Update and modification of Task Force Criteria for ARVD/C

更新和修改 ARVD/C 工作组标准

• 批准号: 7407009
• 负责人: FRANK I MARCUS
• 金额: $ 2.19万
• 依托单位: UNIVERSITY OF ARIZONA
• 依托单位国家: 美国
• 财政年份: 2006
• 资助国家: 美国
• 起止时间: 2006-08-23 至 2007-07-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7407009
• 关键词: Address; Advisory Committees; Angiography; Arrhythmia; Arrhythmogenic Right Ventricular Dysplasia; Cardiomyopathies; Cessation of life; Clinical; Clinical Management; Complex; Consensus; Diagnosis; Diagnostic; Disease; Echocardiography; Electrocardiogram; Future; Genetic; Heart failure; Image; Impairment; Journals; Left; Minor; Modality; Modification; Normal Range; Pathology; Patients; Pattern; Peer Review; Phenotype; Publishing; Qualifying; Research; Right Ventricular Function; Right ventricular structure; Standardization; Standards of Weights and Measures; Structure; Testing; Uncertainty; Update; Ventricular; Ventricular Arrhythmia; Ventricular Premature Complexes; Ventricular Tachycardia; Work; base; day; interest; member; new technology; prevent; symposium

DESCRIPTION (provided by applicant): Arrhythmogenic Right Ventricular Dysplasia /Cardiomyopathy (ARVD/C) is a disease characterized by a presence of ventricular arrhythmias originating from the right ventricle including ventricular premature beats, ventricular tachycardia or arrhythmic death. As the disease progresses, right and left heart failure can develop. When patients have typical clinical features including ECG changes and morphological abnormalities limited to the right ventricle, the diagnosis is readily and correctly diagnosed. Since the right ventricular structure and contractile pattern is asymmetrical it can be difficult to differentiate normal from mild abnormalities of this complex ventricular chamber. To address these uncertainties a Task Force was assembled in 1993 to propose diagnostic criteria for ARVD/C. When published in 1994, the Task Force agreed that there was no single test sufficiently specific and sensitive to establish the diagnosis. Therefore, major and minor criteria were selected and it was proposed that the diagnosis of ARVD/C should be made using combinations of these criteria. This working framework was a major advance to standardizing the diagnosis, but it has become evident that it has significant limitations, due to the fact that criteria are vague or based on qualitative rather than quantitative information. For example, there are no quantitative limits to separate mild from moderate or more severe impairment of global right ventricular function. It is now necessary to eliminate subjective assessment and to define quantitative aspects for the Task Force Criteria to better determine the presence of this disease. We propose a two day conference to advance clinical management and scientific progress in the diagnosis of this disease by bringing together several members from the original Task Force on ARVD/C, and current experts in the imaging modalities, electrocardiography and pathology who have a major interest in ARVD/C. The objectives of the proposed conference are: 1) The modification and revision of the Task Force Criteria for the diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), 2) Incorporation of quantitative standards and 3) To eliminate the subjective and qualitative description of qualifying abnormalities. A consensus will be sought on the normal values to be referenced and the parameters or cutoffs as standard deviations from the normal values for mild, moderate and severe abnormalities to be used within the various imaging modalities of echocardiography, angiography and MR I and incorporated into the Task Force Criteria. This standardization will aid the diagnosis of ARVD/C, provide a more defined phenotype and will incorporate the newer technologies for assessing the structure and function of the right ventricle. The revised Task Force Criteria will be published in a peer reviewed journal. This conference to update and modify the Task Force Criteria will enhance future research, and the ability to correctly diagnose the disease and prevent misdiagnosis of an inheritable genetic arrhythmia and cardiomyopathy. null

描述(由申请人提供)： 致心律失常性右室发育不良/心肌病(ARVD/C)是一种以起源于右室的室性心律失常为特征的疾病，包括室性早搏、室性心动过速或心律失常死亡。随着疾病的发展，右心和左心衰竭可能会发展。当患者有典型的临床特征，包括心电图改变和局限于右心室的形态异常时，诊断很容易和正确。由于右室结构和收缩模式是不对称的，因此很难区分这种复杂的室腔的正常和轻度异常。为了解决这些不确定因素，1993年成立了一个工作队，以提出ARVD/C的诊断标准。在1994年发表时，工作队一致认为，没有一种测试足够具体和灵敏来确定诊断。因此，选择了主要标准和次要标准，并建议ARVD/C的诊断应结合这些标准。这一工作框架是诊断标准化方面的重大进步，但由于标准含糊或以定性信息而非定量信息为基础，它显然有很大的局限性。例如，没有数量限制来区分轻度和中度或更严重的右室功能损害。现在必须消除主观评估，并确定工作队标准的量化方面，以便更好地确定这种疾病的存在。我们建议召开一次为期两天的会议，通过聚集最初的ARVD/C工作组的几名成员，以及对ARVD/C有主要兴趣的成像模式、心电图学和病理学的现有专家，来推动临床管理和科学进步。拟议的会议的目标是：1)修改和修订工作组诊断致心律失常性右室发育不良/心肌病(ARVD/C)的标准，2)纳入量化标准，3)消除对定性异常的主观和定性描述。将寻求在超声心动图、血管造影术和磁共振I的各种成像方式中使用的正常参考值以及作为轻度、中度和严重异常的正常值标准偏差的参数或截止值达成共识，并纳入工作队的标准。这一标准化将有助于ARVD/C的诊断，提供更明确的表型，并将纳入评估右室结构和功能的新技术。修订后的工作队标准将在同行评议的期刊上发表。这次更新和修改特别工作组标准的会议将加强未来的研究，以及正确诊断疾病和防止遗传性心律失常和心肌病误诊的能力。 空

项目成果

Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria.

• DOI: 10.1161/circulationaha.108.840827
• 发表时间: 2010-04-06
• 期刊: Circulation
• 影响因子: 37.8
• 作者: Marcus FI;McKenna WJ;Sherrill D;Basso C;Bauce B;Bluemke DA;Calkins H;Corrado D;Cox MG;Daubert JP;Fontaine G;Gear K;Hauer R;Nava A;Picard MH;Protonotarios N;Saffitz JE;Sanborn DM;Steinberg JS;Tandri H;Thiene G;Towbin JA;Tsatsopoulou A;Wichter T;Zareba W
• 通讯作者: Zareba W

Pitfalls in the diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia.

• DOI: 10.1016/j.amjcard.2009.11.033
• 发表时间: 2010-04-01
• 期刊: AMERICAN JOURNAL OF CARDIOLOGY
• 影响因子: 2.8
• 作者: Marcus, Frank;Basso, Cristina;Gear, Kathleen;Sorrell, Vincent L.
• 通讯作者: Sorrell, Vincent L.