2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients: Developed in collaboration with and endorsed by the Heart Rhythm Society (HRS), the American College of Cardiology (ACC), the American Heart Association (AHA), and the Association for European Paediatric and Congenital Cardiology (AEPC) Endorsed by the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS).

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.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

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.

2021 PACES Expert Consensus Statement on the Indications and Management of Cardiovascular Implantable Electronic Devices in Pediatric Patients.

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.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients: Executive summary.

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.

2021 PACES expert consensus statement on the indications and management of cardiovascular implantable electronic devices in pediatric patients.

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.

Remisión espontánea a largo plazo de la forma congénita de la taquicardia ectópica de la unión / Long-term Spontaneous Remission of Congenital Junctional Ectopic Tachycardia

RESUMEN Introducción: La forma congénita de la taquicardia ectópica de la unión (junctional ectopic tachycardia, JET) es una taquicardia supraventricular poco común, con alta morbimortalidad. Su tratamiento requiere, frecuentemente, el uso de múltiples fármacos durante períodos prolongados. En algunos casos se necesita, además, realizar un procedimiento invasivo, como la crioablación. Algunos estudios han demostrados la existencia de casos de remisión espontánea de la arritmia a largo plazo (con la reversión a ritmo sinusal o a un ritmo nodal acelerado), sin requerimiento de fármacos y sin mediar intervención alguna. Objetivos: Evaluar la incidencia de remisión espontanea de la JET congénita en una población pediátrica en el seguimiento a largo plazo. Material y métodos: Se incluyeron 23 pacientes consecutivos con JET congénita evaluados en el Hospital J.P. Garrahan y en el Hospital Italiano de Buenos Aires, entre los años 1999 y 2017, con un seguimiento medio de 8,8 años (rango intercuartil, RIC: 5,5-14,1). Se evaluó a largo plazo la incidencia de remisión espontánea de la arritmia. Resultados: La mediana para la edad de presentación fue 2 meses (RIC: 0,625-3); 15 pacientes (62,5%) presentaron signos de insuficiencia cardíaca y 11 pacientes (45,8%), taquicardiomiopatía. A 2 pacientes se les realizó una crioablación en forma exitosa. La tasa de remisión espontánea fue del 52%. En aquellos pacientes con un seguimiento mayor a 10 años (15 sujetos), la remisión fue del 62,5%. La media para edad de remisión fue 9,6 años. Conclusiones: La JET congénita es una taquicardia potencialmente grave en los primeros meses de vida, pero con alta tasa de remisión espontánea a largo plazo.

ABSTRACT Background: The congenital form of junctional ectopic tachycardia (JET) is a rare supraventricular tachycardia with high morbidity and mortality. Its treatment frequently requires the prolonged use of multiple drugs and, in some cases, an inva-sive procedure, such as cryoablation, is necessary. Some studies have demonstrated the existence of spontaneous remission of arrhythmia in the long term (with reversal to sinus rhythm or to an accelerated nodal rhythm), without need of drugs or any other form of intervention. Objectives: The aim of this study was to evaluate the long-term incidence of spontaneous remission of congenital JET in a pediatric population. Methods: Twenty-three consecutive patients with congenital JET were evaluated at Hospital J.P. Garrahan and Hospital Italiano de Buenos Aires, between 1999 and 2017, with a mean follow-up of 8.8 years interquartile range (IQR): 5.5-14.1). The incidence of spontaneous arrhythmia remission was evaluated in the long term. Results: Median presentation age was 2 months (IQR: 0.625-3); 15 patients (62.5%) presented signs of heart failure and 11 patients (45.8%) tachycardiomyopathy. Two patients underwent successful cryoablation. The spontaneous remission rate was 52%. In patients with follow-up above 10 years (15 subjects), remission was 62.5%. Mean remission age was 9.6 years. Conclusions: Congenital JET is a potentially severe tachycardia in the first months of life, but with a high rate of long-term spontaneous remission.

