Arrhythmogenic cardiomyopathy

致心律失常性心肌病
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  • 文章类型: Practice Guideline
    鉴于当前时代心血管可植入电子设备(CIED)和患者的复杂性日益增加,实践指南,根据需要,变得越来越具体。本文件是一项专家共识声明,旨在更新和进一步描述儿科患者CIED的适应症和管理。定义为≤21岁,并旨在主要关注特定疾病类别中CIED的适应症。该文件还强调了以前发表的成人和儿科CIED建议之间的差异,并为潜在的重要差异提供了理由。该文件讨论了低收入和中等收入国家对CIED获取的一些阻碍因素以及规避这些因素的策略。文件部分由写作委员会成员根据他们的专业知识进行划分和起草。这些建议代表了整个写作委员会的共识意见,按推荐类别和证据级别分级。本文件中涉及的几个问题要么不适合临床试验,要么是罕见的疾病实体,在这些情况下,建议是基于一致的专家意见。此外,具体建议,即使有大量数据支持,不要取代临床判断和患者特定决策的需要。这些建议已向儿科及先天性电生理学会(PACES)成员公开征询公众意见,并接受心律学会(HRS)科学及临床文件委员会的外部审查,美国心脏协会(AHA)的科学咨询和协调委员会,美国心脏病学会(ACC),和欧洲儿科和先天性心脏病协会(AEPC)。该文件获得了所有合作者和亚太心律协会(APHRS)的认可,印度心脏节律学会(IHRS),和拉丁美洲心律协会(LAHRS)。该文件有望为临床医生和患者提供支持,以允许适当的CIED使用,适当的CIED管理,并对儿科患者进行适当的CIED随访。
    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.
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  • 文章类型: Practice Guideline
    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.
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  • 文章类型: Journal Article
    鉴于当前时代心血管可植入电子设备(CIED)和患者的复杂性日益增加,实践指南,根据需要,变得越来越具体。本文件是一项专家共识声明,旨在更新和进一步描述儿科患者CIED的适应症和管理。定义为≤21岁,并旨在主要关注特定疾病类别中CIED的适应症。该文件还强调了以前发表的成人和儿科CIED建议之间的差异,并为潜在的重要差异提供了理由。该文件讨论了低收入和中等收入国家对CIED获取的一些阻碍因素以及规避这些因素的策略。文件部分由写作委员会成员根据他们的专业知识进行划分和起草。这些建议代表了整个写作委员会的共识意见,按推荐类别和证据级别分级。本文件中涉及的几个问题要么不适合临床试验,要么是罕见的疾病实体,在这些情况下,建议是基于一致的专家意见。此外,具体建议,即使有大量数据支持,不要取代临床判断和患者特定决策的需要。这些建议已向儿科及先天性电生理学会(PACES)成员公开征询公众意见,并接受心律学会(HRS)科学及临床文件委员会的外部审查,美国心脏协会(AHA)的科学咨询和协调委员会,美国心脏病学会(ACC),和欧洲儿科和先天性心脏病协会(AEPC)。该文件获得了所有合作者和亚太心律协会(APHRS)的认可,印度心脏节律学会(IHRS),和拉丁美洲心律协会(LAHRS)。该文件有望为临床医生和患者提供支持,以允许适当的CIED使用,适当的CIED管理,并对儿科患者进行适当的CIED随访。
    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.
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  • 文章类型: Journal Article
    自1984年发表最初的ACC/AHA起搏器指南以来,心脏植入式电子设备(CIED)的植入指南已经发展[1]。CIED已经发展到包括新形式的心脏起搏,植入式心律转复除颤器(ICD)的发展以及用于长期监测心律和其他生理参数的装置的引入。鉴于设备和患者的复杂性日益增加,实践指南,根据需要,变得越来越具体。2018年,ACC/AHA/HRS发布了《心动过缓和心脏传导延迟患者评估和管理指南》[2]。这是针对年龄>18岁患者的具体建议。鉴于年轻患者中CIED的不同适应症以及特定尺寸的技术因素,建立了此年龄特定阈值。因此,以下文件是为了更新和进一步描述儿科患者使用和管理CIED的适应症,定义为≤21岁,认识到18至21岁之间的专利护理通常存在重叠。本文件是简短的专家共识声明(ECS),旨在主要关注特定疾病/诊断类别中CIED的适应症。本文件还将为CIED儿科患者提供有关铅系统的管理和随访评估的指导。建议以简化的模块化格式提出,每个部分都包括完整的建议表以及支持文本的简短摘要,并选择参考资料,为建议提供一些背景。本文件无意详尽讨论每一项建议的依据,在全面的PACES-CIED文件[3]中进一步讨论了这些问题,更多的数据可以在电子搜索或教科书中轻松获取。
    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.
