Catecholaminergic polymorphic ventricular tachycardia

儿茶酚胺能多形性室性心动过速
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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
    该国际多学科文件旨在为临床医生提供循证实用的以患者为中心的建议,以评估(中止)心脏骤停患者和死者及其家人。该文件包括一个调查家庭的框架,允许采取步骤,如果发现遗传条件,尽量减少受影响亲属的进一步事件。这个过程不可或缺的是对患者和家属的咨询,不仅仅是因为情绪激动的话题,但是因为找到(或不找到)逮捕的原因可能会影响家庭成员的管理。多学科团队的形成对于为患者及其家属提供完整的服务至关重要,编写委员会的各种专业知识是为了反映这一需求而制定的。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,按推荐等级和证据等级分级。这些建议已公开征询公众意见,并由亚太心律协会(APHRS)和心律协会(HRS)的相关科学和临床文件委员会进行审查;该文件经过了合作伙伴和合作协会的外部审查和认可。虽然建议是为了最佳护理,人们认识到,并非所有临床医生都能获得所有资源.然而,该文件阐明了临床医生应该渴望为心脏骤停患者提供的评估,突然不明原因死亡的死者,和他们的家人。
    This international multidisciplinary document intends to provide clinicians with evidence-based practical patient-centered recommendations for evaluating patients and decedents with (aborted) sudden cardiac arrest and their families. The document includes a framework for the investigation of the family allowing steps to be taken, should an inherited condition be found, to minimize further events in affected relatives. Integral to the process is counseling of the patients and families, not only because of the emotionally charged subject, but because finding (or not finding) the cause of the arrest may influence management of family members. The formation of multidisciplinary teams is essential to provide a complete service to the patients and their families, and the varied expertise of the writing committee was formulated to reflect this need. 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. The recommendations were opened for public comment and reviewed by the relevant scientific and clinical document committees of the Asia Pacific Heart Rhythm Society (APHRS) and the Heart Rhythm Society (HRS); the document underwent external review and endorsement by the partner and collaborating societies. While the recommendations are for optimal care, it is recognized that not all resources will be available to all clinicians. Nevertheless, this document articulates the evaluation that the clinician should aspire to provide for patients with sudden cardiac arrest, decedents with sudden unexplained death, and their families.
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  • 文章类型: Journal Article
    该国际多学科文件旨在为临床医生提供循证实用的以患者为中心的建议,以评估(中止)心脏骤停患者和死者及其家人。该文件包括一个调查家庭的框架,允许采取步骤,如果发现遗传条件,尽量减少受影响亲属的进一步事件。这个过程不可或缺的是对患者和家属的咨询,不仅仅是因为情绪激动的话题,但是因为找到(或不找到)逮捕的原因可能会影响家庭成员的管理。多学科团队的形成对于为患者及其家属提供完整的服务至关重要,编写委员会的各种专业知识是为了反映这一需求而制定的。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,按推荐等级和证据等级分级。这些建议已公开征询公众意见,并由亚太心律协会(APHRS)和心律协会(HRS)的相关科学和临床文件委员会进行审查;该文件经过了合作伙伴和合作协会的外部审查和认可。虽然建议是为了最佳护理,人们认识到,并非所有临床医生都能获得所有资源.然而,该文件阐明了临床医生应该渴望为心脏骤停患者提供的评估,突然不明原因死亡的死者,和他们的家人。
    This international multidisciplinary document intends to provide clinicians with evidence-based practical patient-centered recommendations for evaluating patients and decedents with (aborted) sudden cardiac arrest and their families. The document includes a framework for the investigation of the family allowing steps to be taken, should an inherited condition be found, to minimize further events in affected relatives. Integral to the process is counseling of the patients and families, not only because of the emotionally charged subject, but because finding (or not finding) the cause of the arrest may influence management of family members. The formation of multidisciplinary teams is essential to provide a complete service to the patients and their families, and the varied expertise of the writing committee was formulated to reflect this need. 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. The recommendations were opened for public comment and reviewed by the relevant scientific and clinical document committees of the Asia Pacific Heart Rhythm Society (APHRS) and the Heart Rhythm Society (HRS); the document underwent external review and endorsement by the partner and collaborating societies. While the recommendations are for optimal care, it is recognized that not all resources will be available to all clinicians. Nevertheless, this document articulates the evaluation that the clinician should aspire to provide for patients with sudden cardiac arrest, decedents with sudden unexplained death, and their families.
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