Usefulness of remote Monitoring of Pediatric Patients with Cardiac implantable electronic devices / Utilidad de los dispositivos cardíaco-eléctricos con monitoreo a distancia en pacientes pediátricos

ABSTRACT: Background: The aim of this study is to evaluate the usefulness of cardiac implantable electronic devices with remote monitoring system in a pediatric population and the limitations of its implementation in Argentina. Methods: Twenty-seven patients receiving a cardiac implantable electronic device with remote monitoring system at Hospital Nacional Garrahan and Hospital Italiano de Buenos Aires were included in the study. The rate of events, complications and device-related therapies were evaluated. The anticipated actions taken in response to alert notifications were described. Mean follow-up was 46.6 ± 32.1 months. results: Median age was 12.2 years (IQR: 8.75-13.3). An implantable cardioverter defibrillator device was placed in 7 patients (25.9%) and 20 (74%) underwent pacemaker implant. Five patients (18.5%) presented seven red alerts: 3 due to ventricular arrhyth-mia in monitoring zone of ventricular fibrillation and 4 due to lead dysfunction. Twelve patients (44%) presented a yellow alert: 6 due to lead dysfunction, 4 due to deactivation of the monitoring system because of lack of signal reception, one due to ventricular tachycardia and another with sinus tachycardia in monitoring zone of ventricular tachycardia. Active actions were taken in 9 pa-tients (33.3%) to manage the alert notification: the atrial lead was replaced in one patient and the ventricular in lead in another; in 2 patients non-compliance with pharmacological treatment and exercise limitation were detected and in the rest of the patients, the device was reprogrammed according to the abnormalities observed in the recording or capture. Conclusions: Remote monitoring of cardiac implantable electronic devices is very useful in the pediatric population, allowing for the rapid detection and management of device failure or significant arrhythmias.

RESUMEN: Objetivo: Evaluar la utilidad de los dispositivos cardíacos eléctricos con sistema de monitoreo a distancia en una población pediátrica y las limitaciones de su implementación en la República Argentina. Material y métodos: Se incluyeron 27 pacientes a quienes se le implantó un dispositivo cardíaco-eléctrico implantable con sistema de monitoreo a distancia en el Hospital Nacional Garrahan y en el Hospital Italiano de Bs. As. Se evaluó la tasa de eventos, complicaciones y terapias por parte de los dispositivos. Se describieron las conductas anticipadas según la alerta recibida. Se realizó un seguimiento medio de 46,6 meses ± 32,1. resultados: La edad fue 12,2 años (RIC: 8,75-13,3), a 7 pacientes (28%) se les implantó un cardiodesfibrilador implantable y 20 pacientes (78%) un marcapaso endocavitario. Cinco pacientes (18,5%) presentaron 7 alertas rojas: 3 por arritmia ventricular en rango de fibrilación ventricular y 4 por alteraciones en alguno de los cables. Doce pacientes (44%) presentaron una alerta amarilla: 6, por alteraciones en los cables; 4, por desactivación del sistema por falta de recepción de señal; 1, por taquicardia ventricular; y 1, por taquicardia sinusal en rango de taquicardia ventricular. En 9 pacientes (33,3%) se tomó una conducta activa para resolver el aviso de alerta: en 2 pacientes se realizó recambio de cable auricular en uno y ventricular en otro, en 2 pacientes se detectó incumplimiento del tratamiento farmacológico y en la limitación del ejercicio, en el resto se reprogramó el dispositivo, según el tipo de alteración en el registro o la captura. Conclusiones: El sistema de monitoreo remoto de los dispositivos cardíaco-eléctricos implantables es muy útil en la población pediátrica, lo que permite una rápida detección y acción cuando se produce un fallo en el dispositivo o un evento arrítmico de relevancia clínica.

[An apparent life threatening secondary to long Qt syndrome]. / Aparente amenaza para la vida por el síndrome de QT prolongado.

We report the case of an infant with an episode of loss of consciousness, in whom ventricular fibrillation was diagnosed. He was successfully defibrillated and long QT syndrome was diagnosed as his baseline disease. This case constitutes a documented example of this entity as a cause of the sudden infant death syndrome.

Aparente amenaza para la vida por el síndrome de QT prolongado / An apparent life threatening secondary to long Qt syndrome

Se presenta el caso de un lactante con un episodio de pérdida de conocimiento, en quien se diagnosticó fibrilación ventricular. Se realizó desfibrilación externa con éxito, permitiendo luego arribar al diagnóstico etiológico de síndrome de QT prolongado, constituyendo un ejemplo documentado de esta entidad como causa del síndrome de muerte súbita del lactante.

We report the case of an infant with an episode of loss of consciousness, in whom ventricular fibrillation was diagnosed. He was successfully defibrillated and long QT syndrome was diagnosed as his baseline disease. This case constitutes a documented example of this entity as a cause of the sudden infant death syndrome.

