients (18 in the case series and 4 via systematic review; aged 7-16 years; 19 males [86%]) were identified as having experienced suspected or proven ventricular arrhythmia during electronic gaming; 6 (27%) had experienced cardiac arrest, and 4 (18%) died suddenly. A proarrhythmic cardiac diagnosis was known in 7 (31%) patients before their gaming event and was established afterward in 12 (54%). Ten patients (45%) had catecholaminergic polymorphic ventricular tachycardia, 4 (18%) had long QT syndrome, 2 (9%) were post-congenital cardiac surgery, 2 (9%) had "idiopathic" ventricular fibrillation, and 1 (after Kawasaki disease) had coronary ischemia. In 3 patients (14%), including 2 who died, the diagnosis remains unknown. In 13 (59%) patients for whom the electronic game details were known, 8 (62%) were war games. CONCLUSION: Electronic gaming can precipitate lethal cardiac arrhythmias in susceptible children. The incidence appears to be low, but syncope in this setting should be investigated thoroughly. In children with proarrhythmic cardiac conditions, electronic war games in particular are a potent arrhythmic trigger.
Assuntos
Taquicardia Ventricular , Jogos de Vídeo , Masculino , Criança , Humanos , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/complicações , Coração , Taquicardia Ventricular/etiologia , Taquicardia Ventricular/complicações , Morte Súbita , Jogos de Vídeo/efeitos adversos , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/etiologiaRESUMO
Wide complex tachycardia (WCT) is an infrequently encountered condition in paediatric patients and may be due to a variety of causes including supraventricular tachycardia with aberrant conduction, ventricular activation via an accessory pathway, ventricular pacing, or ventricular tachycardia. Immediate tachycardia termination is required in haemodynamically unstable patients. After stabilization or in those with haemodynamically tolerated WCT, a careful review of electrocardiographic tracings and diagnostic manoeuvres are essential to help elucidate the cause. Subacute and chronic management for WCT will depend on the underlying cause as well as features of the patient and the tachycardia presentation. This article will review the epidemiology, potential causes, and management of WCT in children. A detailed review of the pathophysiology, differential diagnosis, and diagnostic and treatment options is provided to enable the reader to develop a practical approach to managing this condition in young patients.
La tachycardie à complexes QRS larges est rare en pédiatrie et peut avoir diverses causes, notamment une tachycardie supraventriculaire avec trouble de la conduction, l'activation ventriculaire par une voie accessoire, une stimulation ventriculaire ou une tachycardie ventriculaire. La suppression immédiate de la tachycardie est primordiale lorsque l'état hémodynamique du patient est instable. Une fois l'état du patient stabilisé, ou en cas de tachycardie à complexes QRS larges tolérée sur le plan hémodynamique, l'examen minutieux des tracés électrocardiographiques et des manÅuvres diagnostiques est crucial pour en élucider la cause. La prise en charge des cas subaigus et chroniques de tachycardie à complexes QRS larges dépend de sa cause sous-jacente ainsi que des caractéristiques du patient et du tableau clinique de la tachycardie. Cet article porte sur l'épidémiologie, les causes possibles et la prise en charge de la tachycardie à complexes QRS larges chez les enfants. Un examen approfondi de la physiopathologie, du diagnostic différentiel et des options diagnostiques et thérapeutiques est présenté pour permettre au lecteur d'élaborer une approche pratique pour la prise en charge de cette affection chez leurs jeunes patients.
Assuntos
Desfibriladores Implantáveis , Taquicardia Ventricular , Criança , Morte Súbita Cardíaca , Eletrônica , Coração , HumanosRESUMO
In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.
Assuntos
Cardiologia , Desfibriladores Implantáveis , Adulto , American Heart Association , Criança , Eletrônica , Humanos , América Latina , Estados UnidosRESUMO
In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.
Assuntos
Cardiologia , Desfibriladores Implantáveis , American Heart Association , Eletrofisiologia Cardíaca , Criança , Consenso , Eletrônica , Humanos , Estados UnidosRESUMO
Guidelines for the implantation of cardiac implantable electronic devices (CIEDs) have evolved since publication of the initial ACC/AHA pacemaker guidelines in 1984 [1]. CIEDs have evolved to include novel forms of cardiac pacing, the development of implantable cardioverter defibrillators (ICDs) and the introduction of devices for long term monitoring of heart rhythm and other physiologic parameters. In view of the increasing complexity of both devices and patients, practice guidelines, by necessity, have become increasingly specific. In 2018, the ACC/AHA/HRS published Guidelines on the Evaluation and Management of Patients with Bradycardia and Cardiac Conduction Delay [2], which were specific recommendations for patients >18 years of age. This age-specific threshold was established in view of the differing indications for CIEDs in young patients as well as size-specific technology factors. Therefore, the following document was developed to update and further delineate indications for the use and management of CIEDs in pediatric patients, defined as ≤21 years of age, with recognition that there is often overlap in the care of patents between 18 and 21 years of age. This document is an abbreviated expert consensus statement (ECS) intended to focus primarily on the indications for CIEDs in the setting of specific disease/diagnostic categories. This document will also provide guidance regarding the management of lead systems and follow-up evaluation for pediatric patients with CIEDs. The recommendations are presented in an abbreviated modular format, with each section including the complete table of recommendations along with a brief synopsis of supportive text and select references to provide some context for the recommendations. This document is not intended to provide an exhaustive discussion of the basis for each of the recommendations, which are further addressed in the comprehensive PACES-CIED document [3], with further data easily accessible in electronic searches or textbooks.
RESUMO
In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.