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  • 文章类型: Journal Article
    鉴于当前时代心血管可植入电子设备(CIED)和患者的复杂性日益增加,实践指南,根据需要,变得越来越具体。本文件是一项专家共识声明,旨在更新和进一步描述儿科患者CIED的适应症和管理。定义为≤21岁,并旨在主要关注特定疾病类别中CIED的适应症。该文件还强调了以前发表的成人和儿科CIED建议之间的差异,并为潜在的重要差异提供了理由。该文件讨论了低收入和中等收入国家对CIED获取的一些阻碍因素以及规避这些因素的策略。文件部分由写作委员会成员根据他们的专业知识进行划分和起草。这些建议代表了整个写作委员会的共识意见,按推荐类别和证据级别分级。本文件中涉及的几个问题要么不适合临床试验,要么是罕见的疾病实体,在这些情况下,建议是基于一致的专家意见。此外,具体建议,即使有大量数据支持,不要取代临床判断和患者特定决策的需要。这些建议已向儿科及先天性电生理学会(PACES)成员公开征询公众意见,并接受心律学会(HRS)科学及临床文件委员会的外部审查,美国心脏协会(AHA)的科学咨询和协调委员会,美国心脏病学会(ACC),和欧洲儿科和先天性心脏病协会(AEPC)。该文件获得了所有合作者和亚太心律协会(APHRS)的认可,印度心脏节律学会(IHRS),和拉丁美洲心律协会(LAHRS)。该文件有望为临床医生和患者提供支持,以允许适当的CIED使用,适当的CIED管理,并对儿科患者进行适当的CIED随访。
    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.
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  • 文章类型: Journal Article
    致心律失常性心肌病(ACM)是一种不继发于缺血性,高血压,或者心脏瓣膜病.ACM包含了广泛的遗传,系统性,传染性,和炎症性疾病。此名称包括,但不限于,致心律失常右心室/左心室心肌病,心脏淀粉样变性,结节病,查加斯病,和左心室不紧密。ACM表型与其他心肌病重叠,尤其是伴有心律失常的扩张型心肌病,可能与心室扩张和/或收缩功能受损有关。该专家共识声明为临床医生提供了有关ACM评估和管理的指导,并包括有关遗传学和疾病机制的临床相关信息。PICO问题被用来评估当代证据,并提供与致心律失常性右心室心肌病运动相关的临床指导。建议由专家编写小组制定和批准,在用证据表进行系统的文献检索后,讨论他们自己的临床经验,介绍该领域的最新知识。每个建议都是使用美国心脏病学会和美国心脏协会制定的建议类别和证据水平系统提出的,并附有参考文献和解释性文本,以提供必要的上下文。对ACM遗传基础的持续认识为检查疾病和心律失常发展的多种触发因素和潜在的共同途径提供了机会。
    Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
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    Arrhythmogenic cardiomyopathy (ACM) is an arrhythmogenic disorder of the myocardium not secondary to ischemic, hypertensive, or valvular heart disease. ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right/left ventricular cardiomyopathy, cardiac amyloidosis, sarcoidosis, Chagas disease, and left ventricular noncompaction. The ACM phenotype overlaps with other cardiomyopathies, particularly dilated cardiomyopathy with arrhythmia presentation that may be associated with ventricular dilatation and/or impaired systolic function. This expert consensus statement provides the clinician with guidance on evaluation and management of ACM and includes clinically relevant information on genetics and disease mechanisms. PICO questions were utilized to evaluate contemporary evidence and provide clinical guidance related to exercise in arrhythmogenic right ventricular cardiomyopathy. Recommendations were developed and approved by an expert writing group, after a systematic literature search with evidence tables, and discussion of their own clinical experience, to present the current knowledge in the field. Each recommendation is presented using the Class of Recommendation and Level of Evidence system formulated by the American College of Cardiology and the American Heart Association and is accompanied by references and explanatory text to provide essential context. The ongoing recognition of the genetic basis of ACM provides the opportunity to examine the diverse triggers and potential common pathway for the development of disease and arrhythmia.
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