Aparente amenaza para la vida por el síndrome de QT prolongado / An apparent life threatening secondary to long Qt syndrome

Se presenta el caso de un lactante con un episodio de pérdida de conocimiento, en quien se diagnosticó fibrilación ventricular. Se realizó desfibrilación externa con éxito, permitiendo luego arribar al diagnóstico etiológico de síndrome de QT prolongado, constituyendo un ejemplo documentado de esta entidad como causa del síndrome de muerte súbita del lactante.(AU)

We report the case of an infant with an episode of loss of consciousness, in whom ventricular fibrillation was diagnosed. He was successfully defibrillated and long QT syndrome was diagnosed as his baseline disease. This case constitutes a documented example of this entity as a cause of the sudden infant death syndrome.(AU)

Aparente amenaza para la vida por el síndrome de QT prolongado. / [An apparent life threatening secondary to long Qt syndrome].

We report the case of an infant with an episode of loss of consciousness, in whom ventricular fibrillation was diagnosed. He was successfully defibrillated and long QT syndrome was diagnosed as his baseline disease. This case constitutes a documented example of this entity as a cause of the sudden infant death syndrome.

[Congenital junctional ectopic tachycardia. Pharmacologic management during infancy]. / Taquicardia ectópica congénita de la unión. Tratamiento farmacológico en el primer año de vida.

Congenital junctional ectopic tachycardia (JET) is a rare arrhythmia that can be refractory to medical therapy with high morbidity and mortality rates. The aim of this study was to report our experience with pharmacologic management of congenital JET in infants. Seven patients with congenital JET were identified between 2008 and 2010. Only two of them presented dilated cardiomyopathy. There were no congenital structural defects. Amiodarone was given to all the patients, as single therapy in one, and in combination with propranolol in four. In one patient flecainide was administered together with amiodarone and propranolol, and in another patient was used combined with amiodarone. During follow- up with an average time of 12.2 months (median 9.75 months, range 1-28 months), sinus rhythm alternating with slow junctional tachycardia was successfully achieved in 3 patients; no side effects were detected. There was only one death in our study group. The combination of different antiarrhythmics (amiodarone plus propranolol, and eventually flecainide) is a valid option for rhythm control and management of JET in infants.

Taquicardia ectópica congénita de la unión: Tratamiento farmacológico en el primer año de vida / Congenital junctional ectopic tachycardia: Pharmacologic management during infancy

La forma congénita de la taquicardia ectópica de la unión (TEU) es una arritmia poco frecuente que suele presentar dificultades en su manejo farmacológico, con altas tasas de morbilidad y mortalidad. El objetivo de este trabajo fue informar la experiencia en el seguimiento y el tratamiento de esta forma de taquicardia supraventricular en pacientes menores de un año. Se identificaron siete pacientes con TEU congénita en 28 meses de seguimiento entre 2008 y 2010. El diagnóstico fue realizado en el primer día de vida en cuatro pacientes y dentro de los 150 días de vida en los 3 restantes. Sólo dos presentaron miocardiopatía dilatada. Ninguno presentó cardiopatía estructural. Se utilizó amiodarona en todos los pacientes, en un caso como única droga, asociándose a propanolol en cuatro. En un paciente se asoció flecainida a estos dos fármacos y en otro se la combinó con amiodarona. En un tiempo de seguimiento con un rango de 1-28 meses (media 12.2 meses, mediana 9.75 meses) en tres de los pacientes se consiguió obtener ritmo sinusal alternante con taquicardia nodal lenta; ninguno presentó efectos adversos secundarios a la medicación, ni deterioro de la función ventricular. Hubo sólo una muerte en el grupo estudiado. En conclusión, la combinación de fármacos antiarrítmicos (amiodarona más propranolol y eventualmente flecainida) constituye una alternativa válida para un adecuado control de la TEU congénita en pacientes menores de un año de edad.

Congenital junctional ectopic tachycardia (JET) is a rare arrhythmia that can be refractory to medical therapy with high morbidity and mortality rates. The aim of this study was to report our experience with pharmacologic management of congenital JET in infants. Seven patients with congenital JET were identified between 2008 and 2010. Only two of them presented dilated cardiomyopathy. There were no congenital structural defects. Amiodarone was given to all the patients, as single therapy in one, and in combination with propranolol in four. In one patient flecainide was administered together with amiodarone and propranolol, and in another patient was used combined with amiodarone. During follow- up with an average time of 12.2 months (median 9.75 months, range 1-28 months), sinus rhythm alternating with slow junctional tachycardia was successfully achieved in 3 patients; no side effects were detected. There was only one death in our study group. The combination of different antiarrhythmics (amiodarone plus propranolol, and eventually flecainide) is a valid option for rhythm control and management of JET in